Acute liver transplantation in a 41-year-old male patient presenting symptoms of adult-onset Still's disease

Abstract Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology that is categorized as a non-hereditary disease. Neonatal hemophagocytic lymphohistiocytosis (HLH) is also a rare, but potentially fatal condition. Neonatal HLH is one of the causes of neonatal acute liver failure that often requires urgent liver transplantation. The relationship between AOSD during pregnancy and neonatal HLH currently remains unclear. We encountered a case of AOSD that developed during pregnancy, and an offspring was born with neonatal HLH resulting in severe liver failure. The mother with AOSD only presented with liver dysfunction during pregnancy; however, disease activity was exacerbated after delivery. The maternal clinical course was quite severe and refractory that she required biological therapy in addition to high-dose corticosteroids and immunosuppressants. Additionally, the severe condition of the neonate with HLH and acute liver failure required intensive care with the administration of steroids and intravenous immunoglobulin treatments, and ultimately liver transplantation. This is the first case that severe maternal AOSD associated with a neonatal HLH resulted in severe clinical courses. Physicians need to be aware of the risk of a mother with AOSD delivering an offspring with neonatal HLH with potentially acute liver failure.

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Systemic Lupus Erythematosus (SLE) in a male patient previously diagnosed as Adult Onset Still’s Disease: A Case Report

Bangladesh Journal of Medicine ◽

10.3329/bjmed.v30i2.41538 ◽

2019 ◽

Vol 30 (2) ◽

pp. 100-103

Author(s):

Md Anwar Sayed ◽

Suman Chowdhury

Keyword(s):

Systemic Lupus Erythematosus ◽

Case Report ◽

Male Patient ◽

Lupus Erythematosus ◽

Adult Onset Still’S Disease ◽

Adult Onset ◽

Still’S Disease ◽

Systemic Lupus ◽

Still's Disease ◽

Adult Onset Still's Disease

Adult-onset Still’s disease (AOSD) is a rare clinical entity with unknown etiology, characterized by evanescent rash, arthritis, fever, and other systemic presentation. In this case report, we describe a male patient of 50 years, previously diagnosed as a case of Adult onset still’s disease based on Yamaguchi criteria after the exclusion of other potential diagnoses. Later he was admitted into the Medicine department of Chittagong Medical College Hospital where he was found to have serological features of Systemic lupus erythematosus, another very much uncommon autoimmune disorder in male. On several occasion of his past admissions, SLE and RA were excluded meticulously. He initially responded to oral steroids only, recurrence of symptoms led us to work on the underlying etiology further. Coexistence of SLE in a patient with AOSD is not so commonly found. In our case, we notice this interesting phenomenon which was crucial for his management. Bangladesh J Medicine July 2019; 30(2) : 100-103

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