U06-16889

#803963500

• 30 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

DiagnosisRenal 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