Adult onset still's disease (AOSD) : A case report - Orion Group

Case Reportmaculo-papular, distributed on upper chest,back and upper limb. The rash was mostnoticeable at the height of temperature. Theinvestigations revealed persistent neutrophilicleucocytosis, raised hepatic enzymes withnormal bilirubin. All the microbiological testswere again negative. Chest X-ray, USG ofwhole abdomen was normal. At that moment,left cervical lymphnodes were palpable. Theywere 2 in number, firm , tender, discrete. Alymph node biopsy was done to excludetuberculosis, it showed chronic non specificlymphadenitis. During that period patient wastreated with I/V antibiotics-ceftriaxone,gentamycine, but there was no response. Anechocardiogram was done to excludeinfective endocarditis, revealed only minimalpericardial effusion. About one week afteradmission, patient developed arthralgiafollowed by arthritis involving multiple bigjoints, e.g. shoulder, hip, wrist, elbow. Thispointed to a possible rheumatologicaldiagnosis. Because of the combinations ofhigh fever, arthritis, evanescent rash, sorethroat and lymphadenopathy-Adult onsetStill's disease was assumed to be a possiblediagnosis. Serum RA, ANA, both werenegative, CRP was positive and serum ferritinlevel was found to be significantly elevated.So with fulfillment of diagnostic criteria(yamaguchi criteria) and exclusion of othercauses-a final diagnosis of Adult Onset Still'sdisease was made.completely. Investigations showed persistentneutrophilic leucocytosis, high ESR, raisedhepatic enzymes & serum ferritin level higherthan before. So decision to start DMARD wastaken. Hydroxy chloroquine was preferredover Methotrexate considering safety profile.4 wks after starting Hydroxy chloroquine 200mg daily along with Aspirin, the patient hadcomplete clinical & biochemical remission.DiscussionThis multisystem inflammatory disease hassome common articular and non-articularmanifestations together with typicallaboratory findings. Most common clinicalfeatures of AOSD are - arthralgia (98 -100%),fever > 39oc (83-100%), Myalgia (84 - 90 %),rash (87 - 90%), sore throat (50 - 92%). 4Fever is an early feature, quotidian ordiquotidian in pattern with rise of temperaturein early morning/late afternoon. Patient withAOSD generally feel very ill while febrile &feel well when body temp is normal. Thisposes a dilemma for the physicians becausehospital rounds usually do not occur duringthe times when the patient is febrile. Alsohere in this case the young male lookedabsolutely fine during morning rounds but thetemperature chart showed high spikes atmidnight associated with severe myalgia. Sothe patient received a number of antibioticsfor presumed sepsis.Patient was treated with NSAID. Aspirin waschosen and given in high dose - 2400 mgdaily along with a proton pump inhibitor. 3 4wks after starting the treatment, most of thesystemic features were improved withsubsidence of fever, improvement of appetite& weakness. But the arthritis did not subsideThe ORION. Vol 24, May 2006The rash of Still's disease is a salmon-pinkcolour evanescent rash, particularly on upperpart of the body. It is often unappreciatedunless specifically sought and may be seenonly when the patient is febrile. The rashshows Koebner phenomenon anddermatographism. The patient also stated thathe had similar rash for a few days before itwww.orion-group.net/journalswww.orion-group.net/medicaljournal