Case Report
 
Adult onset Still’s disease as a cause of acute liver failure
 

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Molly M Thabah, Kirti K Singh, Sethu M Madhavan, Rajiva Gupta
Department of Medicine,
All India Institute of Medical Sciences,
New Delhi-110029



Corresponding Author
: Rajiva Gupta
Email: guptarajiva@hotmail.com


Abstract

A 29-year old lady presented to the hospital with high-grade intermittent fever, arthritis and macular skin rash. Investigations revealed anaemia, polymorphonuclear leucocytosis and raised erythrocyte sedimentation rate. Other tests including those for antinuclear antibody and rheumatoid factor were normal. The serum ferritin level however was raised. On the basis of these parameters a diagnosis of Still’s disease was made. Treatment comprising oral steroids and anti-inflammatory agents was instituted. The patient recovered and was discharged only to present ten days later following 3 episodes of generalised tonic clonic seizures. Investigations revealed a persistently high serum ferritin with abnormal liver function test results. Jaundice developed and the patient went into grade IV hepatic encephalopathy following which she died. Still’s disease is an idiopathic disease, diagnosed purely on the basis of the typical clinical features of the illness which include persistent arthritis, high fever, anaemia and an erythematous rash. Treatment for Still’s disease mainly includes steroids and non-steroidal anti-inflammatory agents. Second-line treatment includes that used for controlling the arthritis and comprises gold, hydroxychloroquine, penicillamine, azathioprine, methotrexate, and cyclophosphamide.

Still’s disease was first discovered in children but is now known to occur, though much less frequently, in adults in whom it is known as adult-onset Still’s disease (AOSD). The diagnosis of AOSD is based solely on clinical features and is a diagnosis of exclusion as serological markers of the disease and specific diagnostic tests do not exist. We report the case of a 29-year old lady with Still’s disease who developed acute liver failure and subsequently died.

CASE REPORT
A 29-year old lady was diagnosed with AOSD when she
developed high-grade, intermittent fever for 3 weeks associated with arthritis involving the knee and ankle joints, with macular skin rash over the trunk. Relevant investigations revealed anaemia (haemoglobin 9.8 g/dL), a predominantly polymorphonuclear leucocytosis (white blood cell count 20,000/mm3) and an erythrocyte sedimentation rate of 90 mm/hour. Urine examination and other biochemical parameters including the liver and renal function tests were normal. Tests for antinuclear antibody and rheumatoid factor were negative. The serum ferritin was 6364.09 ng/ml. She was started on oral prednisolone 40 mg/day and given oral anti-inflammatory indomethacin on p.r.n. basis. She became afebrile after 3 days and was discharged from the hospital. After one week she developed generalised erythematous rash followed by high-grade intermittent fever (104 oF). Three days later she presented to the emergency department following 3 episodes of generalised tonic clonic seizures. Clinical examination revealed generalised, erythematous, blanchable rash, oral ulceration and palpable liver 3 cm below the right costal margin. All metabolic parameters and CT scan of the brain were normal. Phenytoin was started and steroids continued. The next day, the patient developed jaundice. Blood investigation revealed a total bilirubin level of 4.7 mg/dL, AST 4180 IU/L and ALT 2210 IU/L, serum alkaline phosphatase 1590 IU/L and a prolonged prothrombin time of  28 s (control value:11 s). Viral hepatitis serology for IgM HAV, IgM HEV, HBsAg and HCV were negative. She rapidly went into grade IV hepatic encephalopathy and expired following cardiac arrest the next day. Postmortem biopsy of the liver showed central necrosis with collapse of reticulin structure.

Discussion
Still’s disease is an uncommon rheumatic condition of unknown aetiology with recognised increased frequency in adults. The diagnosis of AOSD is based solely on clinical features and is a diagnosis of exclusion as serological markers and specific diagnostic tests do not exist, although a very high serum ferritin level does support the diagnosis. In our patient the diagnosis was made based on the following clinical features: spiking fever, rash, arthralgia, hepatomegaly, leukocytosis, absence of rheumatoid factor and antinuclear antibodies(1) and a very high serum ferritin level. A review of 62 cases of AOSD by Pouchet et al revealed that features of mild hepatitis were present in a majority AOSD cases and only rarely did patients present with life-threatening acute liver failure.(2) Variable levels of serum aminotransferase and alkaline phosphatase are typically seen in such patients during symptomatic flares of the disease, without clinical features of hepatitis.(3) These abnormalities may antedate therapy with non steroidal anti-inflammatory drugs and improve with anti-inflammatory treatment. However most cases of severe hepatitis have been observed in patients treated previously with salicylates or other nonsteroidal anti-inflammatory drugs.(2,3,4) Liver biopsy usually reveals moderate portal mononuclear cell infiltration with occasional focal hepatocyte necrosis in patients with liver dysfunction due to NSAIDS.(5)   We believe acute liver failure in our patient was due to AOSD, although we cannot exclude that NSAIDS could have contributed to the liver damage. Although asymptomatic hepatitis is very common in  AOSD, acute liver failure is a very rare complication. Liver biopsy usually reveals moderate portal mononuclear cell infiltration with occasional focal hepatocyte necrosis in patients with liver dysfunction due to NSAID.(5) The diagnosis of Reye’s syndrome was unlikely as our patient did not have a history of viral prodrome, which usually precedes Reye’s syndrome.(6) Moreover our patient had clinical jaundice which is not usually seen in Reye’s syndrome.(6,7) The mortality is high in AOSD with fulminant hepatic failure,(8,9) but liver transplantation has been attempted in some patients.(10,11)  In conclusion although asymptomatic hepatitis is very common in AOSD, fulminant liver failure is a very rare complication.

References

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2.     Pouchot J, Sampalis JS, Beaudet F, Carette S, Décary F, Salusinsky-Sternbach M, et al. Adult Still’s disease: Manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore).1991;70:118–36.

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4.     Adult onset Still’s disease: Experience in 23 patients and literature review with emphasis on organ failure. Semin Arthritis Rheum.1987;17:39–57.

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6.     Consensus Development Conference: Diagnosis and treatment of Reye’s syndrome. JAMA 1981;246:1441–44.

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8.     Dino O, Proyenzano G, Giannuolo G, Sciarrino E, Pouyet M, Pagliaro M. Fulminant hepatic failure in adult onset Still’s disease. J Rheumatol  1996;23:784–5.

9.     Ott SJ, Baron A, Berghaus T, Lamerz R, Beuers U. Liver failure in adult Still’s disease during corticosteroid treatment. Eur J Gastroenterol Hepatol 2003;15:87–90.

10.   Ogata A, Kitano M, Yamanaka J, et al. Interleukin 18 and hepato-cyte growth factor in fulminant hepatic failure of adult onset still’s disease. JRheumatol 2003;30:1093–6.

11.   McFarlane M, Harth M, Wall WJ. Liver transplant in adult still’s disease. J Rheumatol 1997;24:2038–41.