U # y.o. male with increased proteinuria, arthralgia and lower limb petechial rash. Hypertension ? Renal vasculitis ? Henoch-Schönlein purpura

30 yo male with Nephrotic Syndrome Acute onset lower extremity rash and edema Active urine sediment, hypertension, Cr 146 6g protein / 24 hrs Urgent referral within 1 week PHx: Strep throat, NEC as a neonate

In Clinic Spontaneous clinical improvement –Resolved edema, Cr 106, rash resolving –Still hypertensive and proteinuric 2g/24hrs –LE arthralgias persist, mild diarrhea –Serology all negative except: mildly depressed compliment levels, positive ASOT –No history of URTI

Tx Diuretics, ACEi, ARB Follow up bloodwork at biweekly intervals shows increasing creatinine Renal Biopsy performed

IF IgG- Negative. IgA- Mild mesangial staining. IgM- Negative. C3- Moderate vascular staining. Mild mesangial staining. C1q- Negative. Kappa- Negative. Lambda- Negative. Fibrinogen- Strong staining of glomerular crescents. Albumin- Negative.

IgA

C3

Fibrin

Diagnosis Renal Biopsy: Crescentic diffuse proliferative glomerulonephritis with IgA deposition by IF suggesting Henoch- Schönlein purpura.

Developed Pericarditis Acute pleuritic chest pain –VQ scan negative New rash Referred to ID: ? Rheumatic Fever Placed on penicillin prophylaxis