Systemic sclerosis manifesting as intractable gastro-oesophageal reflux and diarrhoea: a case report from Kenya.

Systemic sclerosis is a rare condition that has not been well reported in Africa, and several multisystemic manifestations, including gastrointestinal ones, have not been well documented locally. We present an unusual case of persistent gastro-oesophageal reflux and diarrhoea in a 74-year-old Kenyan female, who progressively developed abdominal distention, dysphagia and Raynaud´s phenomenon. Stool tests were unremarkable, whereas antinuclear antibody, ribonucleoproteins antibody (anti-nRNP/Sm) and anti-Sjögren's-syndrome-related antigen A autoantibody (anti-SSA) tests were positive. Endoscopic and imaging investigations revealed features of gastrointestinal dysmotility including reflux oesophagitis, gastroparesis and chronic intestinal pseudo-obstruction. A diagnosis of systemic sclerosis was made, and she responded well to medical treatment. We present this case to contribute to the limited literature of a disease associated with high morbidity and mortality, as well as encourage fellow clinicians to have a high level of suspicion in their differentials of persistent gastrointestinal dysmotility.

Autoimmune hepatitis: a manifestation of immune reconstitution inflammatory syndrome in HIV infected patients?

OBJECTIVE: To describe a case series of patients presenting with autoimmune hepatitis after initiation of antiretroviral therapy. MATERIALS AND METHODS: The demographics, clinical and laboratory features, and therapeutic response of HIV-infected patients on antiretroviral therapy presenting to our Division between November 2011 and November 2014 with elevated liver enzymes, were analysed. RESULTS: Nine patients with elevated liver enzymes, immunoglobulin G and autoimmune markers in keeping with autoimmune hepatitis were identified. All were anti-hepatitis C virus negative. One patient was hepatitis B surface antigen positive but his hepatitis B viral load was undetectable. All patients denied using any traditional herbal remedies. Liver histology was consistent with autoimmune hepatitis showing interface hepatitis and infiltrates of lymphocytes and plasma cells. Diagnosis was made according to the Autoimmune Hepatitis Group Scoring Systems. All patients were started on 15-20 mg of oral prednisone with clinical and biochemical improvement after 1-6 weeks. CONCLUSIONS: Immune reconstitution related autoimmune hepatitis should be considered in the differential diagnosis of hepatitis in the HIV-infected patient on antiretroviral therapy. Liver biopsy should be performed and the diagnosis confirmed using scoring systems developed by the Autoimmune Hepatitis Group. Timely treatment with prednisone and other agents for autoimmune hepatitis is indicated, and can be lifesaving in acute liver failure.

Idiopathic pure red cell aplasia: case report with review of literature.

Pure red cell aplasia (PRCA) is characterized by near absence of red blood cell precursors in the bone marrow with associated anemia and reticulocytopenia. Megakaryocytes and white blood cell precursors remain intact. Acute self-limited PRCA is the most common form. Acquired chronic PRCA is often idiopathic but can be associated with underlying disorders such as thymoma or autoimmune diseases. Congenital PRCA is associated with physical abnormalities. Pure red blood cell aplasia should always be included in the differential diagnosis of chronic unexplained anemia. We report a case of idiopathic pure red blood cell aplasia in an elderly male who has had a chronic anemia with recurrent blood transfusions for about 30 years.