Systemic Lupus Erythematosus with Severe Nephritis That Mimicked Henoch-Schoenlein Purpura

Abstract

Introduction: Systemic lupus erythematosus (SLE) belongs to a family of related autoimmune rheumatic disorders that are capable of affecting multiple organs, and they are all associated with a variety of autoantibodies. Henoch Schoenlein purpura (HSP) is a sort of systemic vasculitis that is not associated with auto-antibodies and can affect different organs including the kidneys.Case report: A 12 year-old girl presented with abdominal pain, low grade fever, swollen and tender feet and left hand, skin rash on the lower extremities, and high blood pressure. Initial laboratory tests revealed severe proteinuria, microscopic hematuria and low C3 level. Renal biopsy showed diffuse proliferative glomerulonephritis with IgA, fibrinogen and C3 deposits. The case was accordingly diagnosed as HSP with severe IgA nephropathy. Treatment was started with mycophenolate mofetil (MMF) and pulse methylprednisolone followed by prednisolone. The patient improved and treatment was discontinued after 5.5 months. One month after withdrawal of her medications, the patient presented again with serositis and recurrent proteinuria. Both antinuclear antibodies (ANA) and anti dsDNA were positive. At this point she was diagnosed to have SLE disease and immunosuppressive treatment was restarted. Following this, symptoms disappeared, proteinuria regressed and anti-dsDNA titer dropped. Conclusion:This case presented with features of HSP and was later-on diagnosed to have SLE. This kind of clinical overlapping has not been reported in the literature to the best of our knowledge.

Henoch-Schönlein purpura in a pediatric patient with lupus

“…In 2011, Al-Attrach et al . first reported a 12-year-old girl who presented with features of HSP and was later diagnosed to have SLE [6]. …”

Henoch Schonlein Purpura Mimicking Lupus Nephritis-A Rare Case

“…Al attarch et al reported a 12 year old girl who presented with HSP with severe IgA nephropathy and responded to treatment but eventually developed lupus. The histopathology did not reveal a full house in the child on immunofluorescence [15]. Abdawani et al reported a 13 year old boy with HSP with nephritic range proteinuria and a full house picture on immunofluorescence which improved with aggressive treatment like our patient [16].…”

IgA vasculitis (Henoch – Schönlein Purpura) as the first manifestation of juvenile Systemic Lupus Erythematosus: Case-control study and systematic review

“…There are only four case-reports published in the medical literature in which the association between IgA vasculitis (HSP) and jSLE is present [13–16]. The case described by Al-Attrach [14] is particularly interesting due to the similarity to our cases. The patient was a 12-year-old girl diagnosed with IgA vasculitis (HSP) based on skin, joint, abdominal, and renal affection, after 5 months on treatment with corticosteroids and mycophenolate mofetil, she attained withdrawal of medications.…”