Clinical Study on Adult Onset Still's Disease: Analysis in 15 Cases / 대한류마티스학회지

OBJECTIVE: To understand the clinical manifestations and disease course of adult onset Still' s disease (AOSD). METHODS: 15 patients of AOSD diagnosed at Severance hospital, Yonsei University College of Medicine were retrospectively analysed in the period of September 1988 to September 1995. RESULTS: There were 3 men and 12 women (male to female ratio of 1:4). Age of disease onset ranged from 17-55 years, and over 86% of the patients were younger than age 40 at disease onset. The prevalence of clinical features were as follows fever (100%), arthritis (93%), skin rash (93%), sore throat (60%), abnormal liver function (73%), lymphadenopathy (47%), splenomegaly (47%), hepatomegaly (20%), serositis (13%). Fever was the most common initial symptom. Common labaratory features were leukocytosis with neutrophilia (87%), anemia' Hgb <10 g/dL (67%), increased serum ferritin (83%), ESR (87%) and CRP (93%). Serum ferritin was markedly raised at disease onset and correlated with disease activity. In 2 patients, the disease was controlled with NSAID alone, but most of the patients required steroid to control the disease activity. In 6 patients, MTX was added for steroid sparing effect and for steroid resistant arthritis. Most of AOSD patients had intermittent and chronic disease course. Root joint arthritis and polyarthritis were factors associated with chronicity. CONCLUSION: The clinical features of AOSD in our study generally resemble previous reports. Serum ferritin was a useful marker of disease activity. Most patients of AOSD had intermittent and chronic disease course. Root joint athritis and polyarticular pattern at disease onset were factors associated with chronicity.

A Case of Hereditary Sensory Neuropathy Type II with Acroosteolysis / 대한류마티스학회지

The hereditary sensory neuropathy is a very rare disease characterized by prominent sensory loss without corresponding motor involvement, but may be associated with autonomic features. Currently, the disease is divided into five main types and most frequent are Type I and Type II. The type II hereditary sensory neuropathy is characterized by autosomal recessive inheritance, onset in utero or in infancy, loss of touch-pressure sense more than paintemperature sense, and almost total absence of myelinated nerve fibers. In this case, we describe a 23 years old female patient with acroosteolysis and heel ulcer who was diagnosed as hereditary sensory neuropathy type II.

A Case of Protracted Wegener`s Granulomatosis Which is Progress to Generalized Form / 대한내과학회지

Wegener's granulomatosis is a necrotizing and granulomatous vasculitis which involves upper and lower respiratory tract, kidney and skin. The patient who have the protracted Wegener's granulomatosis live a long peroid without major organ imvolvement, In mild cases, the progression is slow and may not need systemic cyclophosphamide treatment. Some cases, however, demonstrate renal involvement and may result in renal failure and death if adequate treatment is not provided. We experienced a case of protracted Wegener's granulomatosis that was a new concept and has not been reported !n Korea. The diagnosis was confirmed by clinical finding and histopathologic features of tiasue biopsy. This case represents a protracted Wegener's granulomatosis with paranasal sinusitis, rhinitis and skin purpura and progress to a gener alized form with kidney involvement after 6years. Treatment with oral cyclophosphamide, steroid and sulfamethoxazole-trimethoprime result improvement of skin, nasal symptom and labratory parameters.