Autoimmune disorders such as those discussed in this chapter are generally treated with immunosuppressive therapy. Corticosteroids are a mainstay of therapy in all of these disorders, but doses may vary widely, depending on the severity of disease manifestations. Among other immunosuppressives, those with more serious potential side effects are reserved for more severe disease manifestations. Often, however, the dermatologic manifestations of SLE and DM can be treated by hydroxychloroquine. This is a long-acting anti-inflammatory agent, not generally considered immunosuppressive, whose precise mechanism of action remains unclear. Hydroxychloroquine is frequently used alone or in combination with immunosuppressive therapy when skin rash is present; however, it is not effective for the skin changes of scleroderma or the myositis of DM/PM.
Certain immunosuppressive agents may be efficacious for particular manifestations of a disease, such as cyclophosphamide for diffuse proliferative glomerulonephritis in SLE or methotrexate for synovitis in RA, but may lack efficacy for other manifestations of the same disease. It is, therefore, difficult to generalize about choices of immunosuppressive agents, especially when confronted with an unusual manifestation in the head or neck. No randomized, controlled trials have specifically addressed the otorhinolaryngeal manifestations of these rheumatic diseases, in part because of their rarity.
The cricoarytenoid muscle hypertrophy associated with DM has been reported to be amenable to surgical treatment (27).
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