6/17/08

Brad Weaver

SarcoidosisMultisystem & heterogeneous disease of

unknown etiology characterized by noncaseating granulomasThree to four times more common among blacksMost often presents between ages 10-40

Common symptomsCoughSkin lesionsEye lesionsSystemic: fatigue, malaise, fever, and weight loss

CXR often shows b/l hilar adenopathy and/or pulmonary reticular opacities

Organ involved # %Lungs 699 95.0

Skin   117 15.9

Lymph node 112 15.2

Eye 87 11.8

Liver 85 11.5

Erythema nodosum 61 8.3

Spleen 49 6.7

Neurologic 34 4.6

Parotid/salivary 29 3.9

Bone marrow 29 3.9

Calcium 27 3.7

ENT 22 3.0

Cardiac 17 2.3

Renal 5 0.7

Bone/joint 4 0.5

Muscle 3 0.4

Am J Respir Crit Care Med 2001;164:1885.

Lab findings in sarcoidosisEosinophilia 25%Leukopenia 5-10%Hypergammaglobulinemia 30-80%Elevated serum ACE level 75%. False

positives are rare.BAL showing increased CD4/CD8 ratioHypercalcemia 2/2 extrarenal production of

calcitriol by activated macrophagesHypercalciuria is reported to be more

common than hypercalcemia

Renal involvementSarcoidosis can cause wide variety of renal

disease including ESRDHistologic changes are more common than

clinical disease which is rare20-50% on several small series of kidney biopsies

and postmortem analysisNephrolithiasis : may be the presenting symptom

of sarcoidosisPolyuria

Nephrogenic DI: 2/2 hypercalcemiaCentral 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

biopsyEtiologies 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 obstructionNephrolithiasisRP fibrosisRP lymph node enlargement

Treatment: prednisone 1mg/kg/day Transplant has been reported to be

successful

TINU syndromeTubulointerstitial 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