Dermatomyositis
Leczenie
Dermatomyositis to przewlekła choroba autoimmunologiczna charakteryzująca się zapaleniem mięśni i zmianami skórnymi, wymagająca kompleksowego leczenia wielodyscyplinarnego. Podstawą terapii są glikokortykosteroidy, głównie prednizon w dawce początkowej 0,5-2 mg/kg/dobę (do 60 mg/dobę), z możliwością dożylnego podania metyloprednizolonu w pulsach 1000 mg/dobę przez 3-4 dni w ciężkich przypadkach. Leczenie trwa zwykle 9-12 miesięcy, a efekty kliniczne pojawiają się po kilku tygodniach (normalizacja enzymów mięśniowych po około 6 tygodniach, poprawa siły mięśniowej do 3 miesięcy). W celu ograniczenia działań niepożądanych steroidów stosuje się leki immunosupresyjne, takie jak metotreksat, azatiopryna, mykofenolan mofetylu, cyklosporyna i takrolimus. W lipcu 2021 roku FDA zatwierdziła OCTAGAM 10% (immunoglobulina dożylna) jako pierwszy lek dedykowany dermatomyositis u dorosłych, szczególnie w przypadkach opornych na standardowe leczenie lub z zaburzeniami połykania. W terapii opornej stosuje się także leki biologiczne, m.in. rytuksymab oraz inhibitory JAK (tofacytynib, baricytynib).
- Wprowadzenie do leczenia dermatomyositis
- Leczenie farmakologiczne dermatomyositis
- Kortykosteroidy jako terapia pierwszego rzutu
- Leki immunosupresyjne i oszczędzające steroidy
- Immunoglobulina dożylna (IVIg)
- Leki biologiczne i terapie celowane
- Leczenie zmian skórnych
- Leczenie wapnicy (calcinosis)
- Fizjoterapia i rehabilitacja w leczeniu dermatomyositis
- Terapia mowy i porady dietetyczne
- Leczenie Juvenille Dermatomyositis (postaci młodzieńczej)
- Monitorowanie i długoterminowa opieka
- Rokowanie i przebieg choroby
- Podsumowanie leczenia dermatomyositis
Wprowadzenie do leczenia dermatomyositis
Dermatomyositis to rzadka choroba autoimmunologiczna charakteryzująca się zapaleniem mięśni i charakterystycznymi zmianami skórnymi. Choć obecnie nie istnieje metoda leczenia przyczynowego (nie ma leku, który całkowicie wyleczyłby tę chorobę), dostępne są liczne opcje terapeutyczne, które mogą skutecznie kontrolować objawy, poprawiać siłę mięśniową, zmniejszać zmiany skórne oraz zapobiegać powikłaniom.12 Celem leczenia dermatomyositis jest zmniejszenie stanu zapalnego, poprawa funkcji mięśni, kontrola zmian skórnych oraz leczenie ewentualnych powikłań narządowych.3 Obecnie jedynym lekiem zatwierdzonym przez FDA do leczenia dermatomyositis u dorosłych jest OCTAGAM 10% (immunoglobulina dożylna), który został zatwierdzony w lipcu 2021 roku.45
Leczenie farmakologiczne dermatomyositis
Kortykosteroidy jako terapia pierwszego rzutu
Kortykosteroidy stanowią podstawę leczenia dermatomyositis, szczególnie w fazie ostrej choroby. Najczęściej stosowanym lekiem jest prednizon (Rayos), który szybko kontroluje objawy choroby. Typowa początkowa dawka wynosi 0,5-2 mg/kg/dobę (do 60 mg/dobę).16 W przypadkach ciężkiego zapalenia mięśni można rozważyć podanie metyloprednizolonu dożylnie w pulsach (1000 mg dziennie przez 3-4 kolejne dni).78
Kortykosteroidy zmniejszają odpowiedź układu immunologicznego, co prowadzi do redukcji liczby przeciwciał wywołujących stan zapalny. Efekty leczenia są zwykle widoczne po kilku tygodniach terapii – do normalizacji enzymów mięśniowych dochodzi zwykle po około sześciu tygodniach, natomiast poprawa siły mięśniowej może wymagać nawet trzech miesięcy.2 Po uzyskaniu odpowiedniej odpowiedzi dawkę kortykosteroidów stopniowo się zmniejsza. Całkowity czas terapii kortykosteroidami wynosi zazwyczaj 9-12 miesięcy.2
Należy pamiętać, że długotrwałe stosowanie wysokich dawek kortykosteroidów może wiązać się z poważnymi działaniami niepożądanymi, takimi jak osteoporoza, zwiększona podatność na infekcje, cechy cushingoidalne czy wtórna cukrzyca.9 Stosowanie wysokich dawek glikokortykosteroidów przez ponad sześć tygodni może również prowadzić do miopatii posteroidowej.2
Leki immunosupresyjne i oszczędzające steroidy
W celu zmniejszenia dawki i działań niepożądanych kortykosteroidów stosuje się leki immunosupresyjne, często określane jako leki oszczędzające steroidy. Najczęściej stosowanymi lekami w tej grupie są:16
- Metotreksat (Trexall) – zazwyczaj stosowany jako lek pierwszego wyboru w terapii łączonej z kortykosteroidami10
- Azatiopryna (Azasan, Imuran) – alternatywa dla metotreksatu1
- Mykofenolan mofetylu (Cellcept) – szczególnie skuteczny w przypadku zajęcia płuc1
- Cyklosporyna – stosowana w wybranych przypadkach11
- Takrolimus – może być skuteczny w przypadkach opornych na leczenie6
Leki immunosupresyjne są często wdrażane już na początku leczenia, równocześnie z kortykosteroidami, szczególnie w przypadkach ciężkiej miopatii, przewlekłych schorzeń (np. cukrzycy) lub poważnych powikłań pozamięśniowych (np. śródmiąższowej choroby płuc, dysfunkcji przełyku).9 Należy jednak pamiętać, że pełen efekt terapeutyczny tych leków może być widoczny dopiero po kilku miesiącach stosowania.8
Immunoglobulina dożylna (IVIg)
Immunoglobulina dożylna (IVIg) to oczyszczony produkt krwiopochodny zawierający zdrowe przeciwciała od tysięcy dawców krwi. Przeciwciała te mogą blokować szkodliwe przeciwciała atakujące mięśnie i skórę w dermatomyositis.12 W lipcu 2021 roku FDA zatwierdziła preparat OCTAGAM 10% (IVIg) jako pierwszy oficjalnie zatwierdzony lek do leczenia dermatomyositis u osób dorosłych.513
IVIg jest stosowana szczególnie w przypadkach opornych na standardowe leczenie lub w ciężkim przebiegu choroby, zwłaszcza gdy występują zaburzenia połykania.8 Leczenie IVIg jest podawane w infuzji dożylnej, a zabiegi mogą wymagać regularnego powtarzania, zwykle co 4 tygodnie, aby efekty utrzymywały się.1214 Chociaż terapia ta jest kosztowna, wykazuje dobrą skuteczność zarówno w leczeniu objawów mięśniowych, jak i skórnych.15
Leki biologiczne i terapie celowane
W przypadkach opornych na standardowe leczenie stosuje się również leki biologiczne i inne terapie celowane:616
- Rytuksymab (Rituxan) – przeciwciało chimeryczne skierowane przeciwko komórkom B CD20+, stosowane w przypadkach opornych na standardowe leczenie176
- Inhibitory JAK (np. tofacytynib, baricytynib) – badania pokazują obiecujące wyniki w leczeniu dermatomyositis1819
- Apremilast – badany jako terapia dodatkowa z pozytywnymi wynikami19
- Lenabasum – kannabinoidowy agonista receptora typu 2, badany w leczeniu dermatomyositis20
Warto zaznaczyć, że obecnie trwają badania kliniczne nad nowymi terapiami, takimi jak brepocytynib (Priovant Therapeutics i Pfizer), HIZENTRA (CSL Behring), ULTOMIRIS (ravulizumab, AstraZeneca), efgartigimod (Argenx) oraz PF-06823859 (anty-beta interferon, Pfizer).21
Leczenie zmian skórnych
Leczenie zmian skórnych w dermatomyositis często stanowi wyzwanie i wymaga podejścia wielokierunkowego:22
1. Ochrona przeciwsłoneczna – ponieważ ekspozycja na promieniowanie UV może zaostrzać objawy skórne, zaleca się:23
- Unikanie ekspozycji na słońce
- Stosowanie kremów z filtrem SPF 30 lub wyższym
- Noszenie odzieży ochronnej
2. Leki miejscowe:24
- Miejscowe kortykosteroidy (np. krem z triamcynolonem 0,1%)
- Miejscowe inhibitory kalcyneuryny (np. maść z takrolimusem 0,1%)
3. Leki ogólne stosowane w leczeniu zmian skórnych:2224
- Leki przeciwmalaryczne (np. hydroksychlorochina/Plaquenil) – skuteczne w leczeniu uporczywej wysypki, choć u pacjentów z dermatomyositis mogą wystąpić skórne działania niepożądane częściej niż u pacjentów z SLE1719
- Metotreksat – często stosowany jako lek pierwszego wyboru, jeśli leki przeciwmalaryczne zawiodą lub są przeciwwskazane22
- Mykofenolan mofetylu – skuteczny w leczeniu zmian skórnych8
- IVIg – może być skuteczna w ciężkich przypadkach zmian skórnych opornych na standardowe leczenie22
4. W przypadku świądu:10
- Leki przeciwhistaminowe (np. hydroksyzyna, doksepina)
Leczenie wapnicy (calcinosis)
Wapnica (odkładanie się złogów wapnia w tkankach) jest częstym powikłaniem dermatomyositis, szczególnie w postaci młodzieńczej. W leczeniu tego powikłania stosuje się:2225
- Diltiazem – bloker kanału wapniowego, który może przyczyniać się do stopniowego ustępowania wapnicy22
- Kolchicyna – może być pomocna w leczeniu wapnicy25
- Alendronian – bisfosfonian badany w leczeniu wapnicy25
- Warfaryna – może wykazywać potencjalne korzyści w leczeniu wapnicy25
- Chirurgiczne usunięcie ogniskowych obszarów wapnicy, szczególnie tych, które są bolesne lub powodują nawracające infekcje1225
Fizjoterapia i rehabilitacja w leczeniu dermatomyositis
Fizjoterapia odgrywa kluczową rolę w leczeniu dermatomyositis, pomagając w utrzymaniu i poprawie siły mięśniowej oraz zakresu ruchu. Powinna być rozpoczęta, gdy tylko objawy są pod kontrolą.26
Badania naukowe potwierdzają, że odpowiednio dobrane ćwiczenia fizyczne są bezpieczne i korzystne dla pacjentów z dermatomyositis:27
- Trening rozpoczęty 2-3 tygodnie po ostrym zaostrzeniu choroby jest bezpieczny i korzystny
- Ćwiczenia mogą częściowo zapobiegać zanikowi mięśni spowodowanemu brakiem aktywności
- Intensywny trening aerobowy może poprawić siłę, równowagę, wydolność w czynnościach życia codziennego i prędkość chodzenia
- Intensywny trening mięśniowy może zmniejszyć upośledzenie i zwiększyć poziom aktywności bez zwiększania stanu zapalnego mięśni
Program fizjoterapii powinien być dostosowany do indywidualnych potrzeb pacjenta i może obejmować:1726
- Ćwiczenia rozciągające i wzmacniające mięśnie
- Ćwiczenia poprawiające zakres ruchu stawów
- Ćwiczenia poprawiające koordynację
- Aktywności takie jak szybki marsz, pływanie, joga czy tai chi
Z czasem fizjoterapeuta może zaproponować bardziej wymagające ćwiczenia. Fizjoterapia i regularne ćwiczenia poprawiają przepływ krwi, pomagając w procesie gojenia, a także mogą poprawić ogólne samopoczucie.26
Terapia mowy i porady dietetyczne
Gdy dermatomyositis wpływa na mięśnie odpowiedzialne za połykanie, konieczne może być wdrożenie dodatkowych form terapii:1712
Terapia mowy
Terapia mowy może pomóc pacjentom, u których występują zaburzenia połykania z powodu osłabienia mięśni gardła. Logopeda może nauczyć technik kompensacyjnych, które ułatwiają bezpieczne połykanie i zmniejszają ryzyko zakrztuszenia oraz aspiracji.1728
Porady dietetyczne
Konsultacja z dietetykiem może być korzystna dla pacjentów z trudnościami w żuciu i połykaniu. Dietetyk może pomóc w opracowaniu diety zawierającej pokarmy łatwe do spożycia, a jednocześnie zapewniające odpowiednią wartość odżywczą.12 Szczególnie istotne jest to w późniejszych stadiach choroby, gdy zaburzenia połykania mogą się nasilić.12
Ponadto, szczególną uwagę należy zwrócić na zdrową dietę podczas przyjmowania kortykosteroidów, które mogą prowadzić do znacznego przyrostu masy ciała, co może powodować dodatkowe obciążenie dla już osłabionych mięśni.29
Leczenie Juvenille Dermatomyositis (postaci młodzieńczej)
Leczenie młodzieńczego dermatomyositis (JDM) opiera się na podobnych zasadach jak leczenie postaci dorosłych, jednak istnieją pewne różnice. Dzieci z JDM są leczone lekami immunosupresyjnymi, takimi jak steroidy, metotreksat i IVIg.30
Stowarzyszenie Childhood Arthritis and Rheumatology Research Alliance (CARRA) opracowało protokoły kliniczne leczenia umiarkowanego/ciężkiego JDM. Każdy protokół obejmuje prednizon lub dożylny metyloprednizolon w połączeniu z metotreksatem.31 Metotreksat może skrócić czas leczenia i zmniejszyć całkowitą dawkę steroidów, minimalizując ryzyko działań niepożądanych.31
W zależności od zajęcia narządów, można stosować różne leki immunosupresyjne, takie jak cyklosporyna i inhibitory TNF. Istnieją również doniesienia, że inhibitory JAK mogą być korzystne u młodzieży, podobnie jak w leczeniu dorosłych.30
Co ważne, badania sugerują, że agresywne leczenie JDM, mające na celu szybkie i całkowite opanowanie osłabienia mięśni i stanu zapalnego, poprawia wyniki i zmniejsza powikłania związane z chorobą. W ponad połowie dzieci leczonych w ten sposób (28 z 49) uzyskano długotrwałą remisję bez leków, średnio 38 miesięcy od momentu rozpoznania.32
Monitorowanie i długoterminowa opieka
Dermatomyositis jest chorobą przewlekłą, która często wymaga długoterminowej opieki i monitorowania. Zaleca się regularne kontrole obejmujące:29
- Monitorowanie poziomu kinazy kreatynowej (CK)
- Ocenę siły mięśniowej
- Ocenę czynności oddechowej
- Ocenę funkcji połykania
- Ocenę funkcji serca
Pacjenci przyjmujący długotrwale kortykosteroidy powinni otrzymywać suplementację wapniem i witaminą D w celu zapobiegania osteoporozie. U kobiet po menopauzie można rozważyć hormonalną terapię zastępczą (HTZ). W niektórych przypadkach można przepisać bisfosfoniany w celu zapobiegania osteoporozie wywołanej steroidami.33
Pacjenci przyjmujący wysokie dawki glikokortykosteroidów lub leki immunosupresyjne powinni być rozważeni do profilaktyki przeciwko Pneumocystis Jirovecii za pomocą trimetoprimu i sulfametoksazolu.23
Wszyscy pacjenci powinni otrzymać odpowiednie szczepienia przed rozpoczęciem leczenia immunosupresyjnego.23
Rokowanie i przebieg choroby
Rokowanie w dermatomyositis jest zróżnicowane i zależy od wielu czynników, takich jak wiek zachorowania, obecność powikłań narządowych czy odpowiedź na leczenie. Nowoczesne metody leczenia znacząco poprawiły rokowanie – śmiertelność zmniejszyła się z około 50% do mniej niż 10%.10
W przypadku JDM, przy wczesnym i agresywnym leczeniu, około 90% dzieci leczonych w specjalistycznych ośrodkach osiąga pełną remisję.32 U dorosłych około 20% pacjentów osiąga remisję, podczas gdy 80% ma przewlekły przebieg choroby.34
Wśród pacjentów, którzy przeżywają, 65% osiąga normalną siłę mięśniową, 34% ma łagodne upośledzenie, a 16% nie ma niepełnosprawności.34
Należy podkreślić, że wczesna diagnoza i leczenie są kluczowe dla osiągnięcia najlepszych wyników. Regularne monitorowanie i dostosowywanie planu leczenia do zmieniających się potrzeb pacjenta może znacząco poprawić jakość życia i zapobiec długoterminowym powikłaniom.35
Podsumowanie leczenia dermatomyositis
Leczenie dermatomyositis wymaga podejścia wielodyscyplinarnego, obejmującego reumatologów, dermatologów, neurologów, pulmonologów, fizjoterapeutów oraz terapeutów mowy i języka.3 Chociaż nie ma obecnie leku, który całkowicie wyleczyłby tę chorobę, dostępne opcje terapeutyczne mogą skutecznie kontrolować objawy, poprawiać siłę mięśniową i funkcję narządów oraz zapobiegać powikłaniom.
Podstawą leczenia są glikokortykosteroidy, często w połączeniu z lekami immunosupresyjnymi. W przypadkach opornych na leczenie lub ciężkiego przebiegu choroby, stosuje się immunoglobulinę dożylną i leki biologiczne. Fizjoterapia, terapia mowy i porady dietetyczne stanowią ważne uzupełnienie leczenia farmakologicznego.
Kluczowe znaczenie ma wczesne rozpoczęcie leczenia i regularne monitorowanie, aby dostosować plan terapeutyczny do zmieniających się potrzeb pacjenta. Z odpowiednim leczeniem i opieką, wielu pacjentów z dermatomyositis może osiągnąć znaczną poprawę objawów, a niektórzy nawet długotrwałą remisję.
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 Dermatomyositis – Diagnosis & treatment – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/dermatomyositis/diagnosis-treatment/drc-20353192
There’s no cure for dermatomyositis, but treatment can improve your skin and your muscle strength and function. […] Medications used to treat dermatomyositis include: […] Corticosteroids. Drugs such as prednisone (Rayos) can control dermatomyositis symptoms quickly. But prolonged use can have serious side effects. So your doctor, after prescribing a relatively high dose to control your symptoms, might gradually reduce the dose as your symptoms improve. […] Corticosteroid-sparing agents. When used with a corticosteroid, these drugs can decrease the dose and side effects of the corticosteroid. The two most common medications for dermatomyositis are azathioprine (Azasan, Imuran) and methotrexate (Trexall). Mycophenolate mofetil (Cellcept) is another medication used to treat dermatomyositis, particularly if the lungs are involved.
- #2 Dermatomyositis – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK558917/
The goals of managing dermatomyositis are focused on treating muscle weakness, skin disease, and addressing any other underlying complications. […] The first-line treatment of muscle disease in dermatomyositis is systemic glucocorticoids with or without immunosuppressants. Although there is no standard systemic steroid regimen specified for dermatomyositis, the general principles of therapy are the same. Initially, prednisolone is given at high doses for the first few months until the muscle enzyme levels decline, and muscle strength improves. During this time, patients should be regularly evaluated for an adequate response, keeping in mind that it takes approximately six weeks for muscle enzymes to normalize. Also, it may require as long as three months for muscle weakness to improve. Once an adequate response occurs, the administration of systemic steroids is gradually tapered off over time. The total duration of therapy with systemic steroids usually spans between nine and twelve months. It is important to note that administering high dose glucocorticoids for more than six weeks may lead to glucocorticoid myopathy.
- #3 Dermatomyositis: An Acute Flare and Current Treatmentshttps://pmc.ncbi.nlm.nih.gov/articles/PMC6582284/
The symptomatology experienced in this condition includes but not limited to fatigue, reduced mobility, and dysphagia. […] Systemic corticosteroid is the first-line treatment for DM; however, other agents such as anti-malaria, IVIG, and immunosuppressive therapies have been used successfully. […] The goal of treatment is to reduce inflammation and vasculitis and invariably minimize symptomatology and improve quality of life of the patients. […] The mainstay of therapy for dermatomyositis is the administration of steroids. […] The treatment of choice is high dose of oral prednisone which must be initiated early to improve muscle weakness. […] In situations where prednisone cannot be used, second-line agents such as methotrexate and azathioprine will be appropriate. […] Azathioprine and IVIG are effective agents in the management of muscle involvement of dermatomyositis. […] The use of surgical and pharmacotherapy (diltiazem) was effective in reducing dystrophic calcinosis. […] The treatment of dermatomyositis involves a multidisciplinary approach, as evidenced in this case.
- #4 Navigating the Challenges in Dermatomyositis Treatmenthttps://www.delveinsight.com/blog/dermatomyositis-treatment-outlook
There is no cure for dermatomyositis, but medicines can minimize symptoms, reduce inflammation and vasculitis, and enhance the patients quality of life. The goals of managing dermatomyositis are focused on treating muscle weakness and skin disease and addressing any other underlying complications, including cardiac, pulmonary, gastrointestinal, joints, and malignancies. The recent approval of OCTAGAM as well as the presence of several management guidelines, including the British Society for Rheumatology guideline, the Japanese Society of Rheumatology guideline, and others, have simplified the treatment landscape for dermatomyositis. The first-line treatment for dermatomyositis includes the use of systemic glucocorticoids with or without immunosuppressants. Other dermatomyositis medications, including antimalarial drugs, antibiotics, and topical ointments, are given individually or in combination to eliminate symptoms. Further, rituximab, mycophenolate mofetil, calcineurin inhibitors, IVIG, and cyclophosphamide are preferred for resistant cases, patients unresponsive to initial treatment.
- #5 Dermatomyositis | Myositis Support and Understandinghttps://understandingmyositis.org/myositis/dermatomyositis/
Since steroids, especially in high doses and over prolonged periods, are known to have many adverse side effects and may cause added health issues, the introduction of steroid-sparing medications should be considered. […] Steroid-sparing agents, such as immunosuppressive drugs and Disease-Modifying Antirheumatic Drugs (DMARDs), have an added benefit as they often reduce or eliminate the need for steroids while also improving the symptoms of DM. […] Some commonly used immunosuppressive drugs include azathioprine (Imuran), mycophenolate mofetil (Cellcept), and Tacrolimus (Prograf). […] Hydroxychloroquine (Plaquenil) is one of the commonly used drugs in this class and is used to treat the skin symptoms of DM. […] On July 20, the US Food and Drug Administration (FDA) granted approval to Octagam 10% for the treatment of adult dermatomyositis.
- #6 Dermatomyositis Treatment & Management: Approach Considerations, General Measures, Treatment of Muscle Diseasehttps://emedicine.medscape.com/article/332783-treatment
The mainstay of therapy for the muscle disease is systemically administered corticosteroids. Traditionally, prednisone (0.5-2 mg/kg/d) up to a dose of 60 mg/d is given as initial therapy. The drug should be slowly tapered to avoid relapse of the disease. […] Generally, methotrexate, mycophenolate mofetil, or azathioprine are used first line as glucocorticoid-sparing agents for muscle involvement. […] For refractory cases, the use of monthly high-dose intravenous immune globulin (IVIG) for 6 months has proved beneficial in the short term. […] Rituximab, a chimeric antibody directed against CD20+ B cells, may be effective, but results have been mixed. […] The calcineurin inhibitor tacrolimus appears to be effective, safe, and well tolerated in patients with dermatomyositis that is refractory to other treatments.
- #7 Dermatomyositis: Practical Guidance and Unmet Needs | ITThttps://www.dovepress.com/dermatomyositis-practical-guidance-and-unmet-needs-peer-reviewed-fulltext-article-ITT
Dermatomyositis Treatment has remained largely unchanged over the past several decades. […] Although many dermatomyositis patients can be effectively treated with current options, either as monotherapy or with combination regimens, there is a need for more targeted and effective DM therapies, in general, and for MDA5(+) dermatomyositis-associated rapidly progressive interstitial lung disease. […] Systemic glucocorticoids are the mainstay treatment for DM, especially when myositis is present. […] These are typically prescribed at doses of 0.5-1.0 mg/kg/day initially in an outpatient setting, with tapering based on treatment response. […] When patients are hospitalized because of more severe myositis, higher doses of IV glucocorticoids are often administered in attempts to promptly halt further skeletal muscle damage.
- #8 Dermatomyositis: Practical Guidance and Unmet Needs | ITThttps://www.dovepress.com/dermatomyositis-practical-guidance-and-unmet-needs-peer-reviewed-fulltext-article-ITT
Not uncommonly, pulse methylprednisolone therapy (1000mg daily for 3-4 consecutive days) is given with this goal in mind. […] Typically, steroid-sparing immunomodulatory agents are immediately prescribed along with glucocorticoids at time of diagnosis with the goal of minimizing cumulative glucocorticoid exposure because DM is a chronic disease and most steroid-sparing immunomodulatory agents require several months before optimal effects can be expected. […] The most widely accepted first-line systemic treatments for moderate-to-severe cutaneous disease are MMF and methotrexate, although other previously mentioned therapies may also be used. […] Intravenous immunoglobulin (IVIG) has become a favored treatment for severe/refractory DM. […] In patients with severe myositis, especially when dysphagia is present, IVIG is typically initiated immediately and often along with pulse glucocorticoid therapy. […] Rituximab is another option for refractory DM. […] The choice of therapy may depend on patient comorbidities and/or DM manifestations. […] Vasculopathic cutaneous lesions, as observed in patients with MDA5-associated DM, are challenging to treat.
- #9 Dermatomyositis – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK558917/
Immuno-suppressants help to alleviate the adverse effects of long-term systemic steroids. Such adverse effects could include osteoporosis, increased susceptibility to infections, cushingoid features, and secondary diabetes. Immunosuppressant administration can be initiated along with steroids in cases of severe myopathy, chronic medical conditions (e.g., diabetes), and profound extra-muscular complications (e.g., interstitial lung disease, esophageal dysfunction). First-line agents include azathioprine and methotrexate. […] Patients who do not respond satisfactorily to therapy with steroids and azathioprine or methotrexate are considered resistant. Treatment options for resistant cases include rituximab, mycophenolate mofetil, calcineurin inhibitors, intravenous immunoglobulin (IVIG), and cyclophosphamide.
- #10 Dermatomyositis | AAFPhttps://www.aafp.org/pubs/afp/issues/2001/1101/p1565.html
For control of severe pruritus, antihistamines (such as hydroxyzine [Atarax] or doxepin [Sinequan]) are recommended. […] For further control of the erythematous and pruritic skin changes, a class I (super-high potency) or class II (high potency) topical corticosteroid is recommended. […] Prednisone remains the initial oral pharmacologic agent, given in a single daily dose of 0.5 to 1.5 mg per kg until the serum creatine kinase (CK) level is normalized and then slowly tapered over the following 12 months. […] If no improvement in objective muscle strength occurs after three months of prednisone therapy, other immunosuppressive therapy should be considered. […] Methotrexate (Rheumatrex) is considered the first-line adjuvant therapy in patients who do not respond to prednisone. […] Serum immunoglobulin has been used with success for treatment of patients with refractory dermatomyositis.
- #10 Dermatomyositis | AAFPhttps://www.aafp.org/pubs/afp/issues/2001/1101/p1565.html
Dermatomyositis is an idiopathic inflammatory myopathy with characteristic skin manifestations. […] Patient management includes careful evaluation for underlying malignancy and liberal use of physical therapy, antihistamines, sunscreen and oral corticosteroids. […] The therapeutic goal is to maintain function and prevent or minimize sequelae. […] Modern therapy has reduced mortality from near 50 percent to less than 10 percent. […] The treatment regimen must be instituted early and requires a team approach between the physical therapist, dermatologist and family physician. […] Physical therapy is directed at preventing atrophy and contractures, and is particularly necessary in patients with calcinosis and muscle involvement. […] The use of a broad-spectrum sunscreen is recommended in all patients with dermatomyositis and has the greatest benefit in patients who are photosensitive.
- #11 Dermatomyositis – Myositis Association Australiahttps://myositis.org.au/myositis/about-myositis/dermatomyositis/
Other drugs to suppress the immune system are often given to help reduce the dose of corticosteroid needed. These include: Methotrexate, Azathioprine, Cyclosporine, Mycophenolate mofetil. […] Other drugs that may help some people include cyclophosphamide and tacrolimus. Although most people tolerate these medications, some may experience side effects so it is important to be aware of the potential risks and benefits of each drug and work with your doctor to find the ideal treatment for you. […] IVIg therapy is usually considered in severe cases that do not respond to other treatments but is being used more frequently to treat DM. This is an infusion of immunoglobulins (antibodies) extracted from a large pool of donated blood. It is thought that these extra antibodies confuse and interfere with the body’s immune system.
- #12 Dermatomyositis – Diagnosis & treatment – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/dermatomyositis/diagnosis-treatment/drc-20353192
Dietetic assessment. Later in the course of dermatomyositis, chewing and swallowing can become more difficult. A registered dietitian can teach you how to prepare easy-to-eat foods. […] Intravenous immunoglobulin (IVIg). IVIg is a purified blood product that contains healthy antibodies from thousands of blood donors. These antibodies can block the damaging antibodies that attack muscle and skin in dermatomyositis. Given as an infusion through a vein, IVIg treatments are expensive and might need to be repeated regularly for the effects to continue. […] Surgery. Surgery might be an option to remove painful calcium deposits and prevent recurrent skin infections.
- #13 FDA Approves First Proven Treatment for Dermatomyositis – CSI Pharmacyhttps://csipharmacy.com/fda-approves-first-proven-treatment-for-dermatomyositis/
Octapharma announced that Octagam 10%, an intravenous immune globulin (IVIG), has been approved by the FDA as a therapy for adults with dermatomyositis. […] On July 16, however, the Food and Drug Administration (FDA) approved an intravenous immune globulin (IVIG) therapy called Octagam10% for use by adults with DM. […] This is fantastic news for the myositis community, says Dr. Rohit Aggarwal. Octagam is the first proven FDA approved treatment for a myositis indication. […] Dr. Aggarwal, an internationally recognized myositis expert, was the principal investigator for the Phase 3 clinical trial called ProDERM that demonstrated that Octagam 10% caused significant improvement in DM skin and muscle symptoms as well as other disease criteria. […] The FDA approval of Octagam 10% as a safe, tolerable and efficacious treatment for dermatomyositis in adults is exciting news for patients who previously relied on unapproved treatments, said Octapharma USA President Flemming Nielsen.
- #14 Myositis Skin Care and Treatment – The Myositis Associationhttps://www.myositis.org/myositis-library/live-discussions/myositis-skin-care-treatment/
Dr. Werth: There is no easy way to prevent skin calcifications. Sometimes aggressive treatment of dermatomyositis may be helpful. There is no easy way to treat them. Sometimes cutting them out can be helpful, but that is not a good approach if you have many of them. […] Dr. Werth: Sometimes the rash can improve with plaquenil. The dose of CellCept might need to be as high as 3000 mg/day. IVIG works best if given every 4 weeks, so if you get better for a while and then worse before the next IVIG treatment, you might need to get the IVIG more often. […] Dr. Werth: The outcome of surgery will be better if you can get off steroids. It might be worth trying plaquenil. Sometimes if plaquenil doesn’t work, then quinacrine can be added to plaquenil. Another possible treatment for skin if methotrexate isn’t working is mycophenolate mofetil. Sometimes the dose of methotrexate needed can be 20 or even 25 mg a week.
- #15 Exploring IVIG for Dermatomyositis | AmeriPharma⢠Specialtyhttps://ameripharmaspecialty.com/ivig/exploring-ivig-therapy-for-dermatomyositis/
Dermatomyositis is a rare autoimmune disease that can affect children and adults alike. There is no cure for dermatomyositis yet. However, you can avoid its debilitating consequences by receiving treatment. One effective therapy for dermatomyositis is IVIG. […] IVIG therapy for dermatomyositis is not the only option. […] IVIG treatment for dermatomyositis is a form of infusion therapy that includes pooled plasma containing antibodies collected from hundreds of healthy donors. […] When people with dermatomyositis receive IVIG infusions, these antibodies will enter their bodies. They will target the autoantibodies that are hurting the skin and muscles. This mechanism of action will modulate their immune systems and reduce symptoms. […] Due to its high cost, physicians often reserve IVIG for people resistant to other treatments. However, IVIG is a very effective treatment. Some doctors even recommend it as first-line therapy.
- #16 Dermatomyositis – Wikipediahttps://en.wikipedia.org/wiki/Dermatomyositis
No cure for dermatomyositis is known, but the symptoms can be treated. Options include medication, physical therapy, exercise, heat therapy (including microwave and ultrasound), orthotics and assistive devices, and rest. The standard treatment for dermatomyositis is a corticosteroid drug, given either in pill form or intravenously. Immunosuppressant drugs, such as azathioprine and methotrexate, may reduce inflammation in people who do not respond well to prednisone. Periodic treatment using intravenous immunoglobulin can also improve recovery. Other immunosuppressive agents used to treat the inflammation associated with dermatomyositis include cyclosporine A, cyclophosphamide, and tacrolimus. […] Physical therapy is usually recommended to prevent muscle atrophy and to regain muscle strength and range of motion. Many individuals with dermatomyositis may need a topical ointment, such as topical corticosteroids, for their skin disorder. They should wear high-protection sunscreen and protective clothing. Surgery may be required to remove calcium deposits that cause nerve pain and recurrent infections.
- #17 Dermatomyositis – Diagnosis & treatment – Mayo Clinichttps://www.mayoclinic.org/diseases-conditions/dermatomyositis/diagnosis-treatment/drc-20353192
Rituximab (Rituxan). More commonly used to treat rheumatoid arthritis, rituximab is an option if initial therapies don’t control your symptoms. […] Antimalarial medications. For a persistent rash, your doctor might prescribe an antimalarial medication, such as hydroxychloroquine (Plaquenil). […] Sunscreens. Protecting your skin from sun exposure by applying sunscreen and wearing protective clothing and hats is important for managing the rash of dermatomyositis. […] Depending on the severity of your symptoms, your doctor might suggest: […] Physical therapy. A physical therapist can show you exercises to help maintain and improve your strength and flexibility and advise you about an appropriate level of activity. […] Speech therapy. If your swallowing muscles are affected, speech therapy can help you learn how to compensate for those changes.
- #18 Navigating the Challenges in Dermatomyositis Treatmenthttps://www.delveinsight.com/blog/dermatomyositis-treatment-outlook
Numerous case reports on tofacitinib in dermatomyositis provide compelling evidence supporting the potential efficacy of brepocitinib. The pathobiology of dermatomyositis is influenced by dysregulation in cytokines, with signaling mediated by TYK2 and JAK1, particularly Type I interferon. This suggests that brepocitinib may exhibit greater efficacy than tofacitinib. Comparative analyses between brepocitinib (30 mg daily) and tofacitinib in various autoimmune conditions indicate that dosing brepocitinib at 30 mg daily could yield clinically meaningful efficacy in dermatomyositis. […] In the Phase I trial, PF-06823859 demonstrated an acceptable safety, tolerability, and PK profile that supports clinical development for treating disorders associated with increased interferon levels, such as dermatomyositis or systemic lupus erythematosus. In the Phase II trial, the drug improved clinical parameters like Manual Muscle Testing-8, Physician Global Assessment, patient-reported outcomes, and Health Assessment Questionnaire Disability Index in patients with muscle-predominant refractory moderate-to-severe dermatomyositis.
- #19 Adult-onset dermatomyositishttps://dermnetnz.org/topics/adult-onset-dermatomyositis
What is the treatment for adult-onset dermatomyositis? […] General measures for the treatment of dermatomyositis include: Photoprotection and sun-avoidance as ultraviolet radiation exposure can exacerbate cutaneous symptoms. Bedrest. Physical therapy and activity. Elevating the bedhead for those with difficulty swallowing. […] Measures for more specific treatment of dermatomyositis can include: Topical steroids. Topical calcineurin inhibitors. Systemic corticosteroid such as prednis(ol)one. Hydroxychloroquine, although cutaneous adverse effects are more common in dermatomyositis than in SLE. Intravenous immunoglobulin (IVIG), FDA-approved in 2021. Steroid-sparing agents and other immunosuppressants, including: Methotrexate, Cyclophosphamide, Tacrolimus, Azathioprine, Mycophenolate mofetil, Biological treatments. Diltiazem and colchicine may reduce calcinosis. […] The JAK inhibitor, baricitinib, was studied in a small, 12-patient, open-label trial with clinically significant improvement seen in as early as week 4 of the study. Apremilast was also studied in 8 adult women as add-on therapy with positive results.
- #20 Congress of Clinical Rheumatology Annual Meetinghttps://www.healio.com/news/rheumatology/20220515/hydroxychloroquine-no-longer-best-treatment-for-systemic-dermatomyositis
Hydroxychloroquine may no longer be the optimal treatment option for systemic dermatomyositis, according to data presented at the Congress of Clinical Rheumatology East. […] In her presentation, Avery H. LaChance, MD, MPH, director of the Connective Tissue Diseases Clinic at Brigham and Womens Hospital, and assistant professor of dermatology at Harvard Medical School, said she has moved on from hydroxychloroquine as a first-line systemic therapy for systemic dermatomyositis. […] The treatment of skin disease in dermatomyositis really requires a multimodal approach, she said. […] Rather, patients with systemic disease may benefit from intravenous immunoglobulin, Janus kinase (JAK) inhibition or gabapentin to treat itch or pain. Rituximab (Rituxan, Genentech) can be effective in the muscles and lungs, while lenabasum is an emerging option, LaChance added.
- #21 Navigating the Challenges in Dermatomyositis Treatmenthttps://www.delveinsight.com/blog/dermatomyositis-treatment-outlook
In the last two decades, a substantial advancement in research has yielded new insights into the genetic risk factors and mechanisms underlying dermatomyositis, paving the way for promising therapeutic approaches to address the urgent need for effective dermatomyositis treatments. Priovant Therapeutics and Pfizers brepocitinib, CSL Behrings HIZENTRA, AstraZenecas ULTOMIRIS (ravulizumab), Argenxs efgartigimod, and Pfizers PF-06823859 (anti-beta interferon), are being actively developed, with these dermatomyositis therapies currently in Phase III and II/III. Many of these dermatomyositis treatments, already approved for other indications, demonstrate a favorable safety profile, and their exploration for dermatomyositis is grounded in evidence from off-label studies or their effectiveness in similar indications.
- #22 Dermatomyositis Treatment & Management: Approach Considerations, General Measures, Treatment of Muscle Diseasehttps://emedicine.medscape.com/article/332783-treatment
Therapy of cutaneous disease of dermatomyositis is often difficult. […] First-line therapy is to recognize that the patient is photosensitive and to prescribe sun avoidance and sun protection measures, including broad-spectrum sunscreens and photoprotective clothing. […] Methotrexate is often considered first-line systemic therapy if antimalarials fail or are contraindicated. […] Azathioprine has been reported to be effective for muscle involvement, but there is a paucity of literature regarding cutaneous response. […] Intravenous immune globulin (IVIG) has benefited muscle involvement and cleared the skin lesions in the patients in whom it was used. […] Subcutaneous immunoglobulin (SCIg) infusion has also been effective in dermatomyositis. […] The use of the calcium channel blocker diltiazem (240 mg bid) is reportedly associated with gradual resolution of calcinosis in a small number of cases. […] Some patients with localized areas of calcinosis may wish to have the calcinotic nodules surgically removed, particularly if they are tender.
- #23 Dermatomyositis – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK558917/
Skin disease in dermatomyositis is managed with general measures, physiotherapy, and medical therapy. These measures should include sun-protective measures, such as sunlight avoidance, the use of sun-protective clothing and sunscreen with sun protective factor (SPF) of 30 or higher. […] Physical therapy and rehabilitation play an essential role in management. Patients with mild disease should be encouraged to participate in active exercise programs. Range of motion exercises can help in preventing contractures. […] Anti-resorptive therapy may be indicated in patients on long term systemic corticosteroids in order to prevent osteoporosis. Patients on high dose systemic glucocorticoids or immunosuppressants should be considered for prophylaxis against Pneumocystis Jirovecii with trimethoprim and sulfamethoxazole. Lastly, all patients should receive the appropriate immunizations prior to receiving immunosuppressants.
- #24 Dermatomyositis Information for Patients | RDShttps://www.rheumaderm-society.org/dermatomyositis-information-for-patients/
Some treatment options for the skin rashes of dermatomyositis that your dermatologist may discuss with you include: Sun protective measures including daily sunscreen, sun-protective clothing, and sun avoidance strategies […] Topical steroid creams and ointments (e.g., triamcinolone 0.1% cream) […] Topical calcineurin inhibitors (e.g., tacrolimus 0.1% ointment) […] Antimalarial medications (e.g., hydroxychloroquine) […] Immune-suppressing medications (e.g., methotrexate, mycophenolate mofetil, azathioprine) […] IVIg this can be effective for severe skin disease that does not respond to more standard treatments noted above, and can be used in patients that have an associated cancer or infection (as this medication does not suppress your immune system).
- #25 Dermatomyositis: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/332783-overview
Therapy for the muscle component of dermatomyositis involves the use of corticosteroids, typically with an immunosuppressive agent. Therapy for the skin disease includes the following, among other options: […] Medications used in the management of dermatomyositis include the following: […] Rituximab may be useful in the treatment of muscle disease in dermatomyositis, and has had mixed results in treatment of skin disease. […] In addition to the medications listed above, diltiazem, colchicine, alendronate, and warfarin are among the medications that have shown potential benefit in treating calcinosis. Surgical excision of focal, tender calcinotic lesions is also considered a therapeutic option. […] General therapeutic measures may include the following: […] Surgical care is usually unnecessary in the management of dermatomyositis. However, some patients may benefit from surgical removal of localized areas of calcinosis, particularly those that are painful. […] See Treatment and Medication for more detail.
- #26 Physical Therapy for Dermatomyositis | NYU Langone Healthhttps://nyulangone.org/conditions/dermatomyositis/treatments/physical-therapy-for-dermatomyositis
Specialists at NYU Langone recommend physical therapy as an important part of treatment for muscle weakness associated with dermatomyositis. Therapy should be started as soon as symptoms are under control. […] Our neuromuscular specialists and rheumatologists, who manage conditions that cause painful swelling in joints and muscles, work with physical therapists at NYU Langones Rusk Rehabilitation to help you rebuild strength in weakened muscles. Physical therapy can restore range of motion in joints that may have become stiff with underuse. It can also increase flexibility and prevent further loss of muscle tissue. […] Over time, your physical therapist may suggest more rigorous exercises, such as brisk walking, swimming, yoga, or tai chi a low-impact activity that consists of simple body movements held for varying periods. Physical therapy and exercise improve blood flow, helping you heal. They can also boost your sense of wellbeing.
- #27 Physical Therapy for Myositis Patients: Strength & Mobilityhttps://www.hss.edu/conditions_physical-therapy-and-myositis.asp
Ms. Yanelli shared studies that demonstrated evidence-based research on the positive effects of exercise for people with dermatomyositis (DM) and polymyositis (DM). […] One study, called the Effects of Physical Exercise Shortly on an Acute Episode of Dermatomyositis/Polymyositis, found that: Physical training begun 2-3 weeks after an acute exacerbation of DM/PM seems to be safe and useful. Muscle atrophy, due to lack of activity, may be partially prevented. Level of disability can be decreased. […] In a study of DM/PM patients on the benefits of intensive aerobic training it was found that: There was improvement in strength, balance, performance in activities of daily living, and walking speed. […] In a further study investigating the benefits of intensive muscular training in patients with DM/PM, it was concluded that: Patients with chronic, stable DM and PM can perform intensive resistive exercise as described in this study – with the approval of a physician. Results may include reduced impairment and an increase in activity level without increased muscle inflammation.
- #28 Dermatomyositis: Symptoms, causes, and treatmenthttps://www.medicalnewstoday.com/articles/318081
Speech therapy: Some people with dermatomyositis may experience difficulty swallowing as a result of muscle weakness. Speech therapy can help a person build these muscles and reduce the risk for choking and aspiration. […] […] A person with dermatomyositis should also protect their skin from the sun’s damaging rays, which seem to worsen the condition’s symptoms. […] […] In rare instances, a person may require surgery to reduce the incidence of calcium deposits under the skin caused by dermatomyositis. People often undergo surgery when the deposits are causing extreme nerve pain.
- #29 Dermatomyositis – Myositis Association Australiahttps://myositis.org.au/myositis/about-myositis/dermatomyositis/
Careful attention to a healthy diet can help with the management of the symptoms, especially when taking corticosteroids as they can lead to significant weight gain which may cause extra strain on the muscles. […] Regular monitoring of creatine kinase (CK) levels, muscle strength, breathing, swallowing and heart function is recommended to allow any problems to be anticipated and treated.
- #30 Medical Management – Dermatomyositis – Diseases | Muscular Dystrophy Associationhttps://www.mda.org/disease/dermatomyositis/medical-management
Treatment plans are typically individualized to meet the needs of the affected person and ongoing monitoring is required to optimize the treatment protocol. In some cases, affected people may require prolonged maintenance therapy throughout their lives, while in others, therapy may be gradually withdrawn as symptoms resolve. Several years of treatment to suppress the immune system may be necessary to achieve these results. […] Children with juvenile dermatomyositis (JDM) are treated with immunosuppressive medications such as steroids, methotrexate, and IVIG. While some treatment options may be similar between adults and juveniles with dermatomyositis, depending on the organ involvement, different immunosuppressants such as cyclosporin and TNF inhibitors may be tried. There are also reports that JAK inhibitors may be beneficial in juveniles, similar to the treatment landscape in adults.
- #31 Juvenile Dermatomyositis | PM&R KnowledgeNowhttps://now.aapmr.org/dermatomyositis/
Patients who receive early aggressive treatment have better overall outcomes and fewer disease complications. The initial treatment plan is based upon the severity of presenting symptoms. […] The Childrens Arthritis and Rheumatology Research Alliance (CARRA) developed clinical protocols for treatment of moderate/severe JDM. Each protocol involves prednisone or intravenous methylprednisolone along with methotrexate. Methotrexate can shorten treatment time and decrease the total dose of steroids, minimizing the risk of side effects. Intravenous immune globulin (IVIG) is common as a second line agent in addition to mycophenolate mofetil and cyclosporine A, cyclophosphamide, tacrolimus, infliximab, and rituximab. These protocols are intended to guide clinicians, but treatment is individually tailored. Based on treatment, gastrointestinal prophylaxis and calcium and/or Vitamin D replacement may be recommended. Sunscreen is recommended because the JDM rash is photosensitive.
- #32 Juvenile Dermatomyositishttps://www.childrenshospital.org/conditions/juvenile-dermatomyositis
If your child has a visible rash or other skin problems relating to JDM, you may be wondering, Will my child look normal again? Fortunately, with treatment, most skin problems clear up, and your child will look and act like any other child his age. […] It’s important to keep in mind that most kids with JDM need to see a rheumatologist regularly. Most children come back to Children’s or visit a rheumatologist in their area at least once every other year once they are in remission. […] Our findings suggested that aggressive treatment of JDM aimed at achieving rapid, complete control of muscle weakness and inflammation improves outcomes and reduces disease-related complications. In more than one-half of the children whose disease was treated in this manner (28 of the 49), a prolonged, medication-free remission was attained an average of 38 months from the time of diagnosis. We concluded that aggressive management of juvenile dermatomyositis results in improved outcome and decreased incidence of calcinosis.
- #32 Juvenile Dermatomyositishttps://www.childrenshospital.org/conditions/juvenile-dermatomyositis
While there’s currently no known cure for juvenile dermatomyositis, we’ve found that using a combination of immunosuppressive therapies can put the disease into remission over time, especially if we work to get the condition under control as early as possible. […] We tailor treatment for your child’s juvenile dermatomyositis to the stage of his condition and his body’s initial response to treatment. Prescribed treatment methods may include: Medications, such as glucocorticosteroids and methotrexate, which treat the inflammatory disorder, and hydroxychloroquine, which treats the skin-related aspect of the condition (dermatomyositis) […] Here at Children’s, we have extensive experience in treating juvenile dermatomyositis. Within our Rheumatology Program, JDM is actually the most common condition we see (second only to arthritis). Compared to other institutions, which report patients in full remission at about 33 percent, nearly 90 percent of the kids treated at Children’s will wind up in full remission.
- #33 Dermatomyositis – Myositis Association Australiahttps://myositis.org.au/myositis/about-myositis/dermatomyositis/
If none of the treatments mentioned above are effective, the doctor will first reconfirm the diagnosis of DM. Then drugs that have been approved to treat other autoimmune conditions may be tried such as rituximab or alemtuzumab. […] Taking steroids can increase the risk of osteoporosis (brittle bones) so it is important to consider supplements of calcium and vitamin D, and hormone replacement therapy (HRT) in post-menopausal women. In some cases a group of drugs called bisphosphonates may be prescribed to help prevent steroid-induced osteoporosis. […] Avoidance of sun exposure during peak hours and use of sunscreen and protective clothing are recommended to avoid worsening the skin aspects of the disease. […] Physiotherapy is recommended to keep the joints supple and physiotherapists can supervise an appropriate exercise program. A gentle exercise program, such as hydrotherapy in a swimming pool, can help to maintain strength and wellbeing. Some people may need a cane, walker or even a wheelchair during acute flare-ups of DM and an occupational therapist and/or physiotherapist can help with arranging these.
- #34 Dermatomyositis – EyeWikihttps://eyewiki.org/Dermatomyositis
65% of patients who survive have normal strength, 34% have mild disability, and 16% have no disability. 20% of patients achieve remission, 80% have a chronic course. […] DM is an idiopathic, microangiopathic myopathy characterized by muscle weakness, Gottron papules and heliotrope rashes, and has a multi-factorial pathogenesis with multiple genetic, immunologic, and environmental factors that contribute.
- #35 Dermatomyositis: Symptoms, Causes & Treatmentshttps://my.clevelandclinic.org/health/diseases/15701-dermatomyositis
Managing your dermatomyositis symptoms will likely be a long-term process possibly for the rest of your life. […] If your provider, physical therapist or speech therapist gives you exercises, do them as often as they suggest. This will help keep your muscles as strong as possible. […] Take any medications as often as you should for as long as your provider prescribes. […] It might take a few months for your symptoms to improve after you start treatment. Most people living with dermatomyositis feel better as they regain their original levels of muscle strength after treatment. […] Talk to your provider about what to expect and when you should notice your symptoms getting better.