Think renal, think wise

A fourteen-years-old male, suffered generalized abdominal pain, vomiting & non-bloody diarrhea, a week later, GTCs occurred He was diagnosis as encephalitis, but was anuric, edematous & hypertensive. Investigations were done and showed anemic with NO thrombocytopenia but with impaired KFT. Renal biopsy revealed TMA/TTP. He received 12 TPE with partial improvement then he developed bleeding/Rectum not responding to medical management, lower GIT endoscopy revealed: Cobble stone appearance in transverse colon & active IBD (Croh’s) so Pentasa & Immuran started GTCs developed again, MRI brain showed PRESS. KFT deteriorated again with oliguria, so 10 TPE sessions with infliximab & FDS were given with partial improvement. Two weeks later, more deterioration occurred. New Investigations: • Non-microangiopathic Non-hemolytic anemia • Normal platelet & TLC • Coomb’s test: negative • eGFR: 25 ml/min/1.732 • Normal Complements • ANA, Anti-DNA-Ab, ANCA: negative • Protein-C, S & Anti-thrombin-III: Normal • ADMATS13 level: Normal • Urinalysis: hemaruria & Heavy proteinuria • MRI: encephalitis vs ischemic areas 2ry to Vasculitis Revision of biopsies: § Colonic: Leukocytoclastic vasculitis § Renal: TMA A diagnosis of MULTI-SYSTEMIC LEUKOCYTOCLASTIC-VASCULITIS was established and started: 1. Pulse steroid 5 doses then oral FDS then withdrawal till reaching the lowest dose 2. MMF 1.2 gm/m2/day Marked improvement of his renal, GIT and CNS condition had achieved: Clinically: No GIT bleeding, convulsion Laboratory: GFR: 75ml/min/1.732, Urinalysis: trace albumin Radiologically: Normal MRI, A & V.