Graves disease is an autoimmune thyroid disease classically characterised by a clinical triad consisting of hyperthyroidism, diffuse goitre, and thyroid eye disease. Thyroid eye disease is an immunologically mediated condition in which humoral immunity is thought to play a central role. Thyroid eye disease is traditionally treated with high-dose glucocorticosteroids and surgical orbital decompression. However, responses are inadequate and alternative treatment options are needed. Rituximab, an anti-CD20 monoclonal antibody, shows promise as a novel therapeutic option for thyroid eye disease. There are 43 cases of thyroid eye disease treated with rituximab in the medical literature, and larger studies are warranted to determine the long-term effectiveness of rituximab. Rituximab may represent an attractive new treatment option for thyroid eye disease, especially in the case of disease that is refractory to current treatment strategies.
Clinical Images: Subscapular bursitis and calcinosis in a patient with dermatomyositisThe patient, a 59-year-old man with dermatomyositis (DM), presented with fever, chills, and acute right subscapular pain in the setting of chronic bilateral subscapular bursitis. DM had been diagnosed in 2003, based on muscle weakness, typical skin findings including shawl sign and Gottron's papules, and confirmatory muscle biopsy results. Jo-1 antibodies were absent, and the patient had not developed interstitial lung disease. The subscapular fluid collections developed during a DM flare (skin and muscle) several years after the initial diagnosis. These fluid collections persisted despite subsequent remission of the DM with hydroxychloroquine monotherapy. Prior therapies had included prednisone, azathioprine, and methotrexate. At the time of this presentation the patient had recently undergone a dental procedure and reported shaking chills and fever of 2 days' duration. Physical examination revealed significant bulging of the bilateral subscapular regions and erythema and tenderness on the right side (A). There was no evidence of rash or muscle weakness at this time. Laboratory assessments revealed leukocytosis 16.2 ϫ 10 3 /l (normal 4-10) with Ͼ80% neutrophils, a C-reactive protein level of 31 mg/dl (normal Ͻ0.8), and an erythrocyte sedimentation rate of 67 mm/hour (normal 0-20). Computed tomography confirmed bilateral subscapular fluid accumulation (arrows) with calcification of the bursa (B). Calcinosis and bursitis both occur in patients with DM, though more commonly in the juvenile population. Bursitis in this location is rare. If asymptomatic, bursitis is often not treated because it commonly recurs after aspiration. There was concern regarding possible septic bursitis in this patient; thus, the right subscapular fluid collection was aspirated, and cultures grew Streptococcus milleri. He underwent surgical drainage followed by antibiotic therapy and is currently doing well, although he reports asymptomatic reaccumulation of subscapular fluid. His DM remains in remission with hydroxychloroquine treatment.
Giant cell arteritis (GCA), a vasculitis of the medium and large arteries, is traditionally managed with glucocorticoids. However, the side effects of chronic glucocorticoid use and the occurrence of refractory cases warrant the consideration of steroid-sparing agents, including biologic agents. Interleukin-6 (IL-6) inhibition shows the most promise as biologic therapy for refractory cases of GCA, but data to support the use of other existing biologic agents are currently lacking. A better understanding of the pathogenesis of GCA as well as clinical trials investigating both existing and emerging biologic agents is needed to expand therapeutic options for the treatment of GCA.
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