Zespół antyfosfolipidowy – Rokowania, prognozy i postęp choroby

Zespół antyfosfolipidowy
Rokowania, prognozy i postęp choroby

Rokowanie w zespole antyfosfolipidowym (APS) jest zróżnicowane i zależy od profilu przeciwciał, współistniejących chorób oraz leczenia. W dużych europejskich kohortach 10-letnie przeżycie wynosi około 90-94%. Profil potrójnie dodatni (obecność antykoagulantu toczniowego (LA) oraz średnich/wysokich mian przeciwciał antykardiolipinowych (aCL) i przeciwciał anty-β2GPI powyżej 99. percentyla) jest najsilniejszym predyktorem nawrotów i manifestacji klinicznych, z ryzykiem nawrotu HR 1,63 (95% CI 1,04-2,55; p=0,031) oraz względnym ryzykiem zakrzepicy wynoszącym 33. Wskaźniki przeżycia bez zdarzeń klinicznych wynoszą 92,6% po 1 roku, 85,2% po 3 latach i 79,8% po 5 latach. U pacjentek z APS i toczniem rumieniowatym układowym (SLE) przeciwciała antyfosfolipidowe są powiązane z chorobą neuropsychiatryczną i nieodwracalnym uszkodzeniem narządów.

Zespół antyfosfolipidowy (APS) – Rokowanie

Wskaźniki przeżycia
Czynniki ryzyka nawrotów
Czynniki predykcyjne zdarzeń zakrzepowych
Przeżycie bez zdarzeń

Rokowanie w połączeniu z ciążą

Czynniki genetyczne w położniczym APS
Score aGAPSS w przewidywaniu wyników położniczych
Zmiany łożyskowe w APS

Różnice między naczyniowym a położniczym APS
Znaczenie kliniczne różnych profili przeciwciał
Rokowanie długoterminowe

Kolejne rozdziały

Zespół antyfosfolipidowy (APS) – Rokowanie

Rokowanie w zespole antyfosfolipidowym (APS) jest zróżnicowane i zależy od wielu czynników, w tym profilu przeciwciał, współistniejących chorób oraz zastosowanego leczenia. Prawidłowo prowadzona terapia pozwala większości pacjentów z pierwotnym APS prowadzić normalne, zdrowe życie, jednak u niektórych osób choroba może mieć przebieg ciężki, prowadzący do znacznej chorobowości lub wczesnej śmierci.¹

Wskaźniki przeżycia

W dużych europejskich badaniach kohortowych 10-letnie przeżycie w APS wynosi około 90-94%.² Rokowanie w APS wtórnym jest podobne do pierwotnego APS, jednak w tym przypadku chorobowość i śmiertelność mogą być również zależne od współistniejącej choroby autoimmunologicznej lub reumatycznej.³ U pacjentów z toczniem rumieniowatym układowym (SLE) i APS, przeciwciała antyfosfolipidowe zostały powiązane z chorobą neuropsychiatryczną i uznane za główny czynnik predykcyjny nieodwracalnego uszkodzenia narządów.⁴

Czynniki ryzyka nawrotów

Profil przeciwciał pacjenta ma istotny wpływ na ryzyko nawrotów i powikłań. Wykazano, że profil potrójnie dodatni (obecność antykoagulantu toczniowego (LA) oraz średnich/wysokich mian przeciwciał antykardiolipinowych (aCL) i przeciwciał przeciwko β2-glikoproteinie I (anty-β2GPI) powyżej 99. percentyla) jest najsilniejszym czynnikiem predykcyjnym manifestacji klinicznych i nawrotów pomimo konwencjonalnego leczenia.⁵⁶

W badaniu obejmującym 204 pacjentów, z których 68 było potrójnie dodatnich, a 136 pojedynczo lub podwójnie dodatnich, wskaźnik nawrotów był znacząco wyższy u pacjentów potrójnie dodatnich w porównaniu z pozostałymi (63,2% vs 39,7%, p=0,002). W analizie wieloczynnikowej profil potrójnie dodatni był związany z wyższym ryzykiem nawrotu (HR 1,63; 95% CI 1,04-2,55; p=0,031).⁷ Pacjenci z potrójnie dodatnim profilem stanowią szczególny podtyp APS z wysokim ryzykiem nawrotu i potrzebą dodatkowych terapii.⁸

Profil potrójnie dodatni wydaje się być najważniejszym czynnikiem ryzyka zakrzepicy, z ryzykiem względnym wynoszącym 33, w porównaniu z ryzykiem względnym wynoszącym 4 i 3 odpowiednio dla LA i anty-β2GPI.⁹

Czynniki predykcyjne zdarzeń zakrzepowych

Badanie retrospektywne sugerowało, że nadciśnienie tętnicze lub średnio-wysokie miana przeciwciał antykardiolipinowych klasy IgG są czynnikami ryzyka pierwszego zdarzenia zakrzepowego u bezobjawowych pacjentów z przeciwciałami antyfosfolipidowymi.¹⁰ Zdarzenia kliniczne są silniej związane z przeciwciałami antyfosfolipidowymi klasy IgG, natomiast izolowana obecność aCL lub anty-β2GPI klasy IgM jest rzadko wykrywana w kohortach APS.¹¹

Niedawne badania wykazały, że pacjenci z wieloma dodatnimi wynikami testów (tj. LA, aCL i przeciwciała anty-β2GPI, szczególnie klasy IgG) wykazują znacznie wyższe ryzyko powikłań klinicznych.¹² Wysokie poziomy przeciwciał o porównywalnej swoistości i wykryte za pomocą zestawu badawczego ELISA zostały ostatnio powiązane ze zwiększonym ryzykiem zdarzeń zakrzepowych w prospektywnej kohorcie pacjentów z SLE lub przeciwciałami antyfosfolipidowymi.¹³

Przeżycie bez zdarzeń

Na podstawie analizy Kaplana-Meiera, wskaźniki 1-, 3- i 5-letniego przeżycia bez zdarzeń wynosiły odpowiednio 92,6% (95% przedział ufności [CI], 90-95,3%), 85,2% (95% CI, 81,3-89,4%) i 79,8% (95% CI, 74,4-85,5%).¹⁴ W badaniu opartym na analizie klastrowej 383 pacjentów (70,2% kobiet; średni wiek 37,7 lat) zidentyfikowano cztery klastry pacjentów, gdzie klaster 3 wykazywał najlepsze wyniki, a klaster 2 cierpieli najwyższą częstość nowo pojawiającej się zakrzepicy tętniczej.¹⁵ Pięcioletnie wskaźniki przeżycia bez zdarzeń w klastrze 2 wynosiły 71,0%, podobnie do wskaźnika 74,9% raportowanego w kohorcie japońskiej.¹⁶

Rokowanie w połączeniu z ciążą

APS jest najważniejszą przyczynę nawracających poronień, którą można leczyć. Pomimo ustalonych terapii przeciwzakrzepowych, wskaźnik żywych urodzeń u pacjentek z APS jest ograniczony do 70-80%.¹⁷ Antykoagulant toczniowy (LA), ale nie przeciwciała antykardiolipinowe (aCL), okazał się być predyktorem niekorzystnego wyniku ciąży.¹⁸

Chociaż wskaźnik żywych urodzeń jest ograniczony do 70-80% u pacjentek poddanych skojarzonej terapii kwasem acetylosalicylowym w małej dawce i heparyną podczas ciąży, jest to obecnie najbardziej ugruntowane leczenie w powszechnym użyciu.¹⁹ Kobiety z przeciwciałami antyfosfolipidowymi, które doświadczają nawracających poronień, mogą mieć korzystne rokowanie w kolejnych ciążach, jeśli są leczone aspiryną i heparyną.²⁰

Czynniki genetyczne w położniczym APS

Badania sugerują, że polimorfizmy genetyczne w genach TSHR i C1D stanowią czynniki ryzyka położniczego APS, wskazując na absolutną potrzebę przyspieszenia badań w tym obszarze.²¹ Wyniki te były zgodne z wykryciem ryzyka rs2288493 w modelu recesywnym.²²

Częstość występowania genotypu ryzyka TSHR u osób homozygotycznych dla allelu ryzyka rs2288493 wynosiła 16,5% u pacjentek i 3,1% u zdrowych kontroli. Częstość występowania allelu ryzyka C1D w tym badaniu wynosiła 8,3% u pacjentek i 1,4% u zdrowych kontroli.²³

Score aGAPSS w przewidywaniu wyników położniczych

Dostosowany Globalny Wynik Zespołu Antyfosfolipidowego (aGAPSS) to narzędzie zaproponowane do kwantyfikacji ryzyka manifestacji klinicznych związanych z przeciwciałami antyfosfolipidowymi. Jednak w badaniu obejmującym nosicieli przeciwciał antyfosfolipidowych bez innych chorób autoimmunologicznych, aGAPSS nie wydaje się być cennym narzędziem do identyfikacji pacjentek zagrożonych powikłaniami położniczymi pomimo leczenia.²⁴

Zmiany łożyskowe w APS

Nieprawidłowości łożyska wystąpiły u pacjentek z APS pomimo ścisłego i optymalnego monitorowania położniczego.²⁵ Ciąże u kobiet z APS są uważane za wysokiego ryzyka i są ściśle związane z niekorzystnymi wynikami ciąży. Pomimo standardowej terapii kwasem acetylosalicylowym w małej dawce (LDA) i heparyną drobnocząsteczkową (LMWH) – oraz dodaniem hydroksychlorochiny (HCQ) w wybranych przypadkach – analiza histopatologiczna łożysk od pacjentek z APS często ujawnia oznaki niedokrwienia matczynego i płodowego.²⁶

Obecność wyższych median mian aCL IgG w łożyskach ciąż z dwoma lub więcej zmianami histologicznymi oraz w tych ze zwiększonymi węzłami syncytialnymi sugeruje, że miana aCL IgG mogą służyć jako markery do przewidywania specyficznych patologii łożyska.²⁷ Wyniki są zgodne z wcześniejszą literaturą wskazującą, że zmiany łożyskowe, takie jak zawały, upośledzona przebudowa tętnic spiralnych i zapalenie doczesnej, są powszechne w APS i przyczyniają się do złych wyników ciąży.²⁸

Rola HCQ w zmniejszaniu tych zmian jest poparta jej działaniem przeciwzapalnym i przeciwzakrzepowym. Wykazano, że HCQ zmniejsza wiązanie przeciwciał antyfosfolipidowych do syncytiotrofoblastów i przywraca ekspresję anneksyny A5, białka tworzącego ochronną przeciwzakrzepową tarczę na powierzchni łożyska.²⁹ Biorąc pod uwagę znane korzyści z HCQ, rozpoczęcie tego leczenia na początku ciąży u pacjentek z opornym APS, oprócz standardowej terapii, wydaje się korzystne.³⁰

Różnice między naczyniowym a położniczym APS

Naczyniowy i położniczy APS zostały zasugerowane jako dwa różne warianty zespołu.³¹ Najbardziej uderzająca różnica polega na potrzebie drugiego bodźca do wywołania zakrzepicy u naiwnych zwierząt biernie otrzymujących ludzkie przeciwciała antyfosfolipidowe, podczas gdy nie jest to wymagane w modelach utraty płodu.³²

W rzeczywistości infuzja frakcji IgG przeciwciał antyfosfolipidowych u ciężarnych naiwnych myszy może sama w sobie wywołać utratę płodu i opóźnienie wzrostu.³³ Niedawno wykazano, że β2GPI wykazuje szczególną dystrybucję tkankową u naiwnych zwierząt w spoczynku, będąc wykrywalną tylko na poziomie śródbłonka macicy, ale nie w innych unaczynowanych tkankach.³⁴

Te wyniki sugerują, że zwiększone poziomy potranslacyjnie zmodyfikowanego β2GPI i wyższa reaktywność przeciwciał przeciwko zmodyfikowanej cząsteczce mogą wpływać na biologiczne konsekwencje wiązania przeciwciał antyfosfolipidowych.³⁵ Zgodnie z ostatnią hipotezą, swoistość epitopowa zależnych od β2GPI przeciwciał IgG u tych osób okazała się częściej skierowana przeciwko domenie IV lub V niż przeciwko domenie I, jak w surowicach z pełnoobjawowym APS.³⁶

Znaczenie kliniczne różnych profili przeciwciał

Przeciwciała anty-β2GPI reagujące z immunodominującym epitopem na domenie I cząsteczki zostały opisane jako dominująca swoistość u pacjentów z APS, korelująca z bardziej agresywnym obrazem klinicznym.³⁷ Istnieją coraz liczniejsze dowody, że pacjenci z kategorii II mają mniejsze ryzyko rozwoju manifestacji APS.³⁸

Pacjenci z izolowaną pozytywnością LA cierpieli podobne ryzyko jak pacjenci z wtórnym APS i pacjenci z wieloma czynnikami ryzyka sercowo-naczyniowego.³⁹ Klaster 4 miał podobne rokowanie jak klaster 1 i klaster 2 w odniesieniu do pierwotnego punktu końcowego, potwierdzając, że LA reprezentuje spektrum przeciwciał wysokiego ryzyka.⁴⁰

Rokowanie długoterminowe

APS zwykle nie ustępuje. Pacjent może nigdy nie doświadczyć zakrzepu ani powikłań, ale prawdopodobnie będzie musiał przyjmować leki przeciwzakrzepowe przez resztę życia.⁴¹ Większość osób zarządzających APS za pomocą leków przeciwzakrzepowych może bezpiecznie zajść w ciążę i mieć biologiczne dzieci.⁴²

Chociaż obecność przeciwciał antyfosfolipidowych, nawet jeśli utrzymuje się w czasie, nie wyjaśnia pełnego spektrum APS.⁴³ Innymi słowy, przeciwciała o tej samej swoistości antygenowej i mianie zostały powiązane z różnymi obrazami klinicznymi i uznano, że wspierają różne mechanizmy patogenne w modelach eksperymentalnych.⁴⁴

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.

Materiały źródłowe

#1 Antiphospholipid Syndrome Follow-up: Further Outpatient Care, Transfer, Deterrence/Prevention
https://emedicine.medscape.com/article/333221-followup
With appropriate medication and lifestyle modifications, most individuals with primary antiphospholipid syndrome (APS) lead normal healthy lives. However, subsets of patients continue to have thrombotic events despite aggressive therapies. In these patients and in patients with catastrophic APS, the disease course can be devastating, often leading to significant morbidity or early death. […] In large European cohort studies, 10-year survival is approximately 90-94%. […] A retrospective study suggested that hypertension or medium-to-high titers of IgG anticardiolipin antibody are risk factors for a first thrombotic event in asymptomatic patients with antiphospholipid (aPL) antibodies. […] Patients with secondary APS carry a prognosis similar to that of patients with primary APS; in the former, however, morbidity and mortality may also be influenced by these patients’ underlying autoimmune or rheumatic condition.
#2 Antiphospholipid Syndrome Follow-up: Further Outpatient Care, Transfer, Deterrence/Prevention
https://emedicine.medscape.com/article/333221-followup
With appropriate medication and lifestyle modifications, most individuals with primary antiphospholipid syndrome (APS) lead normal healthy lives. However, subsets of patients continue to have thrombotic events despite aggressive therapies. In these patients and in patients with catastrophic APS, the disease course can be devastating, often leading to significant morbidity or early death. […] In large European cohort studies, 10-year survival is approximately 90-94%. […] A retrospective study suggested that hypertension or medium-to-high titers of IgG anticardiolipin antibody are risk factors for a first thrombotic event in asymptomatic patients with antiphospholipid (aPL) antibodies. […] Patients with secondary APS carry a prognosis similar to that of patients with primary APS; in the former, however, morbidity and mortality may also be influenced by these patients’ underlying autoimmune or rheumatic condition.
#3 Antiphospholipid Syndrome Follow-up: Further Outpatient Care, Transfer, Deterrence/Prevention
https://emedicine.medscape.com/article/333221-followup
With appropriate medication and lifestyle modifications, most individuals with primary antiphospholipid syndrome (APS) lead normal healthy lives. However, subsets of patients continue to have thrombotic events despite aggressive therapies. In these patients and in patients with catastrophic APS, the disease course can be devastating, often leading to significant morbidity or early death. […] In large European cohort studies, 10-year survival is approximately 90-94%. […] A retrospective study suggested that hypertension or medium-to-high titers of IgG anticardiolipin antibody are risk factors for a first thrombotic event in asymptomatic patients with antiphospholipid (aPL) antibodies. […] Patients with secondary APS carry a prognosis similar to that of patients with primary APS; in the former, however, morbidity and mortality may also be influenced by these patients’ underlying autoimmune or rheumatic condition.
#4 Antiphospholipid Syndrome Follow-up: Further Outpatient Care, Transfer, Deterrence/Prevention
https://emedicine.medscape.com/article/333221-followup
In patients with SLE and APS, aPL antibodies have been associated with neuropsychiatric disease and have been recognized as a major predictor of irreversible organ damage. […] Women with aPL antibodies who experience recurrent miscarriages may have favorable prognoses in subsequent pregnancies if treated with aspirin and heparin.
#5 Antiphospholipid syndrome in 2014: more clinical manifestations, novel pathogenic players and emerging biomarkers | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/ar4549
The clinical spectrum of the anti-phospholipid syndrome (APS) is not limited to vascular thrombosis or miscarriages but includes additional manifestations that cannot be explained solely by a thrombophilic state. […] Anti-2GPI antibodies reacting with an immunodominant epitope on domain I of the molecule were reported as the prevalent specificity in APS patients, correlating with a more aggressive clinical picture. […] Triple positivity, defined by the presence of LA and medium/high titers of aCL and anti-2GPI antibodies (above the 99th percentile), is the most predictive profile for clinical manifestations and recurrences despite conventional treatment. […] There is growing evidence that patients in category II have a lesser risk to develop APS manifestations. […] Clinical events are more robustly associated with aPLs of the IgG isotype, an isolated positivity for aCLs or anti-2GPI antibodies of the IgM isotype being rarely detected in APS cohorts.
#6 Triple positive profile in antiphospholipid syndrome: prognosis, relapse and management from a retrospective multicentre study | RMD Open
https://rmdopen.bmj.com/content/9/1/e002534
204 patients were included in our study, 68 were triple-positive and 136 were single or double positive. […] The relapse rate was higher in triple-positive patients than in others (63.2% vs 39.7%, p=0002). […] In multivariate analysis, the triple-positive profile was associated with a higher risk of relapse (HR 1.63; 95% CI 1.04 to 2.55; p=0.031). […] The triple-positivity is associated with a higher risk of relapse and obstetrical complications. […] Triple-positive APS had higher rates of relapse and tend to have more frequently catastrophic APS. […] The stratification of APS therapies and overall risk on the status of antiphospholipid antibodies triple positivity could be relevant and further studies are needed. […] The triple-positive profile appears to be the most important factor of thrombosis, with a relative risk of 33, compared with a relative risk of 4 and 3 for LA and anti-2GPI respectively.
#7 Triple positive profile in antiphospholipid syndrome: prognosis, relapse and management from a retrospective multicentre study | RMD Open
https://rmdopen.bmj.com/content/9/1/e002534
204 patients were included in our study, 68 were triple-positive and 136 were single or double positive. […] The relapse rate was higher in triple-positive patients than in others (63.2% vs 39.7%, p=0002). […] In multivariate analysis, the triple-positive profile was associated with a higher risk of relapse (HR 1.63; 95% CI 1.04 to 2.55; p=0.031). […] The triple-positivity is associated with a higher risk of relapse and obstetrical complications. […] Triple-positive APS had higher rates of relapse and tend to have more frequently catastrophic APS. […] The stratification of APS therapies and overall risk on the status of antiphospholipid antibodies triple positivity could be relevant and further studies are needed. […] The triple-positive profile appears to be the most important factor of thrombosis, with a relative risk of 33, compared with a relative risk of 4 and 3 for LA and anti-2GPI respectively.
#8 Triple positive profile in antiphospholipid syndrome: prognosis, relapse and management from a retrospective multicentre study | RMD Open
https://rmdopen.bmj.com/content/9/1/e002534
The triple-positive profile was associated with a significant increase in relapse rates (63.2% vs 39.7%) in this cohort with a long-term follow-up of more than 10 years. […] Triple-positive APS patients constitute a particular subtype of APS with a high risk of relapse and a need of additional therapies.
#9 Triple positive profile in antiphospholipid syndrome: prognosis, relapse and management from a retrospective multicentre study | RMD Open
https://rmdopen.bmj.com/content/9/1/e002534
204 patients were included in our study, 68 were triple-positive and 136 were single or double positive. […] The relapse rate was higher in triple-positive patients than in others (63.2% vs 39.7%, p=0002). […] In multivariate analysis, the triple-positive profile was associated with a higher risk of relapse (HR 1.63; 95% CI 1.04 to 2.55; p=0.031). […] The triple-positivity is associated with a higher risk of relapse and obstetrical complications. […] Triple-positive APS had higher rates of relapse and tend to have more frequently catastrophic APS. […] The stratification of APS therapies and overall risk on the status of antiphospholipid antibodies triple positivity could be relevant and further studies are needed. […] The triple-positive profile appears to be the most important factor of thrombosis, with a relative risk of 33, compared with a relative risk of 4 and 3 for LA and anti-2GPI respectively.
#10 Antiphospholipid Syndrome Follow-up: Further Outpatient Care, Transfer, Deterrence/Prevention
https://emedicine.medscape.com/article/333221-followup
With appropriate medication and lifestyle modifications, most individuals with primary antiphospholipid syndrome (APS) lead normal healthy lives. However, subsets of patients continue to have thrombotic events despite aggressive therapies. In these patients and in patients with catastrophic APS, the disease course can be devastating, often leading to significant morbidity or early death. […] In large European cohort studies, 10-year survival is approximately 90-94%. […] A retrospective study suggested that hypertension or medium-to-high titers of IgG anticardiolipin antibody are risk factors for a first thrombotic event in asymptomatic patients with antiphospholipid (aPL) antibodies. […] Patients with secondary APS carry a prognosis similar to that of patients with primary APS; in the former, however, morbidity and mortality may also be influenced by these patients’ underlying autoimmune or rheumatic condition.
#11 Antiphospholipid syndrome in 2014: more clinical manifestations, novel pathogenic players and emerging biomarkers | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/ar4549
The clinical spectrum of the anti-phospholipid syndrome (APS) is not limited to vascular thrombosis or miscarriages but includes additional manifestations that cannot be explained solely by a thrombophilic state. […] Anti-2GPI antibodies reacting with an immunodominant epitope on domain I of the molecule were reported as the prevalent specificity in APS patients, correlating with a more aggressive clinical picture. […] Triple positivity, defined by the presence of LA and medium/high titers of aCL and anti-2GPI antibodies (above the 99th percentile), is the most predictive profile for clinical manifestations and recurrences despite conventional treatment. […] There is growing evidence that patients in category II have a lesser risk to develop APS manifestations. […] Clinical events are more robustly associated with aPLs of the IgG isotype, an isolated positivity for aCLs or anti-2GPI antibodies of the IgM isotype being rarely detected in APS cohorts.
#12 Antiphospholipid syndrome in 2014: more clinical manifestations, novel pathogenic players and emerging biomarkers | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/ar4549
Recent studies have demonstrated that patients with multiple positive test results (that is, LA, aCLs and anti-2GPI autoantibodies particularly of the IgG isotype) display a much higher risk for developing clinical complications. […] High levels of antibodies with comparable specificity and detected by a research ELISA kit have been recently associated with an increased risk for thrombotic events in a prospective cohort of SLE or aPL patients by the same group. […] The presence of aPLs, even if persistent over time, does not explain the full spectrum of APS. […] In other words, autoantibodies with the same autoantigen specificity and titers have been associated with different clinical pictures and found to support diverse pathogenic mechanisms in experimental models. […] Vascular and the obstetric APS have been suggested to represent two different variants of the syndrome.
#13 Antiphospholipid syndrome in 2014: more clinical manifestations, novel pathogenic players and emerging biomarkers | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/ar4549
Recent studies have demonstrated that patients with multiple positive test results (that is, LA, aCLs and anti-2GPI autoantibodies particularly of the IgG isotype) display a much higher risk for developing clinical complications. […] High levels of antibodies with comparable specificity and detected by a research ELISA kit have been recently associated with an increased risk for thrombotic events in a prospective cohort of SLE or aPL patients by the same group. […] The presence of aPLs, even if persistent over time, does not explain the full spectrum of APS. […] In other words, autoantibodies with the same autoantigen specificity and titers have been associated with different clinical pictures and found to support diverse pathogenic mechanisms in experimental models. […] Vascular and the obstetric APS have been suggested to represent two different variants of the syndrome.
#14 Clinical characteristics and prognosis of patients with antiphospholipid antibodies based on cluster analysis: an 8-year cohort study | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-022-02814-w
Four clusters among 383 patients (70.2% female; mean age 37.7years) were identified. […] Cluster 3 showed the best outcome, while cluster 2 suffered highest frequency of newly onset arterial thrombosis. […] Patients with isolated LA positivity suffered similar risks with secondary APS and patients with multiple cardiovascular risk factors. […] The 5-year event-free survival rates in clusters 2 were 71.0%, similar to that of 74.9% reported in the Japanese cohort. […] From Kaplan Meier analysis, 1-, 3-, and 5-year event-free survival rates were 92.6% (95% confidence interval [CI], 90-95.3%), 85.2% (95% CI, 81.3-89.4%) and 79.8% (95% CI, 74.4-85.5%), respectively. […] For primary endpoint and thrombosis endpoint, patients in cluster 3 showed the lowest risks, while patients in clusters 1, 2, and 4 suffered similar risks. […] Cluster 4 shared similar prognosis with cluster 1 and cluster 2 in terms of primary endpoint, confirming that LA represented a high-risk antibody spectrum.
#15 Clinical characteristics and prognosis of patients with antiphospholipid antibodies based on cluster analysis: an 8-year cohort study | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-022-02814-w
Four clusters among 383 patients (70.2% female; mean age 37.7years) were identified. […] Cluster 3 showed the best outcome, while cluster 2 suffered highest frequency of newly onset arterial thrombosis. […] Patients with isolated LA positivity suffered similar risks with secondary APS and patients with multiple cardiovascular risk factors. […] The 5-year event-free survival rates in clusters 2 were 71.0%, similar to that of 74.9% reported in the Japanese cohort. […] From Kaplan Meier analysis, 1-, 3-, and 5-year event-free survival rates were 92.6% (95% confidence interval [CI], 90-95.3%), 85.2% (95% CI, 81.3-89.4%) and 79.8% (95% CI, 74.4-85.5%), respectively. […] For primary endpoint and thrombosis endpoint, patients in cluster 3 showed the lowest risks, while patients in clusters 1, 2, and 4 suffered similar risks. […] Cluster 4 shared similar prognosis with cluster 1 and cluster 2 in terms of primary endpoint, confirming that LA represented a high-risk antibody spectrum.
#16 Clinical characteristics and prognosis of patients with antiphospholipid antibodies based on cluster analysis: an 8-year cohort study | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-022-02814-w
Four clusters among 383 patients (70.2% female; mean age 37.7years) were identified. […] Cluster 3 showed the best outcome, while cluster 2 suffered highest frequency of newly onset arterial thrombosis. […] Patients with isolated LA positivity suffered similar risks with secondary APS and patients with multiple cardiovascular risk factors. […] The 5-year event-free survival rates in clusters 2 were 71.0%, similar to that of 74.9% reported in the Japanese cohort. […] From Kaplan Meier analysis, 1-, 3-, and 5-year event-free survival rates were 92.6% (95% confidence interval [CI], 90-95.3%), 85.2% (95% CI, 81.3-89.4%) and 79.8% (95% CI, 74.4-85.5%), respectively. […] For primary endpoint and thrombosis endpoint, patients in cluster 3 showed the lowest risks, while patients in clusters 1, 2, and 4 suffered similar risks. […] Cluster 4 shared similar prognosis with cluster 1 and cluster 2 in terms of primary endpoint, confirming that LA represented a high-risk antibody spectrum.
#17 The first genome-wide association study identifying new susceptibility loci for obstetric antiphospholipid syndrome | Journal of Human Genetics
https://www.nature.com/articles/jhg201746
Antiphospholipid syndrome (APS) is the most important treatable cause of recurrent pregnancy loss. The live birth rate is limited to only 70-80% in patients with APS undergoing established anticoagulant therapy. […] Lupus anticoagulant (LA), but not anticardiolipin antibody (aCL), was found to predict adverse pregnancy outcome. […] Even though the live birth rate is limited to only 70-80% in patients undergoing low-dose aspirin and heparin combined therapy during pregnancy, this is currently the most established treatment in general use. […] LA, but not aCL, was found to predict adverse pregnancy outcome. […] Despite the large efforts of clinical scientists in this field, the results of clinical research have not been optimistic, basically due to the lack of understanding of the mechanisms involved in obstetric APS.
#18 The first genome-wide association study identifying new susceptibility loci for obstetric antiphospholipid syndrome | Journal of Human Genetics
https://www.nature.com/articles/jhg201746
Antiphospholipid syndrome (APS) is the most important treatable cause of recurrent pregnancy loss. The live birth rate is limited to only 70-80% in patients with APS undergoing established anticoagulant therapy. […] Lupus anticoagulant (LA), but not anticardiolipin antibody (aCL), was found to predict adverse pregnancy outcome. […] Even though the live birth rate is limited to only 70-80% in patients undergoing low-dose aspirin and heparin combined therapy during pregnancy, this is currently the most established treatment in general use. […] LA, but not aCL, was found to predict adverse pregnancy outcome. […] Despite the large efforts of clinical scientists in this field, the results of clinical research have not been optimistic, basically due to the lack of understanding of the mechanisms involved in obstetric APS.
#19 The first genome-wide association study identifying new susceptibility loci for obstetric antiphospholipid syndrome | Journal of Human Genetics
https://www.nature.com/articles/jhg201746
Antiphospholipid syndrome (APS) is the most important treatable cause of recurrent pregnancy loss. The live birth rate is limited to only 70-80% in patients with APS undergoing established anticoagulant therapy. […] Lupus anticoagulant (LA), but not anticardiolipin antibody (aCL), was found to predict adverse pregnancy outcome. […] Even though the live birth rate is limited to only 70-80% in patients undergoing low-dose aspirin and heparin combined therapy during pregnancy, this is currently the most established treatment in general use. […] LA, but not aCL, was found to predict adverse pregnancy outcome. […] Despite the large efforts of clinical scientists in this field, the results of clinical research have not been optimistic, basically due to the lack of understanding of the mechanisms involved in obstetric APS.
#20 Antiphospholipid Syndrome Follow-up: Further Outpatient Care, Transfer, Deterrence/Prevention
https://emedicine.medscape.com/article/333221-followup
In patients with SLE and APS, aPL antibodies have been associated with neuropsychiatric disease and have been recognized as a major predictor of irreversible organ damage. […] Women with aPL antibodies who experience recurrent miscarriages may have favorable prognoses in subsequent pregnancies if treated with aspirin and heparin.
#21 The first genome-wide association study identifying new susceptibility loci for obstetric antiphospholipid syndrome | Journal of Human Genetics
https://www.nature.com/articles/jhg201746
Our results suggest that genetic polymorphisms in TSHR and C1D represent risk factors for obstetric APS, indicating an absolute need for accelerated research in this area. […] The findings was consistent with the detection of a rs2288493 risk under the recessive model. […] The prevalence of a TSHR-risk genotype in subjects who were homozygous for a risk allele of rs2288493 was 16.5% in patients and 3.1% in healthy controls in this study. […] The prevalence of a C1D-risk allele in this study was 8.3% in patients and 1.4% in healthy controls. […] Our results suggest that genetic polymorphisms in TSHR and C1D represent risk factors for obstetric APS, indicating an absolute need for accelerated research in this area.
#22 The first genome-wide association study identifying new susceptibility loci for obstetric antiphospholipid syndrome | Journal of Human Genetics
https://www.nature.com/articles/jhg201746
Our results suggest that genetic polymorphisms in TSHR and C1D represent risk factors for obstetric APS, indicating an absolute need for accelerated research in this area. […] The findings was consistent with the detection of a rs2288493 risk under the recessive model. […] The prevalence of a TSHR-risk genotype in subjects who were homozygous for a risk allele of rs2288493 was 16.5% in patients and 3.1% in healthy controls in this study. […] The prevalence of a C1D-risk allele in this study was 8.3% in patients and 1.4% in healthy controls. […] Our results suggest that genetic polymorphisms in TSHR and C1D represent risk factors for obstetric APS, indicating an absolute need for accelerated research in this area.
#23 The first genome-wide association study identifying new susceptibility loci for obstetric antiphospholipid syndrome | Journal of Human Genetics
https://www.nature.com/articles/jhg201746
Our results suggest that genetic polymorphisms in TSHR and C1D represent risk factors for obstetric APS, indicating an absolute need for accelerated research in this area. […] The findings was consistent with the detection of a rs2288493 risk under the recessive model. […] The prevalence of a TSHR-risk genotype in subjects who were homozygous for a risk allele of rs2288493 was 16.5% in patients and 3.1% in healthy controls in this study. […] The prevalence of a C1D-risk allele in this study was 8.3% in patients and 1.4% in healthy controls. […] Our results suggest that genetic polymorphisms in TSHR and C1D represent risk factors for obstetric APS, indicating an absolute need for accelerated research in this area.
#24 Does Adjusted Global Antiphospholipid Syndrome Score (aGAPSS) Predict the Obstetric Outcome in Antiphospholipid Antibody Carriers? A Single-Center Study
https://pmc.ncbi.nlm.nih.gov/articles/PMC9464174/
The adjusted Global Antiphospholipid Syndrome (APS) Score (aGAPSS) is a tool proposed to quantify the risk for antiphospholipid antibody (aPL)-related clinical manifestations. […] In the present study, including aPL carriers without other autoimmune diseases, aGAPSS is not a valuable tool to identify patients at risk for obstetric complications despite treatment. […] In summary, in the present study, including aPL carriers without other autoimmune diseases, the aGAPSS does not seem to be a valuable tool to identify patients at risk for obstetric complications despite treatment.
#25 Frontiers | Placental lesions in patients with antiphospholipid antibody syndrome: experience of a single tertiary-care Italian reference center
https://www.frontiersin.org/journals/lupus/articles/10.3389/flupu.2024.1459172/full
Placental anomalies have occurred in patients with APS despite close and optimal obstetric monitoring. It is thus tempting to speculate that HCQ may have beneficial effects on pregnancy by decreasing the risk of deciduitis in patients with APS. […] Pregnancies in women with Antiphospholipid Syndrome (APS) are considered high-risk and are closely associated with adverse pregnancy outcomes. Despite standard therapy with LDA and LMWHâand the addition of HCQ in selected casesâhistopathological analysis of placentas from APS patients often reveals signs of maternal and fetal malperfusion. […] The presence of higher median titers of aCL IgG in placentas of pregnancies with two or more histological lesions and in those with increased syncytial knots suggests that aCL IgG titers might serve as markers for predicting specific placental pathologies.
#26 Frontiers | Placental lesions in patients with antiphospholipid antibody syndrome: experience of a single tertiary-care Italian reference center
https://www.frontiersin.org/journals/lupus/articles/10.3389/flupu.2024.1459172/full
Placental anomalies have occurred in patients with APS despite close and optimal obstetric monitoring. It is thus tempting to speculate that HCQ may have beneficial effects on pregnancy by decreasing the risk of deciduitis in patients with APS. […] Pregnancies in women with Antiphospholipid Syndrome (APS) are considered high-risk and are closely associated with adverse pregnancy outcomes. Despite standard therapy with LDA and LMWHâand the addition of HCQ in selected casesâhistopathological analysis of placentas from APS patients often reveals signs of maternal and fetal malperfusion. […] The presence of higher median titers of aCL IgG in placentas of pregnancies with two or more histological lesions and in those with increased syncytial knots suggests that aCL IgG titers might serve as markers for predicting specific placental pathologies.
#27 Frontiers | Placental lesions in patients with antiphospholipid antibody syndrome: experience of a single tertiary-care Italian reference center
https://www.frontiersin.org/journals/lupus/articles/10.3389/flupu.2024.1459172/full
Placental anomalies have occurred in patients with APS despite close and optimal obstetric monitoring. It is thus tempting to speculate that HCQ may have beneficial effects on pregnancy by decreasing the risk of deciduitis in patients with APS. […] Pregnancies in women with Antiphospholipid Syndrome (APS) are considered high-risk and are closely associated with adverse pregnancy outcomes. Despite standard therapy with LDA and LMWHâand the addition of HCQ in selected casesâhistopathological analysis of placentas from APS patients often reveals signs of maternal and fetal malperfusion. […] The presence of higher median titers of aCL IgG in placentas of pregnancies with two or more histological lesions and in those with increased syncytial knots suggests that aCL IgG titers might serve as markers for predicting specific placental pathologies.
#28 Frontiers | Placental lesions in patients with antiphospholipid antibody syndrome: experience of a single tertiary-care Italian reference center
https://www.frontiersin.org/journals/lupus/articles/10.3389/flupu.2024.1459172/full
Our findings align with previous literature indicating that placental lesions such as infarctions, impaired spiral artery remodeling, and decidual inflammation are common in APS and contribute to poor pregnancy outcomes. […] The role of HCQ in reducing these lesions is supported by its anti-inflammatory and antithrombotic effects. HCQ has been shown to decrease the binding of aPL to syncytiotrophoblasts and restore the expression of annexin A5, a protein that forms a protective anticoagulant shield on the placental surface. […] Given the known benefits of HCQ, initiating this treatment at the beginning of pregnancy with refractory APS, in addition to standard therapy, appears beneficial.
#29 Frontiers | Placental lesions in patients with antiphospholipid antibody syndrome: experience of a single tertiary-care Italian reference center
https://www.frontiersin.org/journals/lupus/articles/10.3389/flupu.2024.1459172/full
Our findings align with previous literature indicating that placental lesions such as infarctions, impaired spiral artery remodeling, and decidual inflammation are common in APS and contribute to poor pregnancy outcomes. […] The role of HCQ in reducing these lesions is supported by its anti-inflammatory and antithrombotic effects. HCQ has been shown to decrease the binding of aPL to syncytiotrophoblasts and restore the expression of annexin A5, a protein that forms a protective anticoagulant shield on the placental surface. […] Given the known benefits of HCQ, initiating this treatment at the beginning of pregnancy with refractory APS, in addition to standard therapy, appears beneficial.
#30 Frontiers | Placental lesions in patients with antiphospholipid antibody syndrome: experience of a single tertiary-care Italian reference center
https://www.frontiersin.org/journals/lupus/articles/10.3389/flupu.2024.1459172/full
Our findings align with previous literature indicating that placental lesions such as infarctions, impaired spiral artery remodeling, and decidual inflammation are common in APS and contribute to poor pregnancy outcomes. […] The role of HCQ in reducing these lesions is supported by its anti-inflammatory and antithrombotic effects. HCQ has been shown to decrease the binding of aPL to syncytiotrophoblasts and restore the expression of annexin A5, a protein that forms a protective anticoagulant shield on the placental surface. […] Given the known benefits of HCQ, initiating this treatment at the beginning of pregnancy with refractory APS, in addition to standard therapy, appears beneficial.
#31 Antiphospholipid syndrome in 2014: more clinical manifestations, novel pathogenic players and emerging biomarkers | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/ar4549
Recent studies have demonstrated that patients with multiple positive test results (that is, LA, aCLs and anti-2GPI autoantibodies particularly of the IgG isotype) display a much higher risk for developing clinical complications. […] High levels of antibodies with comparable specificity and detected by a research ELISA kit have been recently associated with an increased risk for thrombotic events in a prospective cohort of SLE or aPL patients by the same group. […] The presence of aPLs, even if persistent over time, does not explain the full spectrum of APS. […] In other words, autoantibodies with the same autoantigen specificity and titers have been associated with different clinical pictures and found to support diverse pathogenic mechanisms in experimental models. […] Vascular and the obstetric APS have been suggested to represent two different variants of the syndrome.
#32 Antiphospholipid syndrome in 2014: more clinical manifestations, novel pathogenic players and emerging biomarkers | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/ar4549
The most striking difference is represented by the need of a second hit for triggering thrombosis in naive animals passively infused with human aPLs, while this is not apparently required in models of fetal loss. […] In fact, the infusion of aPL IgG fractions in pregnant naive mice can itself induce fetal loss and growth retardation. […] It has been recently demonstrated that 2GPI displays a peculiar tissue distribution in resting naive animals, being detectable only at the level of uterine endothelium but not in other vascularized tissues. […] Altogether, these findings suggest that increased levels of post-transcriptionally modified 2GPI and the higher antibody reactivity against the modified molecule may affect the biological consequences of aPL binding. […] In line with the last hypothesis, the epitope specificity of the 2GPI-dependent IgG antibodies in these subjects was found to be more frequently directed against DIV or DV than against DI as in full-blown APS sera.
#33 Antiphospholipid syndrome in 2014: more clinical manifestations, novel pathogenic players and emerging biomarkers | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/ar4549
The most striking difference is represented by the need of a second hit for triggering thrombosis in naive animals passively infused with human aPLs, while this is not apparently required in models of fetal loss. […] In fact, the infusion of aPL IgG fractions in pregnant naive mice can itself induce fetal loss and growth retardation. […] It has been recently demonstrated that 2GPI displays a peculiar tissue distribution in resting naive animals, being detectable only at the level of uterine endothelium but not in other vascularized tissues. […] Altogether, these findings suggest that increased levels of post-transcriptionally modified 2GPI and the higher antibody reactivity against the modified molecule may affect the biological consequences of aPL binding. […] In line with the last hypothesis, the epitope specificity of the 2GPI-dependent IgG antibodies in these subjects was found to be more frequently directed against DIV or DV than against DI as in full-blown APS sera.
#34 Antiphospholipid syndrome in 2014: more clinical manifestations, novel pathogenic players and emerging biomarkers | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/ar4549
The most striking difference is represented by the need of a second hit for triggering thrombosis in naive animals passively infused with human aPLs, while this is not apparently required in models of fetal loss. […] In fact, the infusion of aPL IgG fractions in pregnant naive mice can itself induce fetal loss and growth retardation. […] It has been recently demonstrated that 2GPI displays a peculiar tissue distribution in resting naive animals, being detectable only at the level of uterine endothelium but not in other vascularized tissues. […] Altogether, these findings suggest that increased levels of post-transcriptionally modified 2GPI and the higher antibody reactivity against the modified molecule may affect the biological consequences of aPL binding. […] In line with the last hypothesis, the epitope specificity of the 2GPI-dependent IgG antibodies in these subjects was found to be more frequently directed against DIV or DV than against DI as in full-blown APS sera.
#35 Antiphospholipid syndrome in 2014: more clinical manifestations, novel pathogenic players and emerging biomarkers | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/ar4549
The most striking difference is represented by the need of a second hit for triggering thrombosis in naive animals passively infused with human aPLs, while this is not apparently required in models of fetal loss. […] In fact, the infusion of aPL IgG fractions in pregnant naive mice can itself induce fetal loss and growth retardation. […] It has been recently demonstrated that 2GPI displays a peculiar tissue distribution in resting naive animals, being detectable only at the level of uterine endothelium but not in other vascularized tissues. […] Altogether, these findings suggest that increased levels of post-transcriptionally modified 2GPI and the higher antibody reactivity against the modified molecule may affect the biological consequences of aPL binding. […] In line with the last hypothesis, the epitope specificity of the 2GPI-dependent IgG antibodies in these subjects was found to be more frequently directed against DIV or DV than against DI as in full-blown APS sera.
#36 Antiphospholipid syndrome in 2014: more clinical manifestations, novel pathogenic players and emerging biomarkers | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/ar4549
The most striking difference is represented by the need of a second hit for triggering thrombosis in naive animals passively infused with human aPLs, while this is not apparently required in models of fetal loss. […] In fact, the infusion of aPL IgG fractions in pregnant naive mice can itself induce fetal loss and growth retardation. […] It has been recently demonstrated that 2GPI displays a peculiar tissue distribution in resting naive animals, being detectable only at the level of uterine endothelium but not in other vascularized tissues. […] Altogether, these findings suggest that increased levels of post-transcriptionally modified 2GPI and the higher antibody reactivity against the modified molecule may affect the biological consequences of aPL binding. […] In line with the last hypothesis, the epitope specificity of the 2GPI-dependent IgG antibodies in these subjects was found to be more frequently directed against DIV or DV than against DI as in full-blown APS sera.
#37 Antiphospholipid syndrome in 2014: more clinical manifestations, novel pathogenic players and emerging biomarkers | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/ar4549
The clinical spectrum of the anti-phospholipid syndrome (APS) is not limited to vascular thrombosis or miscarriages but includes additional manifestations that cannot be explained solely by a thrombophilic state. […] Anti-2GPI antibodies reacting with an immunodominant epitope on domain I of the molecule were reported as the prevalent specificity in APS patients, correlating with a more aggressive clinical picture. […] Triple positivity, defined by the presence of LA and medium/high titers of aCL and anti-2GPI antibodies (above the 99th percentile), is the most predictive profile for clinical manifestations and recurrences despite conventional treatment. […] There is growing evidence that patients in category II have a lesser risk to develop APS manifestations. […] Clinical events are more robustly associated with aPLs of the IgG isotype, an isolated positivity for aCLs or anti-2GPI antibodies of the IgM isotype being rarely detected in APS cohorts.
#38 Antiphospholipid syndrome in 2014: more clinical manifestations, novel pathogenic players and emerging biomarkers | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/ar4549
The clinical spectrum of the anti-phospholipid syndrome (APS) is not limited to vascular thrombosis or miscarriages but includes additional manifestations that cannot be explained solely by a thrombophilic state. […] Anti-2GPI antibodies reacting with an immunodominant epitope on domain I of the molecule were reported as the prevalent specificity in APS patients, correlating with a more aggressive clinical picture. […] Triple positivity, defined by the presence of LA and medium/high titers of aCL and anti-2GPI antibodies (above the 99th percentile), is the most predictive profile for clinical manifestations and recurrences despite conventional treatment. […] There is growing evidence that patients in category II have a lesser risk to develop APS manifestations. […] Clinical events are more robustly associated with aPLs of the IgG isotype, an isolated positivity for aCLs or anti-2GPI antibodies of the IgM isotype being rarely detected in APS cohorts.
#39 Clinical characteristics and prognosis of patients with antiphospholipid antibodies based on cluster analysis: an 8-year cohort study | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-022-02814-w
Four clusters among 383 patients (70.2% female; mean age 37.7years) were identified. […] Cluster 3 showed the best outcome, while cluster 2 suffered highest frequency of newly onset arterial thrombosis. […] Patients with isolated LA positivity suffered similar risks with secondary APS and patients with multiple cardiovascular risk factors. […] The 5-year event-free survival rates in clusters 2 were 71.0%, similar to that of 74.9% reported in the Japanese cohort. […] From Kaplan Meier analysis, 1-, 3-, and 5-year event-free survival rates were 92.6% (95% confidence interval [CI], 90-95.3%), 85.2% (95% CI, 81.3-89.4%) and 79.8% (95% CI, 74.4-85.5%), respectively. […] For primary endpoint and thrombosis endpoint, patients in cluster 3 showed the lowest risks, while patients in clusters 1, 2, and 4 suffered similar risks. […] Cluster 4 shared similar prognosis with cluster 1 and cluster 2 in terms of primary endpoint, confirming that LA represented a high-risk antibody spectrum.
#40 Clinical characteristics and prognosis of patients with antiphospholipid antibodies based on cluster analysis: an 8-year cohort study | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-022-02814-w
Four clusters among 383 patients (70.2% female; mean age 37.7years) were identified. […] Cluster 3 showed the best outcome, while cluster 2 suffered highest frequency of newly onset arterial thrombosis. […] Patients with isolated LA positivity suffered similar risks with secondary APS and patients with multiple cardiovascular risk factors. […] The 5-year event-free survival rates in clusters 2 were 71.0%, similar to that of 74.9% reported in the Japanese cohort. […] From Kaplan Meier analysis, 1-, 3-, and 5-year event-free survival rates were 92.6% (95% confidence interval [CI], 90-95.3%), 85.2% (95% CI, 81.3-89.4%) and 79.8% (95% CI, 74.4-85.5%), respectively. […] For primary endpoint and thrombosis endpoint, patients in cluster 3 showed the lowest risks, while patients in clusters 1, 2, and 4 suffered similar risks. […] Cluster 4 shared similar prognosis with cluster 1 and cluster 2 in terms of primary endpoint, confirming that LA represented a high-risk antibody spectrum.
#41 Antiphospholipid Syndrome: Symptoms, Diagnosis & Treatment
https://my.clevelandclinic.org/health/diseases/21685-antiphospholipid-syndrome
APS responds well to treatment with blood thinners, and you should be able to do all your usual activities once you start treatment. Most people managing APS with blood thinners can safely get pregnant and have biological children. […] APS doesn’t usually go away. You may never have a clot or experience complications, but you’ll probably need to take blood thinners for the rest of your life. […] Antiphospholipid syndrome can increase the risk of pregnancy-related complications. Talk to your healthcare provider if you’re concerned about any risks or pregnancy complications you might experience.
#42 Antiphospholipid Syndrome: Symptoms, Diagnosis & Treatment
https://my.clevelandclinic.org/health/diseases/21685-antiphospholipid-syndrome
APS responds well to treatment with blood thinners, and you should be able to do all your usual activities once you start treatment. Most people managing APS with blood thinners can safely get pregnant and have biological children. […] APS doesn’t usually go away. You may never have a clot or experience complications, but you’ll probably need to take blood thinners for the rest of your life. […] Antiphospholipid syndrome can increase the risk of pregnancy-related complications. Talk to your healthcare provider if you’re concerned about any risks or pregnancy complications you might experience.
#43 Antiphospholipid syndrome in 2014: more clinical manifestations, novel pathogenic players and emerging biomarkers | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/ar4549
Recent studies have demonstrated that patients with multiple positive test results (that is, LA, aCLs and anti-2GPI autoantibodies particularly of the IgG isotype) display a much higher risk for developing clinical complications. […] High levels of antibodies with comparable specificity and detected by a research ELISA kit have been recently associated with an increased risk for thrombotic events in a prospective cohort of SLE or aPL patients by the same group. […] The presence of aPLs, even if persistent over time, does not explain the full spectrum of APS. […] In other words, autoantibodies with the same autoantigen specificity and titers have been associated with different clinical pictures and found to support diverse pathogenic mechanisms in experimental models. […] Vascular and the obstetric APS have been suggested to represent two different variants of the syndrome.
#44 Antiphospholipid syndrome in 2014: more clinical manifestations, novel pathogenic players and emerging biomarkers | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/ar4549
Recent studies have demonstrated that patients with multiple positive test results (that is, LA, aCLs and anti-2GPI autoantibodies particularly of the IgG isotype) display a much higher risk for developing clinical complications. […] High levels of antibodies with comparable specificity and detected by a research ELISA kit have been recently associated with an increased risk for thrombotic events in a prospective cohort of SLE or aPL patients by the same group. […] The presence of aPLs, even if persistent over time, does not explain the full spectrum of APS. […] In other words, autoantibodies with the same autoantigen specificity and titers have been associated with different clinical pictures and found to support diverse pathogenic mechanisms in experimental models. […] Vascular and the obstetric APS have been suggested to represent two different variants of the syndrome.