Zapalenie naczyń
Diagnostyka i diagnoza

Zapalenie naczyń (vasculitis) to heterogenna grupa chorób charakteryzujących się zapaleniem ścian naczyń krwionośnych, co prowadzi do uszkodzeń narządów i tkanek. Diagnostyka jest wyzwaniem ze względu na niespecyficzne objawy i rzadkość schorzeń, co skutkuje średnim czasem do rozpoznania wynoszącym około 7 miesięcy oraz błędną diagnozą u 73% pacjentów. Kluczowe jest systematyczne podejście diagnostyczne obejmujące szczegółowy wywiad, badanie fizykalne, badania laboratoryjne (m.in. morfologia, OB, CRP, ANCA, ANA, RF, poziomy dopełniacza C3 i C4) oraz obrazowe (RTG, USG, CT, MRI, PET, angiografia). Biopsja zajętego narządu pozostaje złotym standardem potwierdzenia rozpoznania, szczególnie w przypadku zapalenia naczyń OUN, tętnicy skroniowej, nerek czy płuc. W diagnostyce AAV istotne jest wykrycie przeciwciał ANCA: c-ANCA (PR3) w ziarniniakowatości z zapaleniem naczyń (GPA) oraz p-ANCA (MPO) w mikroskopowym zapaleniu naczyń (MPA) i eozynofilowej ziarniniakowatości (EGPA).

Zapalenie naczyń – diagnostyka i rozpoznanie

Zapalenie naczyń (vasculitis) to grupa chorób charakteryzujących się zapaleniem ścian naczyń krwionośnych. Diagnostyka zapalenia naczyń często stanowi wyzwanie dla klinicystów ze względu na niespecyficzność objawów oraz heterogenność poszczególnych typów zapaleń naczyń. Wczesne rozpoznanie i wdrożenie leczenia ma kluczowe znaczenie dla zapobiegania trwałym uszkodzeniom narządów i tkanek.12

Trudności diagnostyczne w zapaleniu naczyń

Diagnostyka zapalenia naczyń może być problematyczna z kilku powodów. Przede wszystkim większość typów zapaleń naczyń to choroby rzadkie. Pacjenci często prezentują objawy, które mogą być przypisane wielu różnym chorobom, ponieważ zapalenie może dotyczyć dowolnego narządu. Symptomy są często niespecyficzne i mogą naśladować inne, częstsze schorzenia. Z powodu tych trudności diagnoza może być opóźniona lub pominięta przez dłuższy czas.12

Badania przeprowadzone wśród pacjentów z zapaleniem naczyń wykazały, że średni czas do postawienia diagnozy wynosi około 7 miesięcy, a aż 73% pacjentów jest początkowo błędnie diagnozowanych. Opóźnienia diagnostyczne wiążą się z negatywnymi konsekwencjami zdrowotnymi dla 82% pacjentów.1

Podejście do diagnostyki zapalenia naczyń

Właściwe podejście diagnostyczne powinno obejmować systematyzację objawów, które na pierwszy rzut oka mogą wydawać się niezależne. Klinicysta powinien ocenić, czy poszczególne objawy miejscowe są wynikiem niedokrwienia spowodowanego zapaleniem naczyń, czy też wynikają z krwawienia.1

Objawy kliniczne pierwotnych zespołów zapalenia naczyń można ogólnie podzielić na dwie kategorie:1

  • Ogólnoustrojowe objawy/symptomy spowodowane zapaleniem
  • Zlokalizowane objawy narządowe specyficzne dla zajętych organów

W przypadku podejrzenia zapalenia naczyń, zaleca się przeprowadzenie diagnostyki różnicowej w celu wykluczenia innych stanów. Należy pamiętać, że wymienione wcześniej objawy ogólnoustrojowe są często spotykane u osób starszych z nowotworami złośliwymi. Dlatego w trakcie procesu diagnostycznego zapalenia naczyń zaleca się również przeprowadzenie badań przesiewowych w kierunku nowotworów złośliwych.2

Metody diagnostyczne w rozpoznawaniu zapalenia naczyń

Ponieważ nie istnieje pojedynczy test umożliwiający diagnozę zapalenia naczyń, kluczowe jest zastosowanie różnych metod diagnostycznych. Diagnostyka powinna obejmować dokładny wywiad medyczny, badanie fizykalne oraz odpowiednie badania laboratoryjne i obrazowe.12

Biopsja tkanek

Biopsja jest uważana za najważniejszą metodę diagnostyczną w zapaleniu naczyń. Polega ona na pobraniu małej próbki tkanki z zajętego obszaru i badaniu jej pod mikroskopem w celu wykrycia oznak zapalenia naczyń krwionośnych.12

Badania krwi, zdjęcia rentgenowskie i inne badania mogą sugerować diagnozę zapalenia naczyń, jednak często jedynym sposobem na potwierdzenie diagnozy jest biopsja zajętej tkanki, zbadanie jej pod mikroskopem w konsultacji z patologiem (najlepiej doświadczonym w badaniu biopsji w zapaleniu naczyń) i znalezienie patologicznych oznak choroby.1

W zależności od objawów, badania fizykalnego i testów diagnostycznych wskazujących na zajęcie określonego narządu, można wykorzystać następujące procedury biopsyjne:23

  • Biopsja nerki – wykonywana w przypadku dowodów na zajęcie nerek przez zapalenie naczyń (np. czerwone krwinki lub białko w moczu)
  • Biopsja nerwu łydkowego – nerw czuciowy nad boczną częścią stopy
  • Biopsja tętnicy skroniowej – wykonywana w celu zdiagnozowania zapalenia tętnicy skroniowej (olbrzymiokomórkowego zapalenia tętnic)
  • Biopsja płuca – może być wykonana na dwa sposoby: otwarta biopsja płuca (znaczna procedura chirurgiczna) lub biopsja torakoskopowa płuca (mniej inwazyjna, ale wciąż istotna procedura)
  • Biopsja mózgu – często niezbędna do potwierdzenia diagnozy zapalenia naczyń ośrodkowego układu nerwowego (CNS)

Idealnie jest wykonać biopsję w ciągu 24-48 godzin od pojawienia się zmian, ponieważ proces zapalny jest dynamiczny i biopsja wykonana zbyt wcześnie lub zbyt późno może nie wykazać klasycznych zmian histopatologicznych.1

Badania laboratoryjne

Badania laboratoryjne są niezbędne do wykrycia oznak zapalenia, określenia typu i zakresu zajęcia narządów, testowania na obecność przeciwciał specyficznych dla zapalenia naczyń oraz wykluczenia przyczyn wtórnych.1

Badania krwi mogą wykazać oznaki zapalenia, takie jak podwyższony poziom białka C-reaktywnego. Pełna morfologia krwi może wskazać, czy masz wystarczającą liczbę czerwonych krwinek. Inne badania krwi, takie jak test na przeciwciała przeciwko cytoplazmie neutrofilów (ANCA), mogą wykryć określone przeciwciała.1

Najczęściej wykonywane badania laboratoryjne obejmują:123

  • Pełną morfologię krwi (CBC)
  • Odczyn Biernackiego (OB) i białko C-reaktywne (CRP) – wskaźniki zapalenia
  • Przeciwciała przeciwko cytoplazmie neutrofilów (ANCA)
  • Przeciwciała przeciwjądrowe (ANA)
  • Czynnik reumatoidalny (RF)
  • Krioglobuliny
  • Poziomy dopełniacza (C3, C4)
  • Badania w kierunku wirusowego zapalenia wątroby typu B i C

Szczególnie przydatne w diagnostyce niektórych typów zapalenia naczyń są przeciwciała ANCA. Badanie ANCA zazwyczaj rozpoczyna się od immunofluorescencji pośredniej w celu wykrycia wzoru barwienia – cytoplazmatycznego (c-ANCA) lub okołojądrowego (p-ANCA).1

W zapaleniu naczyń związanym z ANCA (AAV), takich jak ziarniniakowatość z zapaleniem naczyń (GPA, dawniej ziarniniakowatość Wegenera), mikroskopowe zapalenie naczyń (MPA) oraz eozynofilowe ziarniniakowatość z zapaleniem naczyń (EGPA), można wykryć specyficzne przeciwciała:12

  • Przeciwciała c-ANCA skierowane przeciwko proteinazie 3 (PR3) – do 80% lub więcej pacjentów z GPA ma obecne c-ANCA PR3
  • Przeciwciała p-ANCA skierowane przeciwko mieloperoksydazie (MPO) – często obecne w MPA i EGPA

Badanie moczu może wykryć uszkodzenie nerek, które często towarzyszy zapaleniu naczyń małych naczyń. Obecność krwi lub białka w moczu może wskazywać na zapalenie naczyń nerek.1

Badania obrazowe

Badania obrazowe mogą pokazać, które naczynia krwionośne i narządy są dotknięte chorobą. Mogą również pomóc specjaliście ocenić odpowiedź na leczenie.1

Najczęściej stosowane badania obrazowe w diagnostyce zapalenia naczyń to:123

  • Zdjęcia rentgenowskie – mogą wykazać, czy zapalenie naczyń wpływa na płuca, duże tętnice (np. aortę) lub tętnice płucne
  • Tomografia komputerowa (CT) – poszukuje oznak ziarniniakowatości z zapaleniem naczyń i innych typów zapaleń naczyń
  • Rezonans magnetyczny (MRI) – szczególnie przydatny w obrazowaniu zapalenia ścian naczyń
  • Pozytonowa tomografia emisyjna (PET) – wykrywa zwężenia i uszkodzenia naczyń krwionośnych
  • Ultrasonografia – poszukuje oznak zwężenia i uszkodzenia naczyń krwionośnych lub narządów
  • Angiografia – pomocna w diagnostyce guzkowego zapalenia tętnic (PAN), wykorzystuje kontrast wstrzykiwany do naczyń krwionośnych, aby uwidocznić przepływ krwi

W przypadku podejrzenia olbrzymiokomórkowego zapalenia tętnic (GCA), według zaktualizowanych wytycznych EULAR z 2023 roku, zaleca się wczesne badanie obrazowe w celu potwierdzenia diagnozy klinicznej. Ultrasonografia tętnic skroniowych i pachowych powinna być rozważana jako pierwsza metoda obrazowania w diagnostyce GCA. Alternatywnie można zastosować rezonans magnetyczny wysokiej rozdzielczości lub badanie PET.12

W podejrzeniu zapalenia tętnic Takayasu (TAK), jako pierwsze badanie diagnostyczne zaleca się rezonans magnetyczny w celu oceny zapalenia ścian naczyń lub zmian światła naczyń. Alternatywnie można zastosować badanie PET, CT lub ultrasonografię.1

Kryteria diagnostyczne

Obecnie nie istnieją powszechnie przyjęte standardy diagnostyczne dla zapaleń naczyń. W praktyce klinicznej najczęściej wykorzystywane są kryteria klasyfikacyjne Amerykańskiego Kolegium Reumatologicznego (ACR) oraz nomenklatura uzgodniona na konferencji Chapel Hill Consensus Conference.1

Ostateczne rozpoznanie zapalenia naczyń powinno być ustalone na podstawie charakterystycznych objawów i oznak zapalenia naczyń oraz co najmniej jednego z następujących elementów:12

  • Histologiczne dowody zapalenia naczyń
  • Pozytywny wynik badania serologicznego w kierunku przeciwciał ANCA
  • Specyficzne pośrednie dowody zapalenia naczyń

W przypadku zapalenia naczyń OUN (ośrodkowego układu nerwowego), proponuje się podział na dwie kategorie diagnostyczne: „pewne” zapalenie naczyń OUN, gdzie dostępne są dowody tkankowe, oraz „możliwe”, gdzie takich dowodów brak.12

Diagnostyka poszczególnych typów zapalenia naczyń

Zapalenie naczyń związane z ANCA (AAV)

Zapalenie naczyń związane z ANCA (AAV) jest grupą chorób zapalnych małych naczyń krwionośnych w organizmie. Jest spowodowane przez autoprzeciwciała zwane ANCA (przeciwciała przeciwko cytoplazmie neutrofilów).1

Do AAV należą trzy główne jednostki chorobowe:1

  • Mikroskopowe zapalenie naczyń (MPA)
  • Ziarniniakowatość z zapaleniem naczyń (GPA), dawniej ziarniniakowatość Wegenera
  • Eozynofilowe ziarniniakowatość z zapaleniem naczyń (EGPA), dawniej zespół Churga-Straussa

Chociaż test na obecność ANCA może być bardzo pomocny w wskazaniu diagnozy AAV, nie jest to test doskonały i sam w sobie nie może określić diagnozy.2 Diagnoza AAV opiera się zazwyczaj na połączeniu objawów klinicznych, wyników badań laboratoryjnych oraz biopsji zajętego narządu.1

Przeciwciała ANCA mogą mieć wzorzec cytoplazmatyczny (c-ANCA) lub okołojądrowy (p-ANCA). W przypadku GPA zazwyczaj wykrywa się c-ANCA skierowane przeciwko proteinazie 3 (PR3), natomiast w MPA i EGPA częściej występują p-ANCA skierowane przeciwko mieloperoksydazie (MPO).2

Olbrzymiokomórkowe zapalenie tętnic (GCA)

Olbrzymiokomórkowe zapalenie tętnic (GCA), znane również jako zapalenie tętnicy skroniowej, to rodzaj zapalenia naczyń, które głównie dotyka tętnic po bokach głowy (skroni).1

Diagnostyka GCA opiera się na charakterystycznych objawach klinicznych, takich jak:1

  • Gorączka, zmęczenie, utrata wagi
  • Ból głowy
  • Chromanie żuchwy
  • Przejściowa lub trwała utrata wzroku
  • Proksymalne bóle stawów i mięśni
  • Obrzęk dystalnych części kończyn

W badaniu fizykalnym można stwierdzić:2

  • Nieprawidłowe tętno
  • Nieprawidłowości tętnicy skroniowej (grube, guzkowe lub tkliwe gałęzie czołowe lub ciemieniowe powierzchownej tętnicy skroniowej)
  • Szmery naczyniowe
  • Szmer serca
  • Zmiany w badaniu dna oka

Zaleca się biopsję tętnicy skroniowej w celu rozpoznania GCA, podczas gdy w diagnostyce zapalenia tętnic Takayasu wymagane są badania obrazowe. Do diagnostyki GCA coraz częściej wykorzystuje się także ultrasonografię tętnic skroniowych i pachowych.12

Zapalenie tętnic Takayasu (TAK)

Zapalenie tętnic Takayasu to rodzaj zapalenia naczyń, który głównie dotyka młode kobiety.1

Pacjenci z zapaleniem tętnic Takayasu mają co najmniej trzy z następujących sześciu kryteriów:1

  • Wiek 40 lat lub młodszy w momencie początku choroby
  • Chromanie kończyn
  • Zmniejszone tętnienie jednej lub obu tętnic ramiennych
  • Różnica co najmniej 10 mmHg w ciśnieniu skurczowym krwi między ramionami
  • Szmer nad jedną lub obiema tętnicami podobojczykowymi lub aortą brzuszną
  • Zwężenie lub niedrożność całej aorty, jej głównych gałęzi lub dużych tętnic w proksymalnych częściach kończyn górnych lub dolnych w badaniu arteriograficznym

W diagnostyce zapalenia tętnic Takayasu zaleca się rezonans magnetyczny jako pierwszą metodę obrazowania w celu oceny zapalenia ścian naczyń lub zmian światła naczyń. Alternatywnie można wykorzystać badanie PET, CT lub ultrasonografię.1

Leukocytoklastyczne zapalenie naczyń (LCV)

Leukocytoklastyczne zapalenie naczyń (LCV) to opis histopatologiczny częstej formy zapalenia małych naczyń (SVV), który można znaleźć w różnych typach zapaleń naczyń dotyczących skóry i narządów wewnętrznych.1

Rozpoznanie opiera się na badaniu histopatologicznym, w którym naciek zapalny składa się z neutrofilów z martwicą włóknikoidalną i rozpadem jąder na fragmenty (leukocytoklazja).1

W przypadku podejrzenia LCV konieczne jest przeprowadzenie szczegółowych badań w celu ustalenia, czy proces jest ograniczony do skóry, czy też jest wyrazem układowego zapalenia naczyń lub choroby. Należy przeprowadzić dokładny wywiad i badanie fizykalne; badania obejmują liczbę płytek krwi, funkcję nerek i badanie moczu, testy serologiczne w kierunku wirusów zapalenia wątroby B i C, autoprzeciwciała (przeciwciała przeciwjądrowe i przeciwciała przeciwko cytoplazmie neutrofilów), frakcje dopełniacza oraz barwienie IgA w próbkach z biopsji.2

Zapalenie naczyń ośrodkowego układu nerwowego (CNS)

Zapalenie naczyń OUN wpływa na naczynia krwionośne w mózgu i rdzeniu kręgowym (ośrodkowy układ nerwowy). Zapalenie naczyń OUN może być trudne do zdiagnozowania.1

Rozpoznanie zapalenia naczyń OUN jest często trudne. Nie istnieją specyficzne cechy kliniczne ani klasyczny przebieg kliniczny, ani badania krwi czy obrazowe, które mogłyby potwierdzić diagnozę.1

Ostateczne rozpoznanie zapalenia naczyń OUN jest możliwe tylko za pomocą biopsji. W celu wykonania biopsji chirurg usuwa niewielką ilość tkanki z naczyń krwionośnych w mózgu lub rdzeniu kręgowym. Próbka biopsji jest następnie badana w laboratorium.1

Lekarze mogą przeprowadzić kilka badań w celu odkrycia przyczyny objawów pacjenta:12

  • Badania krwi: Zapalenie naczyń może powodować zmiany we krwi, takie jak niedokrwistość, zwiększenie liczby białych krwinek i zwiększenie poziomu substancji zaangażowanych w reakcje odpornościowe i zapalne.
  • Badanie płynu mózgowo-rdzeniowego: Płyn jest pobierany poprzez nakłucie lędźwiowe i badany pod kątem markerów zapalenia naczyń.
  • Rezonans magnetyczny (MRI): Duży magnes i fale radiowe są używane do tworzenia obrazów ciała, w tym tkanek miękkich, takich jak mózg, rdzeń kręgowy i naczynia krwionośne.
  • Angiografia mózgowa: Specjalista wstrzykuje barwnik do tętnic, co czyni je widocznymi na promieniach rentgenowskich, a następnie wykonuje serię zdjęć rentgenowskich w celu zbadania przepływu krwi.

Wyzwania i przyszłość diagnostyki zapalenia naczyń

Pomimo znacznego postępu w diagnostyce zapaleń naczyń, nadal istnieją wyzwania, które wymagają dalszych badań i rozwoju metod diagnostycznych.1

Opóźnienia diagnostyczne i ich konsekwencje

Pacjenci z zapaleniem naczyń często doświadczają znacznych opóźnień w uzyskaniu dokładnej diagnozy, co może prowadzić do istotnych powikłań zdrowotnych i zwiększonej śmiertelności.1

Badania wykazały, że:1

  • Średni czas do rozpoznania zapalenia naczyń wynosi 7 miesięcy
  • 73% pacjentów jest początkowo błędnie diagnozowanych
  • 82% pacjentów zgłasza, że opóźnienie diagnozy miało negatywne konsekwencje dla ich zdrowia

Do czynników związanych z dłuższym czasem do diagnozy należą: bezrobocie, czas dojazdu do ośrodka medycznego >1 godziny, początkowa błędna diagnoza oraz opóźnienia w konsultacji ze specjalistą.1

Nowe podejścia do diagnostyki

W świetle wyzwań diagnostycznych, opracowywane są nowe podejścia do rozpoznawania zapaleń naczyń. Badanie Diagnostic and Classification Criteria for Vasculitis (DCVAS) to międzynarodowe badanie obserwacyjne, które zostało zaprojektowane w celu opracowania kryteriów diagnostycznych dla pierwotnych zapaleń naczyń według wytycznych Amerykańskiego Kolegium Reumatologicznego (ACR) i Europejskiej Ligi Przeciwreumatycznej (EULAR).1

Zaktualizowane rekomendacje EULAR z 2023 roku dotyczące wykorzystania obrazowania w dużych zapaleniach naczyń podkreślają rolę nowoczesnych technik obrazowych, takich jak ultrasonografia, MRI i PET, w diagnozowaniu i monitorowaniu tych chorób.1

Również w diagnostyce ANCA wprowadzono nowe zrewidowane międzynarodowe zalecenia konsensusowe z 2017 roku, oparte na wynikach wieloośrodkowego projektu European Vasculitis Study Group (EUVAS), który oceniał dokładność diagnostyczną nowych technologii dostępnych do wykrywania przeciwciał PR3 i MPO-ANCA.1

Znaczenie zespołu wielodyscyplinarnego

Biorąc pod uwagę złożoność i różnorodność zapaleń naczyń, podejście wielodyscyplinarne ma kluczowe znaczenie w diagnozowaniu tych chorób.1

Ustalenie rozpoznania często wymaga wkładu wielu specjalistów, w tym reumatologów, nefrologów, patologów i radiologów. Nawet wtedy może być konieczne ponowne przyjrzenie się dowodom, aby dojść do prawdopodobnej diagnozy i wdrożyć plan terapeutyczny.1

Gdy pacjent nie reaguje na leczenie, ważne jest ponowne ocenienie obrazu klinicznego, a czasem nawet domniemanej diagnozy. Konieczna może być gotowość do ponownej oceny i współpracy między oddziałami w celu poprawy opieki nad pacjentem i wspólnego rozwiązania zagadki diagnostycznej.21

Algorytm diagnostyczny zapalenia naczyń

Poniżej przedstawiono praktyczne podejście krok po kroku do diagnostyki zapalenia naczyń:12

  1. Wywiad i badanie fizykalne
    • Zebranie dokładnego wywiadu medycznego
    • Szczegółowe badanie fizykalne ze zwróceniem uwagi na objawy ogólnoustrojowe i narządowe
  2. Badania laboratoryjne
    • Morfologia krwi z rozmazem
    • Badania biochemiczne, ze szczególną oceną funkcji nerek
    • Badanie moczu z mikroskopią
    • Markery zapalne (OB, CRP)
    • Przeciwciała ANCA (przeciwko PR3 i MPO)
    • Inne autoprzeciwciała (ANA, RF, anty-GBM)
    • Badania serologiczne w kierunku wirusowego zapalenia wątroby B i C
  3. Badania obrazowe
    • Zdjęcie rentgenowskie klatki piersiowej
    • Ultrasonografia zajętych obszarów
    • Tomografia komputerowa (CT) lub angiografia CT
    • Rezonans magnetyczny (MRI) lub angiografia MR
    • Pozytonowa tomografia emisyjna (PET)
  4. Biopsja tkanki
    • Biopsja zajętego narządu lub naczynia krwionośnego
    • Badanie histopatologiczne z oceną cech zapalenia naczyń
    • Immunofluorescencja bezpośrednia w celu wykrycia złogów immunoglobulin i dopełniacza
  5. Wykluczenie przyczyn wtórnych
    • Infekcje (bakteryjne, wirusowe)
    • Nowotwory
    • Choroby tkanki łącznej (np. toczeń rumieniowaty układowy)
    • Reakcje polekowe
  6. Określenie typu zapalenia naczyń
    • Na podstawie rozmiaru zajętych naczyń (duże, średnie, małe)
    • Na podstawie obecności specyficznych autoprzeciwciał
    • Na podstawie obrazu klinicznego i histopatologicznego

Podsumowanie znaczenia diagnostyki w zapaleniu naczyń

Wczesne rozpoznanie i leczenie zapalenia naczyń ma kluczowe znaczenie dla zapobiegania trwałym uszkodzeniom narządów i tkanek.11

Diagnostyka zapalenia naczyń jest często trudna ze względu na niespecyficzność objawów i podobieństwo do innych chorób. Kluczowe jest systematyczne podejście diagnostyczne obejmujące dokładny wywiad, badanie fizykalne, badania laboratoryjne, obrazowe oraz biopsję zajętej tkanki.11

Biopsja pozostaje złotym standardem w rozpoznawaniu zapalenia naczyń, jednak w przypadku niektórych typów zapaleń naczyń coraz większą rolę odgrywają nowoczesne techniki obrazowe, takie jak ultrasonografia, MRI i PET.12

Podejście wielodyscyplinarne, angażujące specjalistów z różnych dziedzin medycyny, ma fundamentalne znaczenie w diagnozowaniu i leczeniu zapaleń naczyń. Współpraca między reumatologami, nefrologami, pulmonologami, patologami, radiologami, kardiologami i neurologami umożliwia postawienie właściwej diagnozy i opracowanie odpowiedniego planu leczenia.11

Pomimo postępów w diagnostyce zapaleń naczyń, nadal istnieją wyzwania, które wymagają dalszych badań i rozwoju metod diagnostycznych. Dążenie do skrócenia czasu diagnostyki oraz zwiększenia jej dokładności pozostaje kluczowym celem w opiece nad pacjentami z zapaleniem naczyń.12

Kolejne rozdziały

Zapraszamy do dalszego czytania naszego leksykonu.

Wybierz kolejny rozdział z menu poniżej, aby otworzyć nową podstronę kompedium wiedzy i uzyskać szczegółowe informację o leku, substancji lub chorobie.

  1. 10.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Vasculitis – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/vasculitis/symptoms-causes/syc-20363435
    Vasculitis involves swelling and irritation, called inflammation, of blood vessels. […] Early diagnosis is the key to getting treatment that works.
  • #1 Diagnosing Vasculitis – Vasculitis UK
    https://www.vasculitis.org.uk/about-vasculitis/diagnosing-vasculitis
    It is essential to obtain a diagnosis of vasculitis as early as possible to enable appropriate treatment. Any delay may result in further permanent damage. The methods for diagnosing vasculitis vary depending on the disease concerned see Individual Diseases. […] However, it is important to realise that diagnosing vasculitis can be problematic for the physician, in part because most of these diseases are rare. Many patients develop symptoms which may be attributable to many diseases as any organ can be affected. The symptoms are often non-specific and often mimic other more common conditions. Unfortunately because of these difficulties diagnosis can be missed or delayed for some time. […] Making the right diagnosis will depend on the patients symptoms, what the doctor finds when examining the patient and often a combination of blood tests, x-rays (or other scans such as MRI and PET) and often a biopsy (taking a small piece of tissue) from an affected area. There is no single test for any of the types of vasculitis. […] Once the diagnosis has been made the doctor should discuss with the patient any treatment that is required. Most patients will require some treatment. In some cases of severe disease treatment needs to be given urgently; occasionally in very mild cases no treatment at all is required.
  • #1 Diagnostic delays in vasculitis and factors associated with time to diagnosis | Orphanet Journal of Rare Diseases | Full Text
    https://ojrd.biomedcentral.com/articles/10.1186/s13023-021-01794-5
    Patients with vasculitis, a set of rare diseases, encounter delays in obtaining an accurate diagnosis which can lead to substantial morbidity and increased mortality. […] The median time to diagnosis of vasculitis was 7 months. […] 313/456 (73%) of patients were misdiagnosed initially. […] Unemployment, time to travel to a medical center1 h, initial misdiagnosis, and delays in seeing a specialist were all associated with longer times to diagnosis. […] 373/456 (82%) of patients reported that a delayed diagnosis had negative consequences on their health. […] Patients with vasculitis encounter substantial delays in achieving an accurate diagnosis and these delays are associated with negative health consequences. […] The study aimed to gain a better understanding of the journey that patients with any form of vasculitis go through before receiving their diagnosis and identify factors associated with those delays. […] The results of this study illustrate that various factors, both patient-related and healthcare system-related, lead to delays in diagnosis for patients with vasculitis and that these delays can often have a negative impact on patient health and quality of life.
  • #1 Vasculitis syndrome—diagnosis and therapy
    https://pmc.ncbi.nlm.nih.gov/articles/PMC5689388/
    In patients with connective tissue disease, vascular injury induced by primary or secondary vasculitis syndromes can lead to organ dysfunction due to the loss of nutrient supply from the blood. […] The present review provides an overview of vasculitis syndromes from the viewpoint of diagnosis and treatment. […] The pathophysiologic basis of the clinical features of primary vasculitis is related to the fact that blood vessels supply oxygen to maintain the function of solid organs and the interstitium. […] With regard to diagnosis of primary vasculitis syndrome, it is important to systematize individual localized symptoms, which, at first glance, appear to be independent. […] The clinician should assess whether or not such individual localized symptoms are the results of ischemia caused by the inflammation of blood vessels or due to symptoms that arise as a result of bleeding.
  • #1 Vasculitis syndrome—diagnosis and therapy
    https://pmc.ncbi.nlm.nih.gov/articles/PMC5689388/
    Clinical features of primary vasculitis syndromes can be classified largely into two categories: systemic signs/symptoms caused by inflammation and localized visceral signs/symptoms specific to the affected organs. […] Signs/symptoms that could evoke the possibility of vasculitis syndrome are described as follows. […] The aforementioned systemic symptoms are frequently seen among elderly people with malignant tumors. Therefore, during the process of diagnosis for vasculitis syndrome, it is recommended to also conduct screening examinations for malignant tumors. […] Local symptoms are featured by the simultaneous (or sequential) appearance of symptoms from different affected organs in a patient with vasculitis syndrome. […] As large to medium-sized blood vessels run from the aorta to the organs, the signs/symptoms of vasculitis result from injury to organs supplied by the affected vessels.
  • #1 Diagnosing Vasculitis : Johns Hopkins Vasculitis Center
    https://www.hopkinsvasculitis.org/vasculitis/diagnosing-vasculitis/
    Patients with vasculitis learn that making the diagnosis is sometimes quite difficult. […] The diagnosis of vasculitis usually requires a biopsy of an involved organ (skin, kidney, lung, nerve, temporal artery). […] Blood tests, Xrays, and other studies may suggest the diagnosis of vasculitis, but often the only way to clinch the diagnosis is to biopsy involved tissue, examine the tissue under the microscope in consultation with a pathologist (ideally one experienced at examining biopsies in vasculitis), and find the pathologic hallmarks of the disease. […] If a patients symptoms, physical examination, and diagnostic testing suggest involvement of a particular organ, one of the procedures below may be used to confirm (or exclude) the diagnosis of vasculitis: […] A kidney biopsy will be performed if there is evidence of kidney involvement by vasculitis (red blood cells or protein in the urine, for example).
  • #1 Small-Vessel Vasculitis (Leukocytoclastic Vasculitis) Workup: Approach Considerations, Laboratory Studies, Imaging Studies
    https://emedicine.medscape.com/article/333891-workup
    A skin biopsy of a relatively new lesion should be performed in most adult patients with suspected SVV. The diagnostic yield is higher when performed on a fresh skin lesion, in the first 24 to 48 hours of development. Direct immunofluorescence (DIF) microscopy should be performed to assess for perivascular deposition of IgA; this is best done on lesions within 24 hours of development. […] Skin biopsy reveals the presence of vascular and perivascular infiltration of polymorphonuclear leukocytes with formation of nuclear dust (leukocytoclasis), extravasation of erythrocytes, and fibrinoid necrosis of the vessel walls. This process is dynamic, and biopsy of a lesion performed too early or too late in the evolution may not reveal the classic findings. Neutrophilia is common in skin biopsies of patients with cutaneous vasculitis associated with severe bacterial infection. […] Immunofluorescent staining may reveal immunoglobulins (eg, immunoglobulin G, immunoglobulin M) and complement components (eg, C3, C4) deposited on the skin basement membrane, suggesting immune complex deposition. In Henoch-Schnlein purpura, IgA-predominant deposits may be found.
  • #1 Vasculitis and Thrombophlebitis Workup: Laboratory Studies, Imaging Studies, End-Organ Imaging
    https://emedicine.medscape.com/article/1008239-workup
    Investigations must be performed to detect signs of inflammation, to determine the type and extent of organ involvement, to test for vasculitis-specific autoantibodies, and to rule out secondary causes. […] The initial test for antineutrophil cytoplasmic antibody (ANCA) is indirect immunofluorescence to detect staining pattern, either cytoplasmic or perinuclear. […] ANCA positivity is noted in approximately 90% of pediatric patients with granulomatosis with polyangiitis (GPA) (formerly Wegener granulomatosis), 79% of who are cytoplasmic. […] PR3-ANCA and MPO-ANCA positivity have a high sensitivity and specificity for the diagnosis of GPA and microscopic polyangiitis (MPA), respectively. […] Anti-glomerular basement membrane (GBM) antibody testing is indicated for pulmonary renal syndromes. […] Tissue biopsy may be necessary to confirm a diagnosis of vasculitis in systemic and cutaneous polyarteritis nodosa (PAN), ANCA vasculitides, childhood PACNS, and infrequently in Henoch-Schnlein purpura.
  • #1 Vasculitis – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/vasculitis/diagnosis-treatment/drc-20363485
    Diagnosis starts with a medical history and a physical exam. You may have one or more tests and procedures. These can either diagnose vasculitis or rule out other conditions that cause some of the same symptoms. Tests and procedures might include: […] Blood tests. These tests look for signs of inflammation, such as a high level of C-reactive protein. A complete blood cell count can tell whether you have enough red blood cells. Other blood tests, such as the antineutrophil cytoplasmic antibody (ANCA) test, can look for certain antibodies. These tests can help diagnose vasculitis. […] Imaging tests. Imaging tests can show which blood vessels and organs are affected. They also can help your healthcare professional know how you respond to treatment. Imaging tests for vasculitis include X-rays, ultrasounds, CT scans, MRIs and positron emission tomography (PET) scans. […] Biopsy. This is a surgery in which a healthcare professional removes a small sample of tissue from the affected area of your body. Your health professional then looks at the tissue for signs of vasculitis.
  • #1 Diagnosis of Vasculitis | Hospital Clínic Barcelona
    https://www.clinicbarcelona.org/en/assistance/diseases/vasculitis/diagnosis
    Different criteria have been established to help diagnosis each type of vasculitis. These criteria tend to include specific clinical manifestations (symptoms), altered laboratory values, changes in vascular characteristics observed during radiological tests, and tissue biopsy results revealing inflammation in the blood vessels (vasculitis). […] Blood tests. An increase in erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) levels and platelet levels, and a low haemoglobin level (anaemia) are usually common to all forms of vasculitis. […] Tests for the presence of antineutrophil cytoplasmic antibodies (ANCAs), rheumatoid factor (RF) and cryoglobulins. Other antibodies, such as antinuclear (ANA) and antiDNA antibodies, can be detected in vasculitides associated with other autoimmune diseases.
  • #1 Vasculitis Diagnostics
    https://www.diagnostic.grifols.com/en/-/learning/vasculitis-diagnostics
    Immunology tests: ANCA autoantibodies are a particularly useful indicator of ANCA-associated vasculitides (AAV) MPA, GPA, and EGPA. […] Up to 80% or more of patients with GPA will have the presence of c-ANCA PR3. […] The differences between disease associated with c-ANCA and that linked with p-ANCA have important implications for treatment. […] Imaging tests: Tests from outside the body such as computed tomography (CT), magnetic resonance imaging (MRI), positron emission tomography (PET), ultrasound, and x-rays are used to identify the blood vessels and organs affected by vasculitis. […] Biopsy: During this surgery, a small tissue sample is taken from affected areas of the body. The sample is then analyzed microscopically for any indication of vasculitis.
  • #1 Diagnosing Vasculitis | NYU Langone Health
    https://nyulangone.org/conditions/vasculitis/diagnosis
    NYU Langone rheumatologists use various diagnostic tools to help them diagnose vasculitis, which is an autoimmune disorder that causes inflammation of the blood vessels. […] To diagnose vasculitis, your doctor takes a medical history, asks about your signs and symptoms, conducts a physical exam, and orders laboratory tests. […] Because the disease can affect different organs, a rheumatologist may want to perform several diagnostic tests, based on your symptoms, to determine the best treatment plan for you. […] Your doctor may order a blood test to determine if you have medium or small vessel vasculitis, which can be caused by antineutrophil cytoplasmic antibodies. […] Blood tests reveal other markers of inflammation or the specific type of vasculitis causing your symptoms. […] Your doctor may ask for a urine sample to check for any abnormal levels of proteins or blood cells in the urine, which could be a sign of small vessel vasculitis in the kidneys. […] Angiography is helpful in identifying medium and large vessel vasculitis. […] An X-ray of your chest can show your doctor if vasculitis is affecting your heart, lungs, or large arteries.
  • #1 EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice: 2023 update | Annals of the Rheumatic Diseases
    https://ard.bmj.com/content/83/6/741
    Objectives To update the EULAR recommendations for the use of imaging modalities in primary large vessel vasculitis (LVV). […] A systematic literature review update was performed to retrieve new evidence on ultrasound, MRI, CT and [18F]-fluorodeoxyglucose positron emission tomography (FDG-PET) for diagnosis, monitoring and outcome prediction in LVV. […] The 2023 EULAR recommendations provide up-to-date guidance for the role of imaging in the diagnosis and assessment of patients with LVV. […] Imaging should not delay initiation of treatment. […] In patients with suspected GCA, an early imaging test is recommended to support the clinical diagnosis of GCA, assuming high expertise and prompt availability of the imaging technique. […] Ultrasound of temporal and axillary arteries should be considered as the first imaging modality to investigate mural inflammatory changes in patients with suspected GCA.
  • #1 EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice: 2023 update | Annals of the Rheumatic Diseases
    https://ard.bmj.com/content/83/6/741
    High-resolution MRI or FDG-PET can be used as alternatives to ultrasound for the assessment of cranial arteries in patients with suspected GCA. […] FDG-PET, alternatively MRI or CT, can be used for the detection of mural inflammation or luminal changes of extracranial arteries in patients with suspected GCA. […] In patients with suspected TAK, MRI to investigate mural inflammation or luminal changes should be used as the first imaging test to make a diagnosis of TAK. […] FDG-PET, CT or ultrasound may be used as alternative imaging modalities in patients with suspected TAK. […] In case of a suspected relapse, particularly when laboratory markers of disease activity are unreliable, ultrasound, FDG-PET or alternatively MRI may be considered for the assessment of vessel abnormalities. […] In patients with GCA or TAK, MRA, CTA or ultrasound of extracranial vessels may be used for long-term monitoring of structural damage, particularly at sites of preceding vascular inflammation.
  • #1 Systemic Vasculitis | AAFP
    https://www.aafp.org/pubs/afp/issues/2011/0301/p556.html
    The systemic vasculitides are characterized by inflammation of blood vessel walls. […] The heterogenous nature of vasculitides presents a diagnostic challenge. […] The diagnosis relies on the recognition of a compatible clinical presentation supported by specific laboratory or imaging tests and confirmatory histology. […] A definitive diagnosis of systemic vasculitis should be made by the presence of characteristic symptoms and signs of vasculitis and at least one of the following: histologic evidence of vasculitis, positive serology for antineutrophilic cytoplasmic antibody, or specific indirect evidence of vasculitis. […] There is no recommended diagnostic standard for vasculitis. In the absence of validated diagnostic criteria, the American College of Rheumatology classification criteria and the Chapel Hill Consensus Conference nomenclature are most widely used in clinical practice to distinguish different forms of vasculitis.
  • #1 The diagnosis of primary central nervous system vasculitis | Practical Neurology
    https://pn.bmj.com/content/20/2/109
    We suggest it would be valuable to divide patients into just two diagnostic groups according to the certainty of diagnosis. […] We propose simple draft diagnostic criteria delineating definite and possible primary CNS vasculitis. […] Primary CNS vasculitis has no pathognomic clinical picture. […] To aid the initial clinical suspicion and recognition of primary CNS vasculitis, three distinct presentations encompassing this wide diversity of clinical features were previously delineated. […] As with the clinical features, so for investigations: there are no biochemical, immunological or serological or imaging investigations that are diagnostic of primary CNS vasculitis. […] Therefore, rather than confirming primary CNS vasculitis, the main role of blood tests, CT scans of the chest, abdomen and pelvis, CSF examination, and MR scanning is to help exclude alternative inflammatory or autoimmune, infective, malignant or other disorders, not uncommonly identifying clinically occult systemic involvement and accessible targets for tissue biopsy.
  • #1 ANCA Vasculitis | UNC Kidney Center
    https://unckidneycenter.org/kidneyhealthlibrary/glomerular-disease/anca-vasculitis/
    ANCA vasculitis is a type of autoimmune disease that causes vasculitis. […] ANCA vasculitis is an autoimmune disease affecting small blood vessels in the body. It is caused by autoantibodies called ANCAs, or Anti-Neutrophilic Cytoplasmic Autoantibodies. […] ANCA vasculitis can cause different symptoms depending on what organ or part of the body that is involved. […] ANCA glomerulonephritis is the term we use when ANCA vasculitis has affected or involved the kidneys, and when this happens there is inflammation and swelling in the kidney filters. […] Doctors use a combination of tests as well as history and physical exam to diagnose ANCA vasculitis. […] Although a positive ANCA test can be very helpful in pointing to a diagnosis of ANCA vasculitis, it is not a perfect test and cannot determine the diagnosis alone.
  • #1 Vasculitis | Diagnosis & Disease Info – Rheumatology Advisor
    https://www.rheumatologyadvisor.com/ddi/vasculitis/
    Vasculitis Diagnosis Presentation […] Vasculitis is a heterogeneous set of diseases characterized by blood vessel inflammation. […] The 1994 International Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitides (CHCC1994) suggested names and definitions for common vasculitis types. […] According to the 2012 International Chapel Hill Consensus Conference, large vessel vasculitis includes takayasu arteritis and giant cell arteritis (GCA); medium vessel vasculitis includes polyarteritis nodosa (PAN) and Kawasaki disease; small vessel vasculitis includes antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), which is further grouped into microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and esosinophilic granulomatosis with polyangiitis (EGPA); and variable vessel vasculitis includes Behcet disease and Cogan syndrome.
  • #1 Classification, diagnosis and treatment of ANCA-associated vasculitis
    https://www.wjgnet.com/2220-3214/full/v5/i1/36.htm
    Diagnosis of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis is usually not difficult in patient with systemic disease, including lung and kidneys involvement, and laboratory signs of inflammation. The presence of ANCA and the results of histological investigation confirm diagnosis of ANCA-associated vasculitis. […] Though diagnostic criteria for systemic vasculitis are not established, ANCA-associated vasculitis can be usually suspected in patients with typical clinical manifestations, e.g., fever, joint pain, disease of upper and lower respiratory tract, kidney and other organs, and laboratory signs of inflammation (high ESR and C-reactive protein). […] ANCA-specificity has no decisive diagnostic value though PR3-ANCA are usually detected in GPA patients while MPO-ANCA are more common in MPA.
  • #1 Vasculitis
    https://www.nhs.uk/conditions/vasculitis/
    Vasculitis is the name of a group of conditions that cause inflammation of the blood vessels. […] There are many types of vasculitis. […] Eosinophilic granulomatosis with polyangiitis, also called Churg-Strauss syndrome, is a type of vasculitis that mainly affects adults around 38 to 54. […] Temporal arteritis, also known as giant cell arteritis, is a type of vasculitis where the arteries at the side of the head (the temples) become inflamed. […] Granulomatosis with polyangiitis, also called Wegener’s granulomatosis, is a type of vasculitis that affects blood vessels in the nose, sinuses, ears, lungs and kidneys. […] Henoch-Schnlein purpura is a type of vasculitis that can affect the skin, kidneys or bowel. […] Microscopic polyangiitis is a rare and potentially serious long-term type of vasculitis that most often develops in middle-aged people.
  • #1 Large-Vessel Vasculitis – Rheumatology Advisor
    https://www.rheumatologyadvisor.com/ddi/large-vessel-vasculitis/
    Large-vessel vasculitis symptoms specific to giant cell arteritis come on subacutely. They may include fever, fatigue, weight loss, headache, jaw claudication, transient visual loss, permanent vision loss, especially in cases with prior transient visual loss, proximal polyarthralgias and myalgias, peripheral synovitis, distal extremity swelling with pitting edema, aneurysm of affected vessels, stenosis, aortic aneurysm or dilatation, and other large artery involvement. […] Physical examination findings for suspected giant cell arteritis include abnormal pulses, temporal artery abnormalities, such as thick, nodular, or tender frontal or parietal branches of the superficial temporal arteries, bruits via auscultation, heart murmur, and ocular findings on fundoscopy. […] Biopsy of the temporal artery biopsy is recommended to diagnose giant cell arteritis, while imaging is required for Takayasu arteritis diagnosis. Positron emission tomography (PET) is often used in combination with computed tomography (PET-CT) or magnetic resonance (PET-MR) in the diagnosis of Takayasu arteritis.
  • #1 Vasculitis
    https://www.nhs.uk/conditions/vasculitis/
    Polyarteritis nodosa is a rare type of vasculitis that particularly affects the arteries supplying the gut, kidneys and nerves. […] Polymyalgia rheumatica is a type of vasculitis that’s closely related to temporal arteritis. […] Takayasu arteritis is a type of vasculitis that mainly affects young women. […] Behet’s disease typically causes mouth ulcers and genital ulcers. […] Buerger’s disease affects blood vessels in the legs and arms, leading to reduced blood flow to the hands and feet. […] Cogan’s syndrome is inflammation of the blood vessels in the inner ears and eyes. […] Cryoglobulin-associated vasculitis is caused by abnormal proteins in the blood called cryoglobulins. […] Hypersensitivity vasculitis is usually caused by a reaction to a medicine, such as NSAIDs or certain antibiotics, and results in a temporary rash. […] Primary angiitis of the central nervous system is inflammation of the blood vessels in the brain. […] Rheumatoid vasculitis is vasculitis associated with rheumatoid arthritis.
  • #1 Large-Vessel Vasculitis – Rheumatology Advisor
    https://www.rheumatologyadvisor.com/ddi/large-vessel-vasculitis/
    Patients with Takayasu arteritis have at least three of the following six criteria present. These include aged 40 or younger at disease onset, limb claudication, reduced pulsation of one or both brachial arteries, a difference of at least 10 mmHg in systolic blood pressure between the arms, bruit over one or both subclavian arteries or the abdominal aorta, and or arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities not due to arteriosclerosis, fibromuscular dysplasia, or other causes.
  • #1
    https://link.springer.com/article/10.1007/s11739-021-02688-x
    Leukocytoclastic vasculitis (LCV) is a histopathologic description of a common form of small vessel vasculitis (SVV), that can be found in various types of vasculitis affecting the skin and internal organs. […] The diagnosis relies on histopathological examination, in which the inflammatory infiltrate is composed of neutrophils with fibrinoid necrosis and disintegration of nuclei into fragments (leukocytoclasia). […] When LCV is suspected, an extensive workout is usually necessary to determine whether the process is skin-limited, or expression of a systemic vasculitis or disease. […] A comprehensive history and detailed physical examination must be performed; platelet count, renal function and urinalysis, serological tests for hepatitis B and C viruses, autoantibodies (anti-nuclear antibodies and anti-neutrophil cytoplasmic antibodies), complement fractions and IgA staining in biopsy specimens are part of the usual workout of LCV.
  • #1 CNS (Central Nervous System) Vasculitis: Causes & Treatment
    https://my.clevelandclinic.org/health/diseases/cns-vasculitis
    CNS vasculitis affects blood vessels in your brain and spinal cord (your central nervous system). […] A healthcare provider will diagnose CNS vasculitis with a physical exam and some tests. […] Diagnosing CNS vasculitis can be a differential diagnosis. This means your provider will probably use a few tests to rule out other, more common conditions before diagnosing you with CNS vasculitis. […] Markers are signs of a disease that your provider can look for in your blood or body using laboratory tests. […] Your provider will suggest treatments that manage inflammation. The most important part of treating CNS vasculitis is reducing how much it damages your brain or spinal cord. […] Monitor the symptoms youre experiencing and keep track of any changes you notice. […] Theres no cure for CNS vasculitis. Many people are able to enter extended periods of remission once they find the right medications that work for them. […] Talk to your healthcare provider if youre worried about your health or how much having CNS vasculitis can affect it.
  • #1 The diagnosis of primary central nervous system vasculitis | Practical Neurology
    https://pn.bmj.com/content/20/2/109
    The diagnosis of primary central nervous system (CNS) vasculitis is often difficult. There are neither specific clinical features nor a classical clinical course, and no blood or imaging investigations that can confirm the diagnosis. […] Here we describe an approach to its diagnosis, emphasising the importance of obtaining tissue, and present for discussion a new, binary set of diagnostic criteria, dividing cases into only definite primary CNS vasculitis, where tissue proof is available, and possible, where it is not. […] Diagnosing primary CNS vasculitis is, commonly, far more challenging, for several reasons. Its rarity means that few neurology units have extensive clinical experience of the disorder. […] There is no diagnostically distinct clinical picture. […] There are no fail-safe indirect diagnostic tests (including formal contrast/digital subtraction angiography; see further).
  • #1 Vasculitis Cns Diagnosis & Treatment – NYC | Columbia Neurosurgery in New York City
    https://www.neurosurgery.columbia.edu/patient-care/conditions/vasculitis-cns
    Vasculitis of the central nervous system (CNS) is the inflammation of blood vessels in the brain and spinal cord. […] A definitive diagnosis of CNS vasculitis is only possible with a biopsy. To perform a biopsy, a surgeon removes a small amount of tissue from blood vessels in the brain or spine. The biopsy sample is then studied in the laboratory. […] Early detection and treatment can be of great help to the patient with vasculitis. […] Doctors may perform several tests to discover the cause of a patients symptoms: […] Blood tests: Vasculitis may cause changes in the blood such as anemia; an increase in the number of white blood cells; and an increase in levels of substances involved with immune and inflammatory reactions. […] Examination of the cerebrospinal fluid: The fluid is taken through a spinal tap (sometimes called a lumbar puncture) and examined for markers of vasculitis.
  • #1 Classification, diagnosis and treatment of ANCA-associated vasculitis
    https://www.wjgnet.com/2220-3214/full/v5/i1/36.htm
    The current classification of ANCA-associated vasculitides may be revised in the future. […] The clinical trials also showed efficacy of rituximab that induces depletion of B-cells. Our understanding and management of ANCA-associated vasculitis improved significantly over the last decades but there is still a lot of debate over its classification, diagnostic criteria, assessment of activity and optimum treatment.
  • #1 Classification, diagnosis and treatment of ANCA-associated vasculitis
    https://www.wjgnet.com/2220-3214/full/v5/i1/36.htm
    The presence of ANCA that can be detected with immunofluorescence method or ELISA contributes significantly to the diagnosis of ANCA-associated vasculitis though these autoantibodies can be negative or disappear during immunosuppressive treatment. Biopsy (nose, lung, kidney, etc.) can be used to confirm the diagnosis of systemic vasculitis but histological study is not necessary for all patients. […] The criteria developed by the American College of Rheumatology (ACR) in 1990 can be used for classification of systemic vasculitides, while the categories that were defined in Chapel-Hill represent the nomenclature of these systemic diseases. […] The Diagnostic and Classification Criteria for Vasculitis (DCVAS) study is a multinational observational study that was designed to develop diagnostic criteria for primary systemic vasculitis according to the guidelines of the ACR and the European League against Rheumatism (EULAR).
  • #1 Significance of autoantibodies in diagnostics of systemic vasculitis
    https://www.explorationpub.com/Journals/emd/Article/100712
    Systemic vasculitis is a heterogeneous group of disorders characterized by inflammation and necrosis in the vessel wall. […] The diagnosis is based on clinical features, tissue biopsy, imaging investigations, and serologic tests. The main autoantibodies, important not only in the diagnosis but also in monitoring and prognosis of systemic vasculitides, are anti-neutrophil cytoplasmic antibodies (ANCA), anti-glomerular basement membrane antibodies (anti-GBM), anti-complement component C1q antibodies (anti-C1q), and cryoglobulins. […] The current review aimed to discuss the recent advances in the understanding of the role of ANCA as well as other autoantibodies in the diagnosis and management of the patients with systemic vasculitis. […] The new revised 2017 international consensus recommendations are based on the results of the multicenter European Vasculitis Study Group (EUVAS) project which evaluated the diagnostic accuracy of novel technologies available for PR3 and MPO-ANCA detection.
  • #1 Clinicopathologic Conference to review difficulties of vasculitis diagnosis – ACR Convergence Today
    https://www.acrconvergencetoday.org/clinicopathologic-conference-to-review-difficulties-of-vasculitis-diagnosis/
    Clinicopathologic Conference to review difficulties of vasculitis diagnosis. Vasculitis may seem straightforward, especially when you have access to tissue to help verify the diagnosis. Establishing a diagnosis often requires input from multiple specialties, including rheumatologists, nephrologists, pathologists, and radiologists. Even then, it might be necessary to take a second or a third look at the evidence to come up with a plausible diagnosis and institute a therapeutic plan. […] „When your patient isn’t responding to treatment, it’s important to reassess the clinical picture and sometimes even the presumptive diagnosis,” said Anisha Dua, MD, MPH, Assistant Professor of Rheumatology and Director of the Vasculitis Center at the University of Chicago. […] „When a patient is only partially responding or not significantly improving with treatment, we might be tempted to simply consider an escalation in immunosuppression,” Dr. Reid said. „What we really need to do is to step back and reassess the lens we have used to frame the clinical scenario. We should revisit and reexamine the entire history, starting from the first presentation, and not skip anything along the way.”
  • #1 Clinicopathologic Conference to review difficulties of vasculitis diagnosis – ACR Convergence Today
    https://www.acrconvergencetoday.org/clinicopathologic-conference-to-review-difficulties-of-vasculitis-diagnosis/
    The initial pulmonary and skin findings were, indeed, very important in the final diagnosis, Dr. Dua said. But clinical features alone were not enough to explain the patient’s disease course. It took the expertise of multiple specialists including radiology and pathology to re-evaluate the case and ultimately change the therapeutic plan. […] „Being willing and ready to re-evaluate is one of the take-homes from this case,” Dr. Reid said. „It highlights how we should collaborate between departments in order to improve patient care and work together to solve the diagnostic puzzle.”
  • #1 An Approach to Diagnosis and Initial Management of Systemic Vasculitis | AAFP
    https://www.aafp.org/pubs/afp/issues/1999/1001/p1421.html
    When systemic vasculitis is suspected, the diagnostic work-up can be approached in a stepwise fashion: Attempt to exclude other processes, particularly infection, thrombosis and neoplasia, that can cause secondary vasculitis or can have features that mimic vasculitis. […] The type and extent of organ involvement in vasculitis can be helpful in determining the specific type of vasculitis and the degree of urgency in initiating treatment. […] Attempt to distinguish the specific type of vasculitis on the basis of the above information and the pattern of the clinical features. […] Confirmation of a clinical suspicion of vasculitis usually requires arteriography, biopsy, or both. Evaluation should be directed toward establishing a tissue diagnosis, if possible. […] Although biopsy or angiography can be diagnostic for vasculitis, there are a number of potential pitfalls to remember.
  • #1 General Vasculitis – Vasculitis Foundation
    https://vasculitisfoundation.org/education/general-vasculitis/
    Diagnosing vasculitis can pose a challenge because the symptoms may be similar to those caused by other illnesses or diseases. Your doctor will take a detailed medical history and perform a physical exam. Depending on your symptoms and the type of vasculitis suspected, your doctor may order laboratory tests such as urinalysis and blood tests, imaging studies such as X-rays, computed tomography (CT) or magnetic resonance imaging (MRI) scans, or lung function tests. A biopsy of the affected tissue or organ is usually obtained to confirm diagnosis but is not always feasible. Biopsy is not always required to confirm the diagnosis before starting treatment, which shouldn’t be delayed. […] Early diagnosis and treatment are extremely important to avoid potentially life-threatening complications.
  • #1 Vasculitis: Approach to diagnosis and therapy – Indian Journal of Dermatology, Venereology and Leprology
    https://ijdvl.com/vasculitis-approach-to-diagnosis-and-therapy/
    Vasculitides is a group of disorders characterized by inflammation of vessel walls. The unique feature of this group is multi-organ involvement. […] Hence a systematic approach is required for diagnosis and management of these multi-system disorders. […] In the following section, a diagnostic and therapeutic approach to vasculitis with cutaneous involvement relevant to the dermatologists has been discussed. […] When encountered with a patient having cutaneous features of vasculitis, the initial attempt should be to assess the extent of systemic involvement. […] The extent and severity of systemic involvement determines the prognosis of the disease. Moreover, the diagnostic and therapeutic approach to the patient is dependent on this. […] Thorough history and physical examination of patients help in initial clinical diagnosis. Criteria for diagnosis of both cutaneous and systemic vasculitides proposed by American College of Rheumatology (ACR, 1990) may be used for categorization to the type of vasculitis. […] Laboratory tests are required for confirmation of the clinical diagnosis, screening for underlying organ involvement and occasionally, to assess the risk factors, prognosis or response to treatment. […] Vasculitis is a clinico-pathological diagnosis and microscopic evidence of vascular inflammation is confirmatory. […] Histopathological study of skin biopsy is confirmatory, while DIF study is additive to this and helps to categorize the vasculitis.
  • #1 The diagnosis of primary central nervous system vasculitis | Practical Neurology
    https://pn.bmj.com/content/20/2/109
    Given the lack of alternative methods of achieving a secure diagnosis, it would appear axiomatic that CNS tissue biopsy is required to diagnose primary CNS vasculitis. […] In our view, a number of considerations, combined with a growing body of observational research evidence, very strongly tilt the balance in favour of biopsy. […] We therefore propose simple, readily applied binary diagnostic criteria: 'possible’ or 'definite’ primary CNS vasculitis. […] We propose that there is no probable category, given the low specificity of contrast angiography. Rather, we suggest that all suspected cases lacking histological proof should be described as possible and the role of angiography therefore implicitly restricted to excluding other specific disorders.
  • #1 Vasculitis Diagnosis & Treatment | Mount Sinai – New York
    https://www.mountsinai.org/care/rheumatology/services/vasculitis
    Vasculitis is a group of rare diseases that cause inflammation of the blood vessels. […] The diagnosis of vasculitis requires imaging tests, such as computed tomography, angiography, and magnetic resonance imaging and often a tissue sample (biopsy). […] At Mount Sinai, we work with teams of nephrologists, pulmonologists, pathologists, radiologists, cardiologists, neurologists, and rheumatologists to diagnose and develop an appropriate treatment.
  • #2 Advocate for Prompt Vasculitis Diagnosis & Treatment – The Rheumatologist
    https://www.the-rheumatologist.org/article/advocate-for-prompt-vasculitis-diagnose-treatment/
    As experts who diagnose, treat and monitor complications of multisystem autoimmune diseases, rheumatologists are uniquely positioned to play a crucial role in caring and advocating for patients with vasculitis, according to Tanaz Kermani, MD, MS, a rheumatologist and director of the Vasculitis Program at UCLA in Los Angeles. […] These rapidly progressive conditions require prompt diagnosis and treatment to prevent devastating consequences for our patients, Dr. Kermani says. […] Rheumatologists play an important role in the early detection of vasculitis and in ensuring appropriate work-up. […] Prompt diagnosis and treatment are the cornerstone to minimizing damage from vasculitis, Dr. Kermani stresses. […] The most important thing we can do is raise awareness of these signs and symptoms with other health professionals so they consider a vasculitis diagnosis and make a referral early, Dr. Kermani says. […] The ultimate goal from the advocacy perspective is to ensure better recognition of these conditions to enable earlier diagnosis and treatment.
  • #2 Diagnosing Vasculitis : Johns Hopkins Vasculitis Center
    https://www.hopkinsvasculitis.org/vasculitis/diagnosing-vasculitis/
    Patients with vasculitis learn that making the diagnosis is sometimes quite difficult. […] The diagnosis of vasculitis usually requires a biopsy of an involved organ (skin, kidney, lung, nerve, temporal artery). […] Blood tests, Xrays, and other studies may suggest the diagnosis of vasculitis, but often the only way to clinch the diagnosis is to biopsy involved tissue, examine the tissue under the microscope in consultation with a pathologist (ideally one experienced at examining biopsies in vasculitis), and find the pathologic hallmarks of the disease. […] If a patients symptoms, physical examination, and diagnostic testing suggest involvement of a particular organ, one of the procedures below may be used to confirm (or exclude) the diagnosis of vasculitis: […] A kidney biopsy will be performed if there is evidence of kidney involvement by vasculitis (red blood cells or protein in the urine, for example).
  • #2 Vasculitis syndrome—diagnosis and therapy
    https://pmc.ncbi.nlm.nih.gov/articles/PMC5689388/
    Clinical features of primary vasculitis syndromes can be classified largely into two categories: systemic signs/symptoms caused by inflammation and localized visceral signs/symptoms specific to the affected organs. […] Signs/symptoms that could evoke the possibility of vasculitis syndrome are described as follows. […] The aforementioned systemic symptoms are frequently seen among elderly people with malignant tumors. Therefore, during the process of diagnosis for vasculitis syndrome, it is recommended to also conduct screening examinations for malignant tumors. […] Local symptoms are featured by the simultaneous (or sequential) appearance of symptoms from different affected organs in a patient with vasculitis syndrome. […] As large to medium-sized blood vessels run from the aorta to the organs, the signs/symptoms of vasculitis result from injury to organs supplied by the affected vessels.
  • #2 Vasculitis – Diagnosis | NHLBI, NIH
    https://www.nhlbi.nih.gov/health/vasculitis/diagnosis
    Your healthcare provider may be able to diagnose the type of vasculitis that you have and how serious it is. Depending on your symptoms, your provider may recommend that you see a specialist for more tests or procedures. […] Diagnosis of vasculitis can be difficult. Some types of vasculitis cannot be diagnosed with a test. Instead, your healthcare provider will diagnose you based on your symptoms or order specific procedures. […] A biopsy collects a small sample of your tissue from a specific blood vessel or an organ. A pathologist, someone with special training in laboratory results, will study the sample for specific signs of tissue damage. […] Blood tests detect levels of certain blood cells and antibodies in your blood. […] A chest X-ray finds out whether vasculitis is affecting your lungs, your large arteries, such as the aorta, or your lung arteries.
  • #2 Vasculitis – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/vasculitis/diagnosis-treatment/drc-20363485
    Diagnosis starts with a medical history and a physical exam. You may have one or more tests and procedures. These can either diagnose vasculitis or rule out other conditions that cause some of the same symptoms. Tests and procedures might include: […] Blood tests. These tests look for signs of inflammation, such as a high level of C-reactive protein. A complete blood cell count can tell whether you have enough red blood cells. Other blood tests, such as the antineutrophil cytoplasmic antibody (ANCA) test, can look for certain antibodies. These tests can help diagnose vasculitis. […] Imaging tests. Imaging tests can show which blood vessels and organs are affected. They also can help your healthcare professional know how you respond to treatment. Imaging tests for vasculitis include X-rays, ultrasounds, CT scans, MRIs and positron emission tomography (PET) scans. […] Biopsy. This is a surgery in which a healthcare professional removes a small sample of tissue from the affected area of your body. Your health professional then looks at the tissue for signs of vasculitis.
  • #2 Vasculitis – Manchester University NHS Foundation Trust
    https://mft.nhs.uk/the-trust/other-departments/laboratory-medicine/immunology/clinical-conditions-covered-by-the-immunology-department/vasculitis/
    The term vasculitis refers to inflammation of blood vessels and represents a heterogeneous group of clinical disorders. […] Each form of vasculitis can produce a distinct clinical picture, but in many cases immunology testing can differentiate, confirm and monitor the presence of vasculitis. […] The main immunological tests are Antinuclear antibody (ANA for SLE a connective tissue diseases) and Anti-neutrophil cytoplasmic antibody (ANCA Wegeners granulomatosis and other vasculitides). […] General screening tests for vasculitis should also include serum immunoglobulins, and ANCA. […] Some forms of systemic vasculitis are strongly associated with circulating anti-neutrophil cytoplasmic antigens (ANCA). […] In Wegeners Granulomatosis, (WG) (lung, renal) there is a diffuse cytoplasmic pattern (c-ANCA), as well as polyclonal elevations of IgG, IgA, IgE and raised CRP.
  • #2 Vasculitis – Diagnosis | NHLBI, NIH
    https://www.nhlbi.nih.gov/health/vasculitis/diagnosis
    A computed tomography (CT) scan looks for signs of granulomatosis with polyangiitis. […] Echocardiography is an ultrasound test to learn how well the heart is working. […] A pathergy test diagnoses Behets disease. In this test, a needle pricks the skin, and sometimes a small amount of saline solution may be injected. The test is positive if a red bump or ulcer develops after 2 days. […] CT coronary angiography looks at your blood vessels for damage, signs of inflammation, blockages, or aneurysms. […] Positron electron tomography (PET) scan, a type of nuclear scan, detects narrowing and damage in the blood vessels. […] Ultrasound looks for signs of narrowing and damage in your blood vessels or organs. […] Urinalysis checks for kidney damage. […] Fluorescein retinal angiography looks for signs of retinal vasculitis in the eyes.
  • #2 EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice: 2023 update | Annals of the Rheumatic Diseases
    https://ard.bmj.com/content/83/6/741
    High-resolution MRI or FDG-PET can be used as alternatives to ultrasound for the assessment of cranial arteries in patients with suspected GCA. […] FDG-PET, alternatively MRI or CT, can be used for the detection of mural inflammation or luminal changes of extracranial arteries in patients with suspected GCA. […] In patients with suspected TAK, MRI to investigate mural inflammation or luminal changes should be used as the first imaging test to make a diagnosis of TAK. […] FDG-PET, CT or ultrasound may be used as alternative imaging modalities in patients with suspected TAK. […] In case of a suspected relapse, particularly when laboratory markers of disease activity are unreliable, ultrasound, FDG-PET or alternatively MRI may be considered for the assessment of vessel abnormalities. […] In patients with GCA or TAK, MRA, CTA or ultrasound of extracranial vessels may be used for long-term monitoring of structural damage, particularly at sites of preceding vascular inflammation.
  • #2 Systemic Vasculitis | AAFP
    https://www.aafp.org/pubs/afp/issues/2011/0301/p556.html
    The primary systemic vasculitides are difficult to diagnose because the clinical manifestations mimic several infectious, neoplastic, and autoimmune conditions. […] A definitive diagnosis of systemic vasculitis should be made by the presence of characteristic symptoms and signs of vasculitis and at least one of the following: histologic evidence of vasculitis; positive serology for ANCA; or specific indirect evidence of vasculitis. […] Laboratory testing is important to determine the organs involved, to exclude other diseases, and to monitor the disease and treatment toxicity. […] The definitive diagnosis of vasculitis is established by biopsy of the involved tissue (e.g., skin, sinuses, lung, artery, nerve, kidney), which determines the pattern of vessel inflammation.
  • #2 The diagnosis of primary central nervous system vasculitis | Practical Neurology
    https://pn.bmj.com/content/20/2/109
    Given the lack of alternative methods of achieving a secure diagnosis, it would appear axiomatic that CNS tissue biopsy is required to diagnose primary CNS vasculitis. […] In our view, a number of considerations, combined with a growing body of observational research evidence, very strongly tilt the balance in favour of biopsy. […] We therefore propose simple, readily applied binary diagnostic criteria: 'possible’ or 'definite’ primary CNS vasculitis. […] We propose that there is no probable category, given the low specificity of contrast angiography. Rather, we suggest that all suspected cases lacking histological proof should be described as possible and the role of angiography therefore implicitly restricted to excluding other specific disorders.
  • #2 ANCA Vasculitis | UNC Kidney Center
    https://unckidneycenter.org/kidneyhealthlibrary/glomerular-disease/anca-vasculitis/
    ANCA vasculitis is a type of autoimmune disease that causes vasculitis. […] ANCA vasculitis is an autoimmune disease affecting small blood vessels in the body. It is caused by autoantibodies called ANCAs, or Anti-Neutrophilic Cytoplasmic Autoantibodies. […] ANCA vasculitis can cause different symptoms depending on what organ or part of the body that is involved. […] ANCA glomerulonephritis is the term we use when ANCA vasculitis has affected or involved the kidneys, and when this happens there is inflammation and swelling in the kidney filters. […] Doctors use a combination of tests as well as history and physical exam to diagnose ANCA vasculitis. […] Although a positive ANCA test can be very helpful in pointing to a diagnosis of ANCA vasculitis, it is not a perfect test and cannot determine the diagnosis alone.
  • #2 Classification, diagnosis and treatment of ANCA-associated vasculitis
    https://www.wjgnet.com/2220-3214/full/v5/i1/36.htm
    Diagnosis of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis is usually not difficult in patient with systemic disease, including lung and kidneys involvement, and laboratory signs of inflammation. The presence of ANCA and the results of histological investigation confirm diagnosis of ANCA-associated vasculitis. […] Though diagnostic criteria for systemic vasculitis are not established, ANCA-associated vasculitis can be usually suspected in patients with typical clinical manifestations, e.g., fever, joint pain, disease of upper and lower respiratory tract, kidney and other organs, and laboratory signs of inflammation (high ESR and C-reactive protein). […] ANCA-specificity has no decisive diagnostic value though PR3-ANCA are usually detected in GPA patients while MPO-ANCA are more common in MPA.
  • #2 Large-Vessel Vasculitis – Rheumatology Advisor
    https://www.rheumatologyadvisor.com/ddi/large-vessel-vasculitis/
    Large-vessel vasculitis symptoms specific to giant cell arteritis come on subacutely. They may include fever, fatigue, weight loss, headache, jaw claudication, transient visual loss, permanent vision loss, especially in cases with prior transient visual loss, proximal polyarthralgias and myalgias, peripheral synovitis, distal extremity swelling with pitting edema, aneurysm of affected vessels, stenosis, aortic aneurysm or dilatation, and other large artery involvement. […] Physical examination findings for suspected giant cell arteritis include abnormal pulses, temporal artery abnormalities, such as thick, nodular, or tender frontal or parietal branches of the superficial temporal arteries, bruits via auscultation, heart murmur, and ocular findings on fundoscopy. […] Biopsy of the temporal artery biopsy is recommended to diagnose giant cell arteritis, while imaging is required for Takayasu arteritis diagnosis. Positron emission tomography (PET) is often used in combination with computed tomography (PET-CT) or magnetic resonance (PET-MR) in the diagnosis of Takayasu arteritis.
  • #2 EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice: 2023 update | Annals of the Rheumatic Diseases
    https://ard.bmj.com/content/83/6/741
    Objectives To update the EULAR recommendations for the use of imaging modalities in primary large vessel vasculitis (LVV). […] A systematic literature review update was performed to retrieve new evidence on ultrasound, MRI, CT and [18F]-fluorodeoxyglucose positron emission tomography (FDG-PET) for diagnosis, monitoring and outcome prediction in LVV. […] The 2023 EULAR recommendations provide up-to-date guidance for the role of imaging in the diagnosis and assessment of patients with LVV. […] Imaging should not delay initiation of treatment. […] In patients with suspected GCA, an early imaging test is recommended to support the clinical diagnosis of GCA, assuming high expertise and prompt availability of the imaging technique. […] Ultrasound of temporal and axillary arteries should be considered as the first imaging modality to investigate mural inflammatory changes in patients with suspected GCA.
  • #2
    https://link.springer.com/article/10.1007/s11739-021-02688-x
    Leukocytoclastic vasculitis (LCV) is a histopathologic description of a common form of small vessel vasculitis (SVV), that can be found in various types of vasculitis affecting the skin and internal organs. […] The diagnosis relies on histopathological examination, in which the inflammatory infiltrate is composed of neutrophils with fibrinoid necrosis and disintegration of nuclei into fragments (leukocytoclasia). […] When LCV is suspected, an extensive workout is usually necessary to determine whether the process is skin-limited, or expression of a systemic vasculitis or disease. […] A comprehensive history and detailed physical examination must be performed; platelet count, renal function and urinalysis, serological tests for hepatitis B and C viruses, autoantibodies (anti-nuclear antibodies and anti-neutrophil cytoplasmic antibodies), complement fractions and IgA staining in biopsy specimens are part of the usual workout of LCV.
  • #2 Vasculitis Cns Diagnosis & Treatment – NYC | Columbia Neurosurgery in New York City
    https://www.neurosurgery.columbia.edu/patient-care/conditions/vasculitis-cns
    Magnetic resonance imaging (MRI): A large magnet and radio waves are used to produce images of the body, including of soft tissues like the brain, spinal cord, and blood vessels. […] Cerebral angiography: A specialist injects dye into the arteries, which makes them visible on X-ray and then takes a series of X-rays to study the flow of blood.
  • #2 Clinicopathologic Conference to review difficulties of vasculitis diagnosis – ACR Convergence Today
    https://www.acrconvergencetoday.org/clinicopathologic-conference-to-review-difficulties-of-vasculitis-diagnosis/
    Clinicopathologic Conference to review difficulties of vasculitis diagnosis. Vasculitis may seem straightforward, especially when you have access to tissue to help verify the diagnosis. Establishing a diagnosis often requires input from multiple specialties, including rheumatologists, nephrologists, pathologists, and radiologists. Even then, it might be necessary to take a second or a third look at the evidence to come up with a plausible diagnosis and institute a therapeutic plan. […] „When your patient isn’t responding to treatment, it’s important to reassess the clinical picture and sometimes even the presumptive diagnosis,” said Anisha Dua, MD, MPH, Assistant Professor of Rheumatology and Director of the Vasculitis Center at the University of Chicago. […] „When a patient is only partially responding or not significantly improving with treatment, we might be tempted to simply consider an escalation in immunosuppression,” Dr. Reid said. „What we really need to do is to step back and reassess the lens we have used to frame the clinical scenario. We should revisit and reexamine the entire history, starting from the first presentation, and not skip anything along the way.”
  • #2 Frontiers | Cutaneous vasculitis; An algorithmic approach to diagnosis
    https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2022.1012554/full
    When histopathology is consistent with vasculitis, leukocytoclastic vasculitis is the most commonly observed histopathological appearance. […] The following step after the diagnosis of vasculitis should be the investigation of etiological causes. […] All patients with vasculitis are evaluated as a laboratory to detect the underlying cause and possible systemic involvement. […] The algorithmic approach given here is intended to aid in the diagnosis. […] Stepwise, algorithmic diagnosis of vasculitis is summarized in Figure 1. […] Dermatologists are in a privileged position in the recognition and early diagnosis of cutaneous vasculitis because of the high prevalence of cutaneous involvement in vasculitis and the easy accessibility of the skin. […] Any type of skin lesions resembling vasculitis, including palpable or non-palpable purpura, edematous papule, plaque, ulcers, nodules, pitted scars, white atrophy, livedo racemosa, requires a skin biopsy which is the gold standard for the diagnosis of cutaneous vasculitis.
  • #3 Diagnosing Vasculitis : Johns Hopkins Vasculitis Center
    https://www.hopkinsvasculitis.org/vasculitis/diagnosing-vasculitis/
    The sural nerve is a sensory nerve over the lateral aspect of the foot. […] Performed to diagnose Giant Cell Arteritis, also known as Temporal Arteritis, because the temporal artery is often involved. […] A lung biopsy may be performed in one of two ways: 1) open lung biopsy, a sizable surgical procedure; or 2) thoracoscopic lung biopsy, a less invasive but still significant procedure. […] Often necessary to confirm the diagnosis of Central Nervous System (CNS) Vasculitis. […] Angiography is helpful in the diagnosis of Polyarteritis Nodosa (PAN). […] Frequently part of the workup of CNS vasculitis. […] There are many other tests that are helpful in the diagnosis of vasculitis, or in evaluating the activity of the disease: […] A simple way of determining whether or not the kidneys are involved is to perform a urinalysis. […] ANCA is an abbreviation (acronym) for antineutrophil cytoplasmic antibodies. […] Because of the long list of other conditions that are sometimes associated with ANCAs, however, in most cases it is still VERY IMPORTANT to biopsy an organ involved by vasculitis to verify the diagnosis.
  • #3 Vasculitis Diagnostics
    https://www.diagnostic.grifols.com/en/-/learning/vasculitis-diagnostics
    Vasculitis is defined as inflammation of the blood vessels. […] A number of tests can be used to diagnose vasculitis types, and treatment usually involves two phases: controlling the inflammation to achieve remission, and maintenance treatment to prevent relapse. […] Diagnosing vasculitides is difficult, partly because the disorders are so rare. […] Numerous tests and assessments are often needed before a definite diagnosis of vasculitis can be made. […] The tests and assessments used to diagnose vasculitides vary according to the specific type of vasculitis involved. […] The doctor will initially take a medical history and conduct a physical exam. Then, any or all of the following tests and assessments may be needed to confirm or deny the presence of vasculitis: […] Blood tests: Protein levels (e.g., C-reactive protein) in the blood can indicate inflammation.
  • #3 Vasculitis Diagnostics
    https://www.diagnostic.grifols.com/en/-/learning/vasculitis-diagnostics
    Immunology tests: ANCA autoantibodies are a particularly useful indicator of ANCA-associated vasculitides (AAV) MPA, GPA, and EGPA. […] Up to 80% or more of patients with GPA will have the presence of c-ANCA PR3. […] The differences between disease associated with c-ANCA and that linked with p-ANCA have important implications for treatment. […] Imaging tests: Tests from outside the body such as computed tomography (CT), magnetic resonance imaging (MRI), positron emission tomography (PET), ultrasound, and x-rays are used to identify the blood vessels and organs affected by vasculitis. […] Biopsy: During this surgery, a small tissue sample is taken from affected areas of the body. The sample is then analyzed microscopically for any indication of vasculitis.