Management for mixed connective tissue disease

There are no controlled trials investigating treatments for mixed connective tissue disease, because of small numbers of patients and the diversity of their clinical presentations. Management is individualised according to the presentation and severity of symptoms.

Immunomodulatory drugs may be useful for mixed connective tissue disease when clinical features of systemic lupus erythematosus (SLE) or rheumatoid arthritis are prominent. Useful drugs may include hydroxychloroquine and methotrexate.

If a person with mixed connective tissue disease develops hypertension, it may be associated with scleroderma renal crisis (accelerated hypertension and acute kidney failure in systemic sclerosis). Scleroderma renal crisis is a medical emergency. Immediate treatment with an angiotensin converting enzyme inhibitor (ACEI), preferably captopril, and referral to a specialist centre are recommended; see Scleroderma renal crisis and hypertension.