Raymond J. Enzenauer

Sarcoidosis in autoimmune disease.

Sarcoidosis is a multisystem granulomatous disease of unknown etiology. Sarcoidosis coexisting with connective tissue diseases, once considered rare, complicates various such disorders, including rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, Sjögrens syndrome, and the spondyloarthropathies. Symptoms common to sarcoidosis and autoimmune disease include keratoconjunctivitis sicca, weight loss, fever, lymphadenopathy, pulmonary complaints, and cutaneous lesions. Consequently, the diagnosis of sarcoidosis in association with connective tissue disease is often difficult and may require biopsy of the lung, liver, skin, lymph node, muscle, or bone marrow for pathological confirmation. Abnormalities of immune function as well as autoantibody production, including rheumatoid factor and antinuclear antibodies, are seen in sarcoidosis and in connective tissue diseases, suggesting a common immunopathogenic mechanism. The severity and course of sarcoidosis associated with autoimmune disease is variable. The incidence of sarcoidosis in association with rheumatic disease may be underestimated if new symptoms of sarcoidosis are attributed to the primary rheumatic disease and a secondary diagnosis is not pursued.

Chronic septic arthritis caused by Borrelia burgdorferi.

Chronic arthritis occurs in 10% of Lyme disease patients. A patient had chronic septic Lyme arthritis of the knee for seven years despite multiple antibiotic trials and multiple arthroscopic and open synovectomies. Spirochetes were documented in synovium and synovial fluid (SF). Polymerase chain reaction (PCR) analysis of the SF was consistent with Borrelia infection. Persistent infection should be excluded with silver stains and cultures in any patient with chronic monoarticular arthritis and a history of Lyme disease.

Retinal vasculopathy associated with systemic light chain deposition disease.

A 35-year-old black male with the abrupt onset of blurred vision and decreased visual acuity was found to have severe retinal vasculopathy associated with systemic light chain deposition disease. While ocular manifestations are frequently reported in other plasma cell dyscrasias, ophthalmologic symptoms have not been previously reported in systemic light chain deposition disease alone. A possible mechanism of light chain deposition and damage to the retinal vasculature is proposed.

Pure red cell aplasia in systemic lupus erythematosus.

Pure red cell aplasia (PRCA) is a rare hematologic disorder characterized by a severe progressive anemia and a marked decrease or absence of red cell precursors on bone marrow examination. PRCA can occur in the course of many other diseases of which thymoma is the most common. Rarely, PRCA has occurred in patients with connective tissue disorders, the most common being systemic lupus erythematosus (SLE), with 13 cases reported to date in the English literature. In this subset of patients, PRCA can be difficult to manage, and a high rate of mortality has been reported. Multiple therapeutic modalities have been reported, with no clear consensus on the optimal treatment. To date, splenectomy has not been reported in this subset of patients.We describe a patient with SLE who developed PRCA. Prednisone was the initial treatment, but unacceptably high doses were required to control the disease. Intravenous gamma globulin was given with prompt but temporary improvement. The patient subsequently underwent splenectomy with resolution of the PRCA. The patient continues to have a normal hemoglobin 3 years after splenectomy despite no interim therapy. This case represents the 14th case of PRCA occurring in patients with SLE and is the first case of PRCA in SLE treated with splenectomy. Splenectomy should be considered as a therapeutic modality in the management of corticosteroid-refractory PRCA occurring in patients with SLE.

Subcutaneous sarcoidosis associated with sarcoid tenosynovitis.

Subcutaneous sarcoidosis and sarcoid tenosynovitis are unusual manifestations of systemic sarcoidosis. We report two Japanese women with disseminated sarcoidosis presenting with subcutaneous and tenosynovial involvement demonstrated by computed tomography and magnetic resonance imaging. Sarcoidosis must be considered in the differential diagnosis of unexplained subcutaneous nodulosis or tenosynovitis in patients with or without a previous diagnosis of sarcoidosis.

Inflammatory arthritis associated with thromboangiitis obliterans.

Thromboangiitis obliterans (TAO) is an uncommon to rare vasculitis of small and medium arteries and veins typically affecting young male smokers. Inflammatory arthritis has only been rarely reported in association with TAO. We describe the case of a young male smoker presenting with inflammatory arthritis as the initial manifestation of his TAO. His arthritis resolved with a course of corticosteroid therapy. The vascular disease improved with hyperbaric oxygen therapy, although he continued to smoke. Similar cases have been described, suggesting that rheumatologists should keep Buergers disease in mind in patients with arthritis and vascular disease.