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Nephrology Grand Rounds. 6/17/08. Sarcoidosis in the kidney. Brad Weaver. Sarcoidosis. Multisystem & heterogeneous disease of unknown etiology characterized by noncaseating granulomas Three to four times more common among blacks Most often presents between ages 10-40 Common symptoms
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Nephrology Grand Rounds 6/17/08
Sarcoidosis in the kidney Brad Weaver
Sarcoidosis • Multisystem & heterogeneous disease of unknown etiology characterized by noncaseatinggranulomas • Three to four times more common among blacks • Most often presents between ages 10-40 • Common symptoms • Cough • Skin lesions • Eye lesions • Systemic: fatigue, malaise, fever, and weight loss • CXR often shows b/lhilaradenopathy and/or pulmonary reticular opacities
Organ involved # % Am J Respir Crit Care Med 2001;164:1885.
Lab findings in sarcoidosis • Eosinophilia 25% • Leukopenia 5-10% • Hypergammaglobulinemia 30-80% • Elevated serum ACE level 75%. False positives are rare. • BAL showing increased CD4/CD8 ratio • Hypercalcemia 2/2 extrarenal production of calcitriol by activated macrophages • Hypercalciuria is reported to be more common than hypercalcemia
Renal involvement • Sarcoidosis can cause wide variety of renal disease including ESRD • Histologic changes are more common than clinical disease which is rare • 20-50% on several small series of kidney biopsies and postmortem analysis • Nephrolithiasis : may be the presenting symptom of sarcoidosis • Polyuria • Nephrogenic DI: 2/2 hypercalcemia • Central DI: 2/2 granulomas in hypothalamus (also can cause primary polydipsia)
Granulomatous acute interstitial nephritis Granuloma Interstitial Inflammation
Retrospective study • 40 cases of renal granulomas found on kidney biopsy • Etiologies were determined: • Sarcoidosis 20 (50%) • Drug-induced 7 (17.5%) • TB 3 (7.5%) • Wegener’s 2 (5%) • Leprosy 1 (2.5%) • Mycobacterium avium 1 (2.5%) • Crohn’s disease 1 (2.5%) • No etiology determined 5 (12.5%) Medicine (Baltimore). 2007 May;86(3):170-80.
Renal involvement cont. • Glomerular disease: case reports of membranous, proliferative & crescentic GN, & FSGS • Urinary tract obstruction • Nephrolithiasis • RP fibrosis • RP lymph node enlargement • Treatment: prednisone 1mg/kg/day • Transplant has been reported to be successful
TINU syndrome • Tubulointerstitial nephritis & uveitis (TINU): rare disorder thought to be related to sarcoidosis • About 140 cases have been reported in mainly adolescents & young women • Renal disease can present as flank pain, sterile pyuria, & acute renal failure & often resolve on its own • Cases of progressive renal failure have been treated w/ 3-6 mo. of prednisone