Adult Onset StillÊs Disease

Sir, Adult Onset StillÊs Disease (AOSD) was reported first by Baywaters in 1976 with clinical features identical to those of Juvenile Rheumatoid Arthritis (JRA). Studies from France, Japan and India3 comparing and analyzing clinical features, course and prognosis of JRA and AOSD are basically identical. The pathogenesis and treatment are also the same. It seems therefore reasonable to consider them as a single distinct entity irrespective of age of onset and use a single nosological term viz. StillÊs disease. AOSD is uncommon and even less frequently reported. About 30 cases haven been reported from Northern India. There are no reports from the south and over 300 cases have been reported in world literature. Here is a fairly typical case of StillÊs disease. A 19 year old boy presented with 12 days history of sore throat, high fever with rigors and spikes, bodyache, pain in joints and erythematous rash all over the trunk which was blanching on pressure. Rash was prominent with spikes of fever and almost disappeared with fall in temperature. On examination he was found to be febrile with non-itching macular erythematous rash on the trunk. He had arthralgia of left hip, left knee and right elbow. Inguinal glands were minimally enlarged and non-tender. Serial laboratory tests revealed rise in WBC count from 8700 to 13000/cmm, neutrophils from 69% to 84%, ESR from 15 to 108 normal platelet count and hemoglobin level. Malaria, dengue, infectious mononucleosis, typhoid fever, HIV infection and syphilis were ruled out on the basis of laboratory tests. CXR and abdominal ultrasound were normal. CT abdomen showed mild splenomegaly. Blood cultures were sterile. Rheumatoid factor, ANA, C-ANCA and P-ANCA tests were negative. LE cells were not found in blood. Aspiration and trephine biopsy examination of bone marrow showed mild myeloid hyperplasia and increased iron stores. ASO titer was negative CRP was 96 mg/L (1:16 positive) LDH was 477 IU/L (N=109-193). SGOT was raised. Bilateral lymphnode biopsies showed reactive hyperplasia with no evidence of malignancy. Serum ferritin level was 16887.82 mg/L (N=18.7 to 323 mg/L). Hyperferritinaemia is a powerful marker of AOSD. This costly and time consuming work up to exclude infections, seropositive autoimmune disorders and certain malignancies may be obviated if a proposed new set of (AOSD) criteria suggested by Fautrel B et al1 (Table 1) and the earlier preliminary criteria proposed by Yamanguchi M et al2 (Table 2) are considered first.