(BMJ)—A 5-yo boy presented w/ fever, abdominal pain, and rectal bleeding following a 3-wk hx of rash and arthralgia. Exam: pallor; widespread purpuric rash w/ necrotic lesions; swollen extremities. Labs: decreasing Hgb; UA: erythrocytes, proteinuria. CXR: bilateral focal abnormalities. At intubation, blood noted in trachea. What’s the dx?
Granulomatosis w/ polyangiitis
Kawasaki dz
Henoch-Schönlein purpura
Hypersensitivity vasculitis
Hemorrhagic pancreatitis
You are correct. The child had Henoch-Schönlein purpura (HSP) complicated by pulmonary renal syndrome, w/ glomerulonephritis and pulmonary hemorrhage. Pulmonary and glomerular involvement is a rare but life-threatening complication requiring early recognition and aggressive tx. In a deteriorating child w/ HSP, it’s important to be aware of serious multisystem complications that can be associated w/ the dz. This pt required high-frequency oscillation ventilation due to refractory hypoxemia. He was transfused but didn’t require antihemorrhagic agents. Additional tx included IV cyclophosphamide and plasmapheresis, followed by PO prednisolone and enalapril. He improved slowly and was extubated. Heavy proteinuria and microscopic hematuria were still present, but his renal function gradually improved. He made a good recovery, and w/in 2wk, he was ambulatory, off O2, and asymptomatic.

Archives of Disease in Childhood 2019;104:1214-1215