6/17/08. brad weaver sarcoidosis multisystem & heterogeneous disease of unknown etiology...
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6/17/08
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Brad Weaver
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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
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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.
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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
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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)
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Granulomatous acute interstitial nephritis
Granuloma
Interstitial Inflammation
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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.
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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
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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