Whippleʼs disease, familial Mediterranean fever, and adult-onset Stillʼs disease

Abstract

Whipple's disease is a multisystem disorder thought to be caused by infection by rod-shaped bacilli. Early diagnosis remains difficult, because initial clinical features are nonspecific. Ultrasonography and computed tomographic scanning were used to demonstrate distinctive lymphadenopathy in Whipple's disease. Magnetic resonance imaging showed central nervous system lesions that were reversible with antibiotic therapy. Familial Mediterranean fever, or recurrent polyserositis, is an autosomal recessive disorder common among patients of Mediterranean heritage. Erysipelas-like skin lesions are commonly described. Other skin lesions, including Schönlein-Henoch purpura, nonspecific purpura, diffuse erythema, and angioneurotic edema are now reported. Renal complications, thought previously to be due primarily to amyloid, are also caused by immunoglobulin deposition resulting in mesangial proliferative glomerulonephritis. Adult-onset Still's disease is a systemic illness characterized by quotidian fever and a fleeting, salmon-colored rash. The long-term evolutions of juvenile-onset and adult-onset Still's disease were compared and found to be similar, except for the occurrence of amyloidosis in the latter group of patients. Prognosis of patients with articular features was worse than that of patients with extra-articular features. A multicenter survey of Japanese patients found few significant differences between Japanese and non-Japanese cases. Less well-recognized features of adult-onset Still's disease, including neurologic complications, uveitis, and peritonitis, are reported.