Renal disease in sarcoidosis
- Gianfranco Rizzato, MD
Gianfranco Rizzato, MD
- Primario Emerito Internal Medicine
- Niguarda Hospital (Piazza Ospedale Maggiore)
- Gabriel Choukroun, MD, PhD
Gabriel Choukroun, MD, PhD
- Head of Nephrology, Dialysis, Transplantation Department
- CHU Amiens and Picardie University
Clinically important renal involvement occasionally occurs in sarcoidosis. Renal manifestations include abnormal calcium metabolism, nephrolithiasis and nephrocalcinosis, and acute interstitial nephritis with or without granuloma formation. The classic renal lesion is noncaseating granulomatous interstitial nephritis. However, this lesion rarely causes clinically significant renal disease. Hypercalciuria and hypercalcemia are most often responsible for clinically significant renal disease. Glomerular disease, obstructive uropathy, and end-stage renal disease (ESRD) may also occur, but are uncommon [1,2].
The renal manifestations of sarcoidosis will be reviewed here. General issues related to sarcoidosis and its pathogenesis are discussed separately. (See "Clinical manifestations and diagnosis of pulmonary sarcoidosis" and "Pathogenesis of sarcoidosis".)
The incidence and prevalence of renal involvement in sarcoidosis remain uncertain .
The reported prevalence ranges widely due to the enormous variation in study design and enrolled patient populations and due to the heterogeneity and often asymptomatic nature of renal disease.
Several small series have suggested that renal involvement (as defined by either histologic changes in the kidney or a decline in renal function in the absence of a biopsy) occurs in approximately 35 to 50 percent of patients [4-7], even if, in most cases, the disease may be silent and undetected for many years or forever. In one study, among 46 patients with acute or chronic sarcoidosis, 15 patients had renal abnormalities .
- Muther RS, McCarron DA, Bennett WM. Renal manifestations of sarcoidosis. Arch Intern Med 1981; 141:643.
- Casella FJ, Allon M. The kidney in sarcoidosis. J Am Soc Nephrol 1993; 3:1555.
- BERGER KW, RELMAN AS. Renal impairment due to sarcoid infiltration of the kidney; report of a case proved by renal biopsies before and after treatment with cortisone. N Engl J Med 1955; 252:44.
- LOFGREN S, SNELLMAN B, LINDGREN AG. Renal complications in sarcoidosis; functional and biopsy studies. Acta Med Scand 1957; 159:295.
- Lebacq E, Desmet V, Verhaegen H. Renal involvement in sarcoidosis. Postgrad Med J 1970; 46:526.
- MacSearraigh ET, Doyle CT, Twomey M, O'Sullivan DJ. Sarcoidosis with renal involvement. Postgrad Med J 1978; 54:528.
- Bergner R, Hoffmann M, Waldherr R, Uppenkamp M. Frequency of kidney disease in chronic sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2003; 20:126.
- Berliner AR, Haas M, Choi MJ. Sarcoidosis: the nephrologist's perspective. Am J Kidney Dis 2006; 48:856.
- Singer FR, Adams JS. Abnormal calcium homeostasis in sarcoidosis. N Engl J Med 1986; 315:755.
- LONGCOPE WT, FREIMAN DG. A study of sarcoidosis; based on a combined investigation of 160 cases including 30 autopsies from The Johns Hopkins Hospital and Massachusetts General Hospital. Medicine (Baltimore) 1952; 31:1.
- Rizzato G. Sarcoidosis in Italy. Sarcoidosis 1992; 9(Suppl 1):145.
- Singer DR, Evans DJ. Renal impairment in sarcoidosis: granulomatous nephritis as an isolated cause (two case reports and review of the literature). Clin Nephrol 1986; 26:250.
- Korzets Z, Schneider M, Taragan R, et al. Acute renal failure due to sarcoid granulomatous infiltration of the renal parenchyma. Am J Kidney Dis 1985; 6:250.
- O'Riordan E, Willert RP, Reeve R, et al. Isolated sarcoid granulomatous interstitial nephritis: review of five cases at one center. Clin Nephrol 2001; 55:297.
- Joss N, Morris S, Young B, Geddes C. Granulomatous interstitial nephritis. Clin J Am Soc Nephrol 2007; 2:222.
- Hoffbrand BI. The kidney in sarcoidosis. In: Sarcoidosis and Other Granulomatous Disorders, James DG (Ed), Marcel Dekker, New York 1994. p.335.
- Lower E. Renal sarcoidosis. In: Sarcoidosis, Baughman R (Ed), Taylor & Francis, New York 2006. p.651.
- Rizzato G. Calcium Metabolism. In: Sarcoidosis, Baughman R (Ed), Taylor & Francis, New York 2006. p.635.
- Mahévas M, Lescure FX, Boffa JJ, et al. Renal sarcoidosis: clinical, laboratory, and histologic presentation and outcome in 47 patients. Medicine (Baltimore) 2009; 88:98.
- Löffler C, Löffler U, Tuleweit A, et al. Renal sarcoidosis: epidemiological and follow-up data in a cohort of 27 patients. Sarcoidosis Vasc Diffuse Lung Dis 2015; 31:306.
- Dahl K, Canetta PA, D'Agati VD, Radhakrishnan J. A 56-year-old woman with sarcoidosis and acute renal failure. Kidney Int 2008; 74:817.
- Goldszer RC, Galvanek EG, Lazarus JM. Glomerulonephritis in a patient with sarcoidosis. Report of a case and review of the literature. Arch Pathol Lab Med 1981; 105:478.
- Jones B, Fowler J. Membranous nephropathy associated with sarcoidosis. Response to prednisolone. Nephron 1989; 52:101.
- van Uum SH, Cooreman MP, Assmann KJ, Wetzels JF. A 58-year-old man with sarcoidosis complicated by focal crescentic glomerulonephritis. Nephrol Dial Transplant 1997; 12:2703.
- Inui N, Murayama A, Sasaki S, et al. Correlation between 25-hydroxyvitamin D3 1 alpha-hydroxylase gene expression in alveolar macrophages and the activity of sarcoidosis. Am J Med 2001; 110:687.
- Rizzato G, Fraioli P, Montemurro L. Nephrolithiasis as a presenting feature of chronic sarcoidosis. Thorax 1995; 50:555.
- Rizzato G, Palmieri G, Agrati AM, Zanussi C. The organ-specific extrapulmonary presentation of sarcoidosis: a frequent occurrence but a challenge to an early diagnosis. A 3-year-long prospective observational study. Sarcoidosis Vasc Diffuse Lung Dis 2004; 21:119.
- Procino G, Mastrofrancesco L, Tamma G, et al. Calcium-sensing receptor and aquaporin 2 interplay in hypercalciuria-associated renal concentrating defect in humans. An in vivo and in vitro study. PLoS One 2012; 7:e33145.
- Stuart CA, Neelon FA, Lebovitz HE. Disordered control of thirst in hypothalamic-pituitary sarcoidosis. N Engl J Med 1980; 303:1078.
- Levi M, Peterson L, Berl T. Mechanism of concentrating defect in hypercalcemia. Role of polydipsia and prostaglandins. Kidney Int 1983; 23:489.
- Brause M, Magnusson K, Degenhardt S, et al. Renal involvement in sarcoidosis--a report of 6 cases. Clin Nephrol 2002; 57:142.
- Robson MG, Banerjee D, Hopster D, Cairns HS. Seven cases of granulomatous interstitial nephritis in the absence of extrarenal sarcoid. Nephrol Dial Transplant 2003; 18:280.
- Thumfart J, Müller D, Rudolph B, et al. Isolated sarcoid granulomatous interstitial nephritis responding to infliximab therapy. Am J Kidney Dis 2005; 45:411.
- Javaud N, Belenfant X, Stirnemann J, et al. Renal granulomatoses: a retrospective study of 40 cases and review of the literature. Medicine (Baltimore) 2007; 86:170.
- Bijol V, Mendez GP, Nosé V, Rennke HG. Granulomatous interstitial nephritis: a clinicopathologic study of 46 cases from a single institution. Int J Surg Pathol 2006; 14:57.
- Adams AL, Cook WJ. Granulomatous interstitial nephritis secondary to histoplasmosis. Am J Kidney Dis 2007; 50:681.
- Auinger M, Irsigler K, Breiteneder S, Ulrich W. Normocalcaemic hepatorenal sarcoidosis with crescentic glomerulonephritis. Nephrol Dial Transplant 1997; 12:1474.
- Fraioli P, Montemurro L, Castrignano L, Rizzato G. Retroperitoneal involvement in sarcoidosis. Sarcoidosis 1990; 7:101.
- Godin M, Fillastre JP, Ducastelle T, et al. Sarcoidosis. Retroperitoneal fibrosis, renal arterial involvement, and unilateral focal glomerulosclerosis. Arch Intern Med 1980; 140:1240.
- Mariano RT, Sussman SK. Sarcoidosis of the ureter. AJR Am J Roentgenol 1998; 171:1431.
- Gross KR, Malleson PN, Culham G, et al. Vasculopathy with renal artery stenosis in a child with sarcoidosis. J Pediatr 1986; 108:724.
- Aouizerate J, Matignon M, Kamar N, et al. Renal transplantation in patients with sarcoidosis: a French multicenter study. Clin J Am Soc Nephrol 2010; 5:2101.
- Mann DM, Fyfe B, Osband AJ, et al. Sarcoidosis within a renal allograft: a case report and review of the literature. Transplant Proc 2013; 45:838.
- NEPHROLITHIASIS AND NEPHROCALCINOSIS
- Clinical manifestations
- Differential diagnosis
- INTERSTITIAL NEPHRITIS
- Clinical manifestations
- Differential diagnosis
- URETERAL OBSTRUCTION AND OTHER RENAL MANIFESTATIONS
- RENAL TRANSPLANTATION
- SUMMARY AND RECOMMENDATIONS