Adult Still’s disease

Adult Still s disease presents with seronegative polyarthritis, febrile attacks, lymphadenopathy, skin symptoms, hepatosplenomegaly, jaundice and involvement of the pleura and pericardium. The liver is enlarged in about 30% of patients transaminases may be slightly elevated. Periportal lymphocytic infiltrates and Kupffer cell proliferation can be demonstrated histologically. (68, 69)... 

B., Dufour, R, Blickle, J.F., Grogard, XM., Schlienger, XL. Retrospective monocentric study of 17 patients with adult Still s disease, with special focus on hver abnormalities. Hepato-Gastroenterol. 2003 50 ... 

Maclachlan D, Tyndall A. Acute hepatitis in adult Still s disease apparently resulting from oral iron substitution—a case report. Clin Rheumatol 2000 19(3) 222-5. 

Bliddal H, Helin P. Leucopenia in adult Still s disease durii treatment with a tiiiopiine and sulphasalazine. CUnRheumatol( 9ZT) 6,244-50. 

Chen DY, Lan JL, Lin FJ, Hsieh TY. Proinflammatory cytokine profiles in sera and pathological tissues of patients with active untreated adult onset Still s disease. J Rheumatol 2004 31(11) 2189-2198. 

Kl. Kawaguchi, Y., Terajima, H., Harigai, M., Hara, M., and Kamatani, N., Interleukin-18 as a novel diagnostic marker and indicator of disease severity in adult-onset Still s disease. Arthritis Rheum. 44, 1716-1717 (2001). 

Gambichler, T., Paech, V., Rotterdam, S., Stucker, M., Boms, S., Alt-meyer, P. Hepatitis B-associated adult-onset Still s disease presentling with neutrophilic urticaria. Eur. J. Med. Res. 2003 8 527-530... 

Atsukawa, K., Tsnkada, N., Yonei, Y., Inagaki, Y., Miyamoto, K., Suzuki, O., Kiryu, Y., Sato, S., Kano, S. Two cases of adult-onset Still s disease accompanied with centrilobular hver damage. Acta Hepatol. Japon. 1995 36 53-57... 

Stern A, Buckley L. Worsening of macrophage activation syndrome in a patient with adult onset Still s disease after initiation of etanercept therapy. J Clin Rheumatol 2001 7 252-6. 

A 22-year-old woman with adult-onset Still s disease and massive hyperferritinemia became progressively more anemic, with a fall in hemoglobin to 8.2 g/dl, and was given oral ferrous fumarate 300 g bd (30). She developed acute florid hepatitis with an intraparenchy-matous histiocytic infiltration, which settled on withdrawal of the iron. 

Adult onset Still s disease occurred after 3 months of oral isotretinoin (104). No other causes were found and the symptoms disappeared with appropriate treatment within some months. 

Leibovitch I, Amital H, Levy Y, Langevitz P, Shoenfeld Y. Isotretinoin-induced adult onset Still s disease. Clin Exp Rheumatol 2000 18(5) 616-18. 

Neck fibrosis, an uncommon adverse reaction to ciclosporin, has been reported in a 30-year-old man with adult-onset Still s disease who took ciclosporin 3mg/kg/day, glucocorticoids, and NSAIDs for 1 year [13 ]. There was complete regression 1 year after withdrawal of ciclosporin. Apart from gingival tissue, fibrosis associated with ciclosporin can be found in various other... 

Kobak S, Savas R, Oksel F, Doganavsargil E. Cyclosporine A-induced neck fibrosis in a patient with adult-onset Still s disease. Clin Rheumatol 2010 29 (2) 205-8. 

This was the third published case of fatal valproate liver toxicity in an adult with Friedreich s disease, suggesting that this condition is a predisposing factor. Although the risk of valproate hepatotoxicity in adults is much lower than in children, adults still represented over 15% of the cases known to the authors. Overall, a total of 26 other adults (age range 17-62 years) with fatal valproate-induced Uver failure have been reported of these, 3 were taking monotherapy and 12 had no underlying disease or a clearly non-metabolic and non-hepatic disease. The duration of treatment before the first symptoms varied between 6 days and 6 years. 

Adults - Take 9 mg once daily in the morning for up to 8 weeks. Swallow capsules whole do not chew or break. For recurring episodes of active Crohn disease, a repeat 8-week course of budesonide can be given. Following an 8-week course of treatment for active disease and once the patient s symptoms are controlled (Crohn Disease Activity Index less than 150), budesonide 6 mg is recommended once daily for maintenance of clinical remission for up to 3 months. If symptom control is still maintained at 3 months, an attempt to taper to complete cessation is recommended. Continued treatment with budesonide 6 mg for more than 3 months has not been shown to provide substantial clinical benefit. 

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