Sterile splenic abscesses in systemic Weber-Christian disease. Unique source of abdominal pain.

A patient with Weber-Christian disease and relapsing abdominal pain due to sterile splenic abscesses is presented. Histologically, acute and chronic inflammation with focal suppurative infiltrates of phagocytosed fat was detected in the absence of vascular or embolic disease, infection, or other apparent cause. Abdominal discomfort, an uncommon manifestation of Weber-Christian disease, usually is related to mesenteric panniculitis. Sterile splenic abscesses represent a previously undescribed complication of Weber-Christian disease, and another source of abdominal pain in this disorder. Although the precise pathophysiology of sterile splenic abscess formation in Weber-Christian disease is unknown, splenectomy is an effective means of alleviating pain.

Therapeutic strategies in autoimmune disease: cyclosporin A as a model agent.

Cyclosporin A is the derivative of the cyclic undecapeptide which is produced by 2 strains of fungi, Cylindrocapon lucidum Booth and Tolypocladium inflatum Gams. The drug has achieved widespread use in the treatment of organ transplant recipients as a vanguard against rejection, and currently is being evaluated as an immunosuppressive agent for the therapy of primary autoimmune phenomena as well. Its mechanism of action, and application to the treatment of rheumatologic disorders will be discussed.

Rheumatoid-like arthritis presenting as idiopathic pulmonary hemosiderosis: a report and review of the literature.

Idiopathic pulmonary hemosiderosis, an unusual form of the alveolar hemorrhage syndromes, has been reported in association with several connective tissue disorders. Rheumatoid arthritis (RA), however, rarely presents in this manner. We discuss a young adult with recent onset RA following idiopathic pulmonary hemosiderosis, and review the 3 cases previously reported.

Granulomatous musculoskeletal disease: sarcoidosis versus tuberculosis.

A patient with systemic sarcoidosis insidiously developed tuberculous arthritis. The concurrence of these diagnoses in any individual patient is rare. Musculoskeletal features of sarcoidosis and tuberculosis are reviewed, and the importance of obtaining appropriate cultures to differentiate these granulomatous disorders is illustrated.

Cardiac manifestations of Weber-Christian disease: report and review of the literature.

A patient with chronic systemic Weber-Christian disease who was treated with hydroxychloroquine developed cardiac dilatation with congestive heart failure. Endomyocardial biopsy demonstrated myocyte degeneration and interstitial fibrosis, but no typical features of chloroquine induced cardiomyopathy. Clinical symptoms of congestive heart failure also were recognized in 7 of the 11 reported autopsy cases of Weber-Christian disease having cardiac involvement. This involvement can affect the pericardium and the myocardium.

Azathioprine induced hepatic veno-occlusive disease in rheumatoid arthritis.

A patient with rheumatoid arthritis developed hepatic veno-occlusive disease following the use of azathioprine. Although azathioprine induced veno-occlusive disease is suspected to occur more frequently in patients with autoimmune dysfunction, it has not previously been reported as a complication of treatment in rheumatoid arthritis. The mechanism responsible for this condition remains unknown.