HIV-associated juvenile systemic sclerosis: a case report.

ABSTRACT

INTRODUCTION: Autoimmune connective tissue diseases occur in HIV-infected persons though with a lower frequency than in the general population. However, since the advent of highly active antiretroviral therapy (HAART), the spectrum of autoimmune diseases reported in HIV-infected patients has increased. OBJECTIVE: To describe the occurrence and management of systemic sclerosis in a HIV-infected child. METHODS: A case report of HIV-associated systemic sclerosis and a review of the literature on previously published cases. RESULTS: A nine-year-old girl presented with HIV-associated systemic sclerosis complicated with interstitial lung disease and oesophageal dysmotility. She was treated with intravenous cyclophosphamide with good response on her skin scores and modest improvement in lung function parameters. She manifested no deterioration in her clinical status. However, she developed mild lymphopaenia following the treatment with cyclophosphamide. CONCLUSION: We have described the rare occurrence of HIV infection and systemic sclerosis in a nine-year-old girl. She received cyclophosphamide for management of the systemic manifestations of SSc and did not manifest any major adverse events except mild lymphopaenia. Thus, though cyclophosphamide appeared safe in the management of HIV-associated systemic sclerosis, close monitoring of lymphocyte and CD4 counts should be done in such patients.