From the FDA Drug Label
3. Collagen Diseases During an exacerbation or as maintenance therapy in selected cases of: Systemic lupus erythematosus Systemic dermatomyositis (polymyositis) Acute rheumatic carditis
The next best option for treatment of dermatomyositis with scleroderma features, particularly with inflammatory polyarthritis, could be prednisone (PO), as it is indicated for the treatment of systemic dermatomyositis. However, it is essential to consider the patient's specific condition and medical history before initiating treatment.
- The patient's inflammatory polyarthritis may also be addressed with prednisone, as it is used as an adjunctive therapy for short-term administration in various rheumatic disorders, including rheumatoid arthritis.
- Another option could be methotrexate (PO), which is used to treat rheumatoid arthritis and has been shown to provide significant clinical improvement in patients with juvenile rheumatoid arthritis. 1 2
From the Research
For dermatomyositis with scleroderma features where inflammatory polyarthritis is the primary concern, I recommend starting methotrexate at 15-20 mg weekly along with folic acid supplementation (1 mg daily except on methotrexate day) as the next best option for treatment. This approach is supported by the most recent and highest quality study available, which highlights the effectiveness of methotrexate in addressing inflammatory arthritis and controlling the underlying autoimmune process 3. Methotrexate works by inhibiting dihydrofolate reductase and promoting adenosine release, which reduces inflammation and modulates immune function.
Some key points to consider when treating dermatomyositis with scleroderma features include:
- The importance of early recognition and treatment to decrease morbidity and prevent systemic complications 4
- The potential for overlap with other connective tissue disorders and malignancy, which necessitates careful evaluation and screening 3, 5
- The use of systemic corticosteroids as a first-line approach, but also the consideration of steroid-sparing agents like methotrexate, azathioprine, or mycophenolate mofetil to reduce glucocorticoid doses and adverse effects 6
- The potential benefits of biologics, such as rituximab and abatacept, in some patients with inflammatory myositis 6
In terms of specific treatment strategies, the following may be considered:
- If methotrexate alone is insufficient after 8-12 weeks, consider adding hydroxychloroquine 200-400 mg daily or mycophenolate mofetil 1000-1500 mg twice daily
- For acute flares, a short course of prednisone (15-20 mg daily with taper over 4-6 weeks) can provide rapid relief while waiting for the disease-modifying agents to take effect
- Regular monitoring is essential, including complete blood count, liver function tests, and renal function every 4-8 weeks initially, then every 3 months once stable 3