The coexistence of human immunodeficiency virus (HIV) infection and systemic lupus erythematosus (SLE) is unusual, but the occurrence of SLE after HIV infection in a child is even less common. We report a de novo presentation of SLE in a 10 year‐old African girl on HAART therapy for long standing HIV infection which was contracted following blood transfusion at the age of 1 year. The patient had presented with a 1 month history of fever, dry cough and cervical lymphadenopathy. Parents denied any sick contacts at home, recent travel or any other symptoms. Past medical history revealed that patient had an uneventful perinatal period. Family history was not contributory. Physical examination revealed a cachectic girl with a temperature of 100.7F and enlarged (0.5×0.5cm), tender nodes in the upper cervical region. She also had hyper pigmented healed rash on the right leg from bullae formation 2 years ago.
On admission, laboratory studies revealed low WBC count with positive HIV ELISA and undetectable viral load. Infectious disease work up for fever was negative. Hematological work up revealed sudden drop in hemoglobin with positive DAT. Her urinalysis showed an uptrend in urine proteins with a high urine Protein/Creatinine ratio. Further, rheumatologic work up revealed positive ANA titre and positive anti‐ histone, anti‐smith, anti dsDNA and extractable nuclear antibody (ENA SS‐A and SS‐B). Her clinical presentation and the positive serologies were consistent with SLE. She was started on Prednisone and Hydroxychloroquine. She responded immediatley and was discharged within days, clinically improved and without fever.
Clinical diagnostic challenge. An active SLE and HIV infection share several manifestations. Distinguishing between HIV and SLE based on autoimmune phenomena may be challenging. In addition, the question of the validity of the diagnostic criteria of lupus in the setting of HIV positivity would probably require further refinement in future. Our patient posed a diagnostic dilemma since she presented as FUO, without any classical skin lesions as seen in other adult patients and and underlying chronic infection.
Immunologic factors in coexistence of HIV and SLE. CD4+ T‐cells are one of the main targets of HIV. However, CD4+ T‐cell abnormalities are also important in the pathogenesis of SLE. It is possible that HIV infection might contribute to the development of SLE in patients such as ours. On the other hand, depletion of IFN‐producing plasmacytoid dendritic cells (pDCs) during HIV infection may contribute to the protective effect of HIV against SLE.
Management challenges in coexistence of HIV with SLE. In this HAART era, many questions are still unresolved in this field, including the real effect of immunosuppressive treatment for SLE on HIV infection and the effect of HIV treatment on SLE. The issues of management of these patients have to be adequately addressed, especially in pediatric age group.
The seemingly paradoxical coexistence of these two immune disorders offers intriguing insights into the complex cellular and humoral immune networks that govern autoimmune phenomena and self‐tolerance. Moreover, the clinical management of HIV with lupus in a pediatric patient represents a therapeutic challenge for the physician due to the delicate equilibrium that needs to be achieved between SLE remission and HIV control.