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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2000  |  Volume : 11  |  Issue : 1  |  Page : 48-52
Granulomatous Interstitial Nephritis and Acute Renal Failure due to Renal-limited Sarcoidosis

1 Department of Medicine, Faculty of Medicine, Kuwait University, Kuwait
2 Department of Medicine, Ministry of Health, Kuwait
3 Department of Pathology, Ministry of Health, Kuwait

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Renal failure secondary to granulomatous sarcoidosis without clinical features or radiological evidence of disease is rare. In this case report, we describe a 14­year old girl who developed progressive renal failure over a two-month period which was associated with weight loss and epigastric pain. Physical examination did not show any abnormality. Laboratory investigations were normal except for normocytic normochromic anemia, high serum urea, high serum creatinine (452 tmol/L) and polyclonal gammopathy. Percutaneous kidney biopsy showed severe interstitial nephritis with non-caseating granulomata. She was treated with tapered prednisone after a starting dose of 1 mg/kg. The treatment with prednisolone resulted in a complete remission that lasted up to two years of follow up. This case highlights the variable expression of sarcoidosis, which should be considered in the management of such disease.

Keywords: Biopsy, Granuloma, Sarcoidosis, Renal failure.

How to cite this article:
El-Reshaid KA, Al-Khaldi EH, Madda JP. Granulomatous Interstitial Nephritis and Acute Renal Failure due to Renal-limited Sarcoidosis. Saudi J Kidney Dis Transpl 2000;11:48-52

How to cite this URL:
El-Reshaid KA, Al-Khaldi EH, Madda JP. Granulomatous Interstitial Nephritis and Acute Renal Failure due to Renal-limited Sarcoidosis. Saudi J Kidney Dis Transpl [serial online] 2000 [cited 2020 Aug 12];11:48-52. Available from: http://www.sjkdt.org/text.asp?2000/11/1/48/36693

   Introduction Top

Sarcoidosis is a multisystem disrorder characterized pathologically by the presence of non-caseating granuloma in the involved organs. [1] The lungs are mostly commonly affected. Isolated extrapulmonary involve­ment of the skin, eyes, reticuloendothelial system, musculoskeletal system, nervous system, heart, exocrine glands and kidneys are rarely seen. [2],[3] In the present case, a patient developed acute renal failure secondary to granulomatous interstitial nephritis without systemic sarcoidosis.

   Case Report Top

A 14-year old Kuwaiti girl was referred to Al-Amiri Hospital for evaluation of renal failure. She had diffuse mild epigastric pain for one year, which was burning in character and she lost 10 kg of weight. She denied history of fever, skin rash, joint problems, eye disease, or change in quality or quantity of urine. There was no prior history of significant illness or allergies. Both her parents had died of hypertensive cerebrovascular disease, yet all her six brothers and sisters were apparently healthy. The patient was seen by her family physician two months prior to her present hospitalization and found to have normal hemoglobin, normal total leukocyte and platelet counts as well as normal liver profile. There was, however, a mild elevation of the serum urea and creatinine (6.5 mmol/L and 160 pmol/L, respectively). The family physician prescribed oral antacids for one month and repeat of investigations showed further rise in urea and creatinine (16 mmol/L and 320 pmol/L, respectively). The physician discontinued the antacids and repeated the investigations one moth later, which showed further rise in urea and creatinine (20 mmol/L and 452 pmol/L, respectively).

At presentation to our hospital the patient was conscious, oriented to time place and person and in no distress. She was afebrile, normotensive and did not have evidence for lymphadenopathy, skin rash or edema. The systemic physical examination was unre­markable.

Laboratory investigations revealed normo­cytic normochromic indices with hemoglo­bin (HB) 100 g/L and erythrocyte sedimen­tation rate (ESR) 102 mm/h. Peripheral leucocytic count was normal with normal differential count. Serum haptoglobin was normal. Serum sodium, potassium, phos­phorus, corrected calcium ++ , uric acid and total carbon dioxide content were normal. Serum creatinine phosphokinase, bilirubin, alanine aminotransferase and alkaline phosphatase were normal. Serum albumin was 25 g/L and total proteins 92 g/L. Serum protein electrophoresis revealed polyclonal gammopathy. Urinalysis was negative for proteins and microscopic examination showed only few WBCs and RBCs (<5/high­power field each). Urinary protein excretion was 400 mg/24 h.

Chest x-ray and electrocardiogram were normal. Ultrasound of the abdomen and pelvis showed normal sized kidneys and there was no evidence of organ or lymphnode enlargement Serum antinuclear antibodies (ANA), anti-double stranded-DNA, rheu­matoid factor, antistreptolysin O titer, hepatitis B surface antigen, hepatitis C virus antibodies, antineutrophil cytoplasmic anti­bodies and cryoglobulines were negative. The patient underwent a percutaneous kidney biopsy, which showed widespread mono­nuclear, predominantly lymphocytic, infil­trate with non-caseating granulomatous lesions, which contained many giant cells [Figure - 1]. The glomeruli, tubules and vessels were normal. Ziehl-Neelsen Stain and culture for mycobacteria were negative. Tuberculin skin test was non-reactive,  Brucella More Details slide­agglutination test was negative, and gallium scan did not show uptake in the lungs or hilar regions. The serum angiotensin converting enzyme level was normal. Selective renal and colic arteriography did not show any abnormality. The patient was treated with Prednisone 40 mg daily for four weeks then was tapered off to stop over four more weeks. Two months after treatment, creatinine clearance was 102 ml/min and protein excretion 182 mg/L.

ESR decreased to 4 mm/h and HB became 12 g/L with serum albumin and total protein 41 and 80 g/L, respectively. After two years of follow up, the patient had no clinical, hematological, biochemical or radiological abnormality.

   Discussion Top

This reported case displays an uncommon cause of acute renal failure and unusual form of interstitial nephritis. Granulomatous interstitial nephritis occurs in many conditions. These include infections, such as tuberculosis, leprosy, histoplasmosis,  Brucellosis More Details; [4] adverse reactions to therapeutic agents, such as penicillin, methicillin, ampi­cillin, glafenin, spiramycin, non-steroidal anti-inflammatory drugs, diuretics and diphenylhydantoin; [5] Wegner's vasculitis, Crohn's disease and sarcoidosis. [1],[6] In our patient, the lack of a significant drug history in addition to the presence of high ESR, normocytic normochromic anemia and polyclonal gammopathy and the gratifying response to corticosteroid therapy were suggestive of autoimmune disease. Further­more, the presence of granulomatous inters­titial nephritis was very suggestive of sarcoidosis especially in absence of clinical and histological evidence of chronic infections or vasculitis.

The present case had a number of impor­tant features: (a) the absence of overt systemic sarcoidosis (b) normal levels of serum and urinary excretion of calcium as well as serum angiotensin converting enzyme (ACE) (c) a dramatic response to corticosteroid therapy.

Renal parenchymal granulomatous lesions were first documented in sarcoidosis in 1933.[7] Subsequent autopsy studies have revealed granulomatous renal involvement in 15-40% of case studies. [2],[3] However, in most cases it was clinically and bioche­mically silent. Renal failure, if present, was attributed acutely to hypercalcemia and chronically to nephrocalcinosis or glomerular disease. [2],[3] In 1987, Ford et al, described the first association between granulomatous interstitial nephritis in a patient with sarcoidosis and the development of acute renal failure. [8] In 1981, Muther et al reported their experience with sarcoidosis and documented 10 more cases of acute renal failure due to granulomatous interstitial nephritis, though all had evidence of sarcoidosis elsewhere. [9]

Our case is unique in that when renal failure occurred there was no other clinical feature or radiographic evidence of systemic sarcoidosis and diagnosis was suggested only by renal biopsy. Similar presentation of acute renal failure due to isolated granulomatous sarcoidosis was described in six cases worldwide. [1],[8],[10],[13],[14] In three of these six cases, the diagnosis was made on autopsy finding of epithelioid granulo­matous lesions in the cervical lymph nodes and liver. [8],[10]

Moreover, virtually all those cases had normal serum calcium levels, and nephro­calcinosis was not recorded. Despite this, renal failure was severe in most cases and a relapse was documented, in one patient, nine months after tapering down the corticosteroid therapy. [14]

Similarly, our patient did not have hyper­calcuria, hypercalcemia or elevated ACE level, which may have been the result of low granulomatous load limited only to the kidneys. [15]

We treated our patient with corticosteroids for a total of eight weeks with excellent short-term response and absent renal or systemic evidence of relapse after two years of follow-up. This complete remission of disease was described in nearly half of the reported cases of renal sarcoidosis.[1],[8],[9],[10],[11],[12],[13],[14] Early institution of treatment, milder form disease activity and lack of subsequent relapses may prevent progressive interstitial fibrosis and chronic renal failure.

This unique presentation of isolated renal sarcoidosis is a relevant argument in favor of genetic predisposition to disease. Recent studies suggested a major role for CD4+ cells in the pathogenesis of sarcoidosis. [16] These cells have class I or II Major Histo­-Compatibility (MHC) receptors for antigen recognition. Once MHC receptors are activated, CD4+ cells secrete cytokins, which are responsible for formation of granulomas, fibrosis and polyclonal gammopathy. The different expression of disease, severity, response to therapy and long-term prognosis seem to be patient-specific and needs to be considered in the management of sarcoidosis.

   References Top

1.Heptinstall R. Interstitial nephritis. In: Heptinstall R (ed). Pathology of the kidney. Little, Brown and Company 1992;1315-68  Back to cited text no. 1    
2.Longcope WT, Freiman DG. A study of sarcoidosis based on combined investi­gation of 160 cases including 30 autopsies from John Hopkins Hospital and Massachusetts General Hospital. Medicine 1952;31:1-32.  Back to cited text no. 2  [PUBMED]  
3.Ricker W, Clark M. Sarcoidosis: a clinico­pathologic review of 300 cases including 33 autopsies. Am J Clin Pathol 1959;19:725-49.  Back to cited text no. 3    
4.Zumala A, James DG. Granulomatous infections: etiology and classification. Clin Infect Dis 1996;23:146-58.  Back to cited text no. 4    
5.Vanhille P, Kleinknecht D, Morel-Maroger­L, et al. Drug-induced granulo-matous interstitial nephritis. Proc Eur Dial Transplant Assoc 1983;20:646-9.  Back to cited text no. 5    
6.Kiely PD, Rees DH, Axford JS. Crohn's and sarcoidosis: different manifes-tations of the same disease process? Br J Clin Pract 1994;48:274-5.  Back to cited text no. 6  [PUBMED]  
7.Garland HG, Thomson JG. Uveoparotid tuberculosis (febris uveoparotidea of Heerfordt). Q J Med 1933;2:157-77.  Back to cited text no. 7    
8.Ford MJ, Anderton JL, MacLean N. Granulomatous sarcoid nephropathy. Postgrad Med J 1978;54:416-7.  Back to cited text no. 8  [PUBMED]  
9.Muther RS, McCarron DA, Bennett WM. Renal manifestations of sarcoidosis. Arch Intern Med 1981;141:643-5.  Back to cited text no. 9  [PUBMED]  
10.Mignon F, Mery JP, Mougenot B, Ronco P, Roland J, Morel-Maroger L. Granulomatous interstitial nephritis. Adv Nephrol Necker Hosp 1984;13:219-45.  Back to cited text no. 10    
11.Hannedouche T, Grateau G, Noel LH, et al. Renal granulomatous sarcoidosis: report of six cases. Nephrol Dial Transplant 1990;5: 18-24.  Back to cited text no. 11    
12.Singer DR, Evans DJ. Renal impairment in sarcoidosis: granulomatous nephritis as an isolated cause. Clin Nephrol 1986;26: 250-­6.  Back to cited text no. 12  [PUBMED]  
13.Alcazar JM, Bello I, Arteaga J, Ruilope L, Barrientos A, Rodicio JL. Sarcoidosis and renal failure. Nephron 1981;29:281.  Back to cited text no. 13  [PUBMED]  
14.Williams PF, Thomson D, Anderson JL. Reversible renal failure due to isolated renal sarcoidosis. Nephron 1984;37:246-9.  Back to cited text no. 14    
15.Rohatgi PK, Ryan JW, Lindeman P. Value of serial measurement of serum angiotensin converting enzyme in the management of sarcoidosis. Am J Med 1981;70:44-50.  Back to cited text no. 15  [PUBMED]  
16.Thomas PD, Hunninghake GW. Current concepts of the pathogenesis of sarcoidosis. Am Rev Respir Dis 1987;135:747-60.  Back to cited text no. 16  [PUBMED]  

Correspondence Address:
Kamel A El-Reshaid
Department of Medicine, Faculty of Medicine, Kuwait University, P.O. Box 24923, Safat 13110
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