6/17/08

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 Common symptoms Cough Skin lesions Eye lesions Systemic: fatigue, malaise, fever, and weight loss CXR often shows b/l hilar adenopathy and/or pulmonary reticular opacities

Organ involved# % Lungs Skin Lymph node Eye Liver Erythema nodosum618.3 Spleen496.7 Neurologic344.6 Parotid/salivary293.9 Bone marrow293.9 Calcium273.7 ENT223.0 Cardiac172.3 Renal50.7 Bone/joint40.5 Muscle30.4 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) May;86(3):

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