John S. Sergent

Epsilon aminocaproic acid therapy of hereditary angioneurotic edema. A double-blind study.

Abstract Five patients with hereditary angioneurotic edema were treated with a sequence of courses of epsilon aminocaproic acid (EACA) or placebo in a double-blind study. Attacks of edema were sign...

Vasculitis With Hepatitis B Antigenemia: Long-term Observations in Nine Patients

The development of generalized necrotizing vasculitis in association with hepatitis B antigenemia is the first example in man of a chronic rheumatic disease presumably caused by a viral infection. This report reviews the experience in nine biopsy-proven cases of hepatitis B-associated necrotizing vasculitis followed for up to six years. The natural history of the disease is emphasized and the manifestations of patients with vasculitis who carry hepatitis B antigen are compared with those of vasculitis patients who are antigen negative.

Vasculitis syndromes: clinical and experimental models.

Abstract Dr. Christian: Dr. Sergent has emphasized that diffuse vasculitis is not a disease but rather a manifestation of what may be a variety of etiologic stimuli. There are a few syndromes, such as Wegeners granulomatosis and giant cell arteritis, in which clinical and pathologic features are unique, but the majority of patients with vasculitis do not have distinguishing pathologic expressions. Immunologic mediation of disease is explicit in most animal models of vasculitis and is suspect in the human syndromes. HB virus infection, which may be associated with as many as one-third of the patients with diffuse vasculitis, may play a role analogous to viral infection in chronic LCM disease of mice. The recognition of HB s associated vasculitis adds to speculations that other viral agents may be implicated in arteritis syndromes of man.

Fatal Polymyositis in D-Penicillamine-Treated Rheumatoid Arthritis

Thirteen reports of patients who developed polymyositis or dermatomyositis during treatment with D-penicillamine are reviewed and a fourteenth case is described. Twelve of the fourteen patients recovered after D-penicillamine was withdrawn; two patients died from cardiac involvement. Proximal muscle weakness was present in 13 patients and dermatomyositis in 4 patients. Dysphagia was the presenting symptom in 6 patients. Although D-penicillamine is useful in the management of rheumatoid arthritis, this drug should be used with caution and patients monitored closely for evidence of polymyositis or dermatomyositis.

Necrotizing vasculitis after acute serous otitis media.

Abstract Seven adult patients developed widespread necrotizing vasculitis after acute serous otitis media. Clinically, these patients resembled other patients with vasculitis—neurological signs, re...

Rheumatoid arthritis and pure red cell aplasia.

Three patients with severe, deforming, and long-standing rheumatoid arthritis developed pure red cell aplasia that did not remit after withdrawal of medications, ran a chronic course, and in two patients remitted only after cytotoxic immunosuppressive treatment. An IgG inhibitor of autologous erythroid colony-forming and burst-forming unit growth in vitro was found in the serum of one patient. This specific erythropoietic inhibitor persisted in lower titer in the patients serum even after an azathioprine-induced remission of pure red cell aplasia, indicating the possible need for maintenance immunosuppressive therapy. Chronic pure red cell aplasia may be another extra-articular manifestation of rheumatoid arthritis and should be considered when severe anemia develops in the absence of blood loss or hemolysis.

Pericardial disease in rheumatoid arthritis.

Six patients with rheumatoid constrictive pericarditis, five seen in a two and one half year period, are described. All patients were male, all had rheumatoid factor, and all had active arthritis. Diagnosis was suspected from careful physical examination and confirmed in five patients by cardiac catheterization. Pericardiectomy was successful in all five patients on whom it was performed. Rheumatoid constrictive pericarditis should be suspected in any patient with rheumatoid arthritis and unexplained signs of right heart failure.

Leukocytoclastic Vasculitis and Multiple Myeloma

Excerpt To the editor: McMillen and colleagues (1) recently reported the case of a patient with leukocytoclastic vasculitis who subsequently was found to have an IgA-kappa myeloma. Their patient, a...

Treatment of Central Nervous System Lupus Erythematosus

Excerpt Central nervous system (CNS) complications in patients with systemic lupus erythematosus are common and can be fatal (1, 2). Unlike lupus glomerulonephritis, lupus cerebritis is not charact...

Adrenal Disorders in Rheumatology

Understanding adrenal disorders, whether endogenous or related to glucocorticoid use is important to the day-to-day care of patients with rheumatologic disease.