A Rare and Severe Presentation of Henoch-Schönlein Purpura in an Adolescent With Crescentic Glomerulonephritis, Arrhythmia, Acute Gastrointestinal Bleed, and Neurological Complications

Abstract

Henoch-Schönlein purpura (HSP) is a childhood vasculitis disorder that involves the skin, joints, gastrointestinal (GI) tract, and kidneys. It is related to immunoglobulin A (IgA) antibody deposition in small blood vessels. HSP is a self-limiting disorder, but its morbidity is primarily associated with renal involvement. GI pathologies like intussusception, gastritis, duodenitis, ileitis, or ulcer have been reported to be associated with this disease. However, cardiac and neurological complications are rarely reported.We present the case of a 16-year-old, previously healthy male who was diagnosed with HSP after presenting with a non-blanching purpuric rash in the lower extremities. The patient also had joint and abdominal pain, and swelling in the extremities. There was renal dysfunction at presentation with blood urea nitrogen (BUN) of 67 mg/dL and serum creatinine of 1.9 mg/dL. The serum albumin was low at 2 g/dL, and the patient had nephrotic range proteinuria. Urine microscopy showed red blood cell casts. A renal biopsy was performed, which showed IgA deposition in glomeruli. He was started on intravenous (IV) pulse methylprednisolone and was later prescribed oral steroids. Four weeks after the treatment initiation, he presented with syncope and acute anemia (hemoglobin of 3.5 g/dL). The fecal occult blood was positive. Esophagogastroduodenoscopy (EGD) was not suggestive of gastritis, duodenitis, or ulcer. The pill-cam capsule endoscopy revealed GI bleeding from the terminal ileum near Meckel's diverticulum. He subsequently required blood transfusions, and the bleeding eventually improved with symptomatic management. Six weeks after treatment initiation, he presented with dizziness and palpitations. The EKG showed the presence of atrial fibrillation, and he had an episode of non-sustained ventricular tachycardia on telemetry. Arrhythmia was diagnosed secondary to HSP cardiac vasculitis, and we initiated treatment with metoprolol and amiodarone. Seven weeks after the initial treatment, he had neurological clinical findings of proximal muscle weakness, tremors, and upper and lower extremity clonus. A second renal biopsy was then performed due to the presence of persistently elevated serum creatinine, which showed 75% of glomeruli with cellular crescents. He was treated with IV cyclophosphamide. Subsequently, the renal function improved. There were no other GI, cardiac, or neurological complications after six months of follow-up.The presentation of HSP can be more severe in adolescents, and they need to be closely monitored for GI, cardiac, renal, and neurological complications after the disease occurrence. Bleeding from Meckel's diverticulum or an episode of non-sustained ventricular tachycardia with HSP has not been previously reported to our knowledge. Arrhythmia is an exceptionally unusual occurrence in HSP, and it is usually treated with anti-arrhythmic drugs and intensification of the immunosuppressive regimen.

IgA Vasculitis: a Review and Update on the Management of Renal and Extrarenal Disease, Highlighting What’s New for Biomarkers and Treatment

“…A 2019 retrospective study of 62 children compared IV methylprednisolone (IVMP) to cyclosporine A (CyA) in IgAV nephritis [46]. Outcomes including proteinuria, hematuria, and hypertension were not statistically different at 5 years or longer term final visit follow-up.…”