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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT  
Year : 2020  |  Volume : 31  |  Issue : 2  |  Page : 549-552
A rare case of sarcoidosis causing granulomatous interstitial nephritis presenting as non oliguric acute renal failure in the Indian subcontinent


1 Department of Nephrology, Narayana Medical College, Nellore, Andhra Pradesh, India
2 Advanced Research Center (Genetics), Narayana Medical College, Nellore, Andhra Pradesh, India

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Date of Submission01-Oct-2018
Date of Decision30-Dec-2018
Date of Acceptance01-Jan-2019
Date of Web Publication09-May-2020
 

   Abstract 


Sarcoidosis is a systemic disease characterized by non caseating granulomatous inflammation. Sarcoidosis can affect any organ, but it most commonly involves the lungs and lymph nodes. The exact incidence of renal involvement in sarcoidosis remains unclear, but it is found to be rare. Granulomatous interstitial nephritis is the most common histological pattern, but its presentation with renal insufficiency is rare. Here, we present a case of sarcoidosis causing granulomatous interstitial nephritis presenting as isolated non oliguric acute renal failure.

How to cite this article:
Sapre C, Kolla PK, Rao K V, Sadineni R, Reddy B, Sharma S, Viswanath V, Shaik MV. A rare case of sarcoidosis causing granulomatous interstitial nephritis presenting as non oliguric acute renal failure in the Indian subcontinent. Saudi J Kidney Dis Transpl 2020;31:549-52

How to cite this URL:
Sapre C, Kolla PK, Rao K V, Sadineni R, Reddy B, Sharma S, Viswanath V, Shaik MV. A rare case of sarcoidosis causing granulomatous interstitial nephritis presenting as non oliguric acute renal failure in the Indian subcontinent. Saudi J Kidney Dis Transpl [serial online] 2020 [cited 2020 Jul 7];31:549-52. Available from: http://www.sjkdt.org/text.asp?2020/31/2/549/284036



   Introduction Top


Sarcoidosis is a systemic disease characterized by non caseating granulomatous inflammation.[1] The underlying etiology of the disease is still unknown.[2] Sarcoidosis can affect any organ, but it most commonly involves the lungs and lymph nodes.[3] The incidence of sarcoidosis is 16.5/100,000 and 19/100,000 in men and women, respectively.[4] The exact incidence of renal involvement in sarcoidosis remains unclear, but it is found to be rare.[5] Some studies show that granulomatous interstitial nephritis may lead to chronic kidney disease in the long term.[6] Here, we present a case of sarcoidosis causing granulomatous interstitial nephritis presenting as isolated non oliguric acute renal failure (ARF).


   Case Report Top


Informed consent was obtained from the patient before presenting the report.

A 40-year-old gentleman presented with a history of decreased appetite, fatigue, and poly- uria for three months. He had no complaints of altered sensorium, chest pain, dyspnea, weight loss, tingling, numbness, joint pains, or bone pains. General examination showed pitting pedal edema till the ankle joint. Systemic examination revealed hepatomegaly which was firm and nontender with a liver span of 16 cm. The investigations showed a normal hemogram with a serum creatinine (SCr) of 3.95 mg/dL at presentation. Urine routine microscopy showed 1+ albuminuria with no casts. Urine protein- creatinine ratio was 1.11, and 24-h urinary protein was 840 mg. Ultrasound abdomen showed hepatomegaly, splenomegaly with heterogenous echotexture and diffuse multiple hypoechoic lesions, and bilateral bulky kidneys with hypoechoic cortex [Figure 1]. The Mantoux test was negative. Corrected calcium was 10.81 mg/dL, and 24-h urinary calcium was 430 mg/day. Chest X-ray was normal [Figure 2]. A renal biopsy was done immediately, due to the uncertainty of the cause of acute renal failure, which showed moderately dense infiltrates of lymphocytes, few plasma cells, numerous eosinophils, and many epithe- loid cells, non caseating granulomas along with many giant cells. Stain for acid-fast bacilli and fungus was negative. The picture was compatible with granulomatous interstitial nephritis. The serum angiotensin-converting enzyme (ACE) levels (153 pL) (normal range: 10-50 pL) and Vitamin D levels were found to be elevated (125 ng/mL). The patient was submitted to an eye examination which revealed anterior uveitis. High-resolution computed tomography (CT) chest showed multiple enlarged right and left para-tracheal, pre- vascular lymph nodes with nodules in bilateral lung fields. A contrast enhanced CT scan of the abdomen showed hypodense lesions involving liver, spleen, and moderate volume abdominal lymphadenopathy [Figure 3]. The lymphadenopathy was compressing the inferior vena cava, resulting in pedal edema. Hence, the patient was diagnosed with sarcoidosis and was started on oral prednisolone (60 mg/day) and was continued for one month and then tapered. Maintenance dose of 10 mg/day was continued after tapering. The patient showed improvement in the form of improvement in lymphadenopathy and the normalization of SCr after three months of treatment. The patient is being continued on maintenance steroid and has had no episode of deranged renal function tests till date.
Figure 1: Histopathology.

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Figure 2: Chest X-ray.

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Figure 3: Computed tomography image.

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   Discussion Top


The major organs involved in sarcoidosis include lungs, kidney, heart, and the central nervous system.[7] Sarcoidosis is associated with a broad spectrum of renal manifestations which include hypercalcemia/hypercalciuria, granulomatous interstitial nephritis, glomerular diseases, tubular dysfunction, and obstructive vascular uropathy. Granulomatous interstitial nephritis is the most common histological pattern in renal sarcoidosis, but its presentation with renal insufficiency is rare.[8] Naidu et al[9] studied 14 cases of granulomatous interstitial nephritis in India, but did not find a single case of sarcoidosis causing it. Majority of the cases were found to be secondary to tuberculosis. Prior to their article also, there has been no article published of granulomatous interstital nephritis with sarcoidosis from the Indian subcontinent. Subsequently, Agrawal et al[10] also found tuberculosis to be the most common cause of granulomatous interstitial nephritis in India in a case series of 17 cases, thus making tuberculosis one of the important differential diagnoses to the causes of granulo- matous interstital nephritis, especially in the Indian subcontinent.

The incidence of hypercalcemia and hyper- calciuria in sarcoidosis is 10% to 17% and 40% to 62%, respectively.[11] Disordered calcium homeostasis is the culprit for the development of acute renal dysfunction. It induces dysfunction through several mechanisms. It can decrease glomerular filtration rate by vaso- constriction of the afferent arteriole. In addition, it can also inhibit sodium-potassium ATP-ase, thus causing urinary sodium wasting leading to polyuria and dehydration. This polyuria also occurs because of reduced sensitivity to the anti diuretic hormone. Finally, acute tubular necrosis may occur from intra- cellular calcium increase, and the calcium precipitates may cause tubular obstruction. Sarcoidosis can present with a progressive tubulointerstitial inflammation with associated calcium deposits, leading to nephrocalcinosis.[8] Granulomatous interstitial nephritis is the most common renal lesion in patients with sarcoi- dosis and renal impairment seen on biopsy.[11] Although glomerular involvement in sarcoi- dosis is rare, a variety of different lesions have been described in the literature including membranous nephropathy, focal segmental sclerosis, mesangioproliferative glomeruloneph- ritis, IgA nephropathy, and crescentic glome- rulonephritis.[8]

The diagnosis of sarcoidosis is a diagnosis of exclusion.[10] Serum ACE levels are found to be elevated in most cases, but this test is nonspecific. ACE is produced by epithelioid cells, multinucleated giant cells, and macrophages and thus can be elevated with other granulo- matous conditions as well, thus limiting its use.[8] Urinary manifestations are not specific either with the most common findings of aseptic pyuria and microscopic hematuria. Hypercalciuria is a common finding, and its presence helps in the consolidation of diag-nosis. Hence, diagnosis of sarcoidosis requires demonstration of a typical histopathological picture in at least one affected organ system.[12] Though isolated renal sarcoidosis has been reported; it requires heightened vigilance because granulomatous lesions can be present in other conditions such as tuberculosis, allergic reaction to medications, and autoimmune diseases.[5]

Mainstay of treatment of sarcoidosis consists of steroids and management of hypercalcemia. Other supportive measures such as dialysis are required if the patient presents with renal failure. Our case was managed conservatively and did not require dialysis. As previously stated, disordered calcium homeostasis is the main cause of manifestations, and it has to be treated promptly. The first line of management is intravenous saline administration and gluco- corticoids as they diminish the activity of granulomas, thereby reducing the activity of 1- alpha hydroxylase.[8] other agents that can be used for hypercalcemia management include chloroquine, hydroxychloroquine, and keto- conazole.[8] There is no standardized protocol for the use of glucocorticoids for the management of sarcoidosis. Most authors recommend a starting dose of 0.5-1.0 mg/kg of prednisone depending on the organ involvement.[1],[5],[11],[12] Initial dose should be maintained for at least 4 weeks. After that, the dose can be tapered slowly at the rate of 5 mg/week. Granulomatous interstitial nephritis responds well to treatment and has been found to remain stable on maintenance steroids.[1] The other agents that can be used for glucocorticoid-resistant disease include azathioprine, mycophenolate mofetil, metho- trexate, and tumor necrosis factor alpha inhibitors.[8] Progression to end-stage renal disease with sarcoidosis is rare.


   Conclusion Top


Through this case report, we wish to convey that in a country where tuberculosis is rampant, sarcoidosis remains an important cause of granulomatous interstitial nephritis which can present with isolated ARF and if treated promptly has a good outcome.

Conflict of interest: None declared.



 
   References Top

1.
Mahevas M, Lescure FX, Boffa JJ, et al. Renal sarcoidosis: Clinical, laboratory, and histologic presentation and outcome in 47 patients. Medicine (Baltimore) 2009;88:98-106  Back to cited text no. 1
    
2.
Kellinghaus C, Schilling M, Ludemann P. Neurosarcoidosis: Clinical experience and diagnostic pitfalls. Eur Neurol 2004;51:84-8  Back to cited text no. 2
    
3.
Dahl K, Canetta PA, D’Agati VD, Radhakrishnan J. A 56-year-old woman with sarcoidosis and acute renal failure. Kidney Int 2008;74:817-21  Back to cited text no. 3
    
4.
Nunes H, Bouvry D, Soler P, Valeyre D. Sarcoidosis. Orphanet J Rare Dis 2007;2:46  Back to cited text no. 4
    
5.
Berliner AR, Haas M, Choi MJ. Sarcoidosis: The nephrologist’s perspective. Am J Kidney Dis 2006;48:856-70  Back to cited text no. 5
    
6.
Shen SY, Hall-Craggs M, Posner JN, Shabazz B. Recurrent sarcoid granulomatous nephritis and reactive tuberculin skin test in a renal transplant recipient. Am J Med 1986;80:699- 702  Back to cited text no. 6
    
7.
Judson MA. Extrapulmonary sarcoidosis. Semin Respir Crit Care Med 2007;28:83-101  Back to cited text no. 7
    
8.
Hilderson I, van Laecke S, Wauters A, Donck J. Treatment of renal sarcoidosis: Is there a guideline? Overview of the different treatment options. Nephrol Dial Transplant 2014;29: 1841-7  Back to cited text no. 8
    
9.
Naidu GD, Ram R, Swarnalatha G, Uppin M, Prayaga AK, Dakshinamurty KV. Granulo- matous interstitial nephritis: Our experience of 14 patients. Indian J Nephrol 2013;23:415-8  Back to cited text no. 9
    
10.
Agrawal V, Kaul A, Prasad N, Sharma K, Agarwal V. Etiological diagnosis of granulo- matous tubulointerstitial nephritis in the tropics. Clin Kidney J 2015;8:524-30  Back to cited text no. 10
    
11.
Le Besnerais M, Francois A, Leroy F, et al. Renal sarcoidosis: about a series of five patients. The Journal of Internal Medicine 2011;32:3-8  Back to cited text no. 11
    
12.
Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Crit Care Med 1999;160:736-55.  Back to cited text no. 12
    

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Correspondence Address:
Praveen Kumar Kolla
Department of Nephrology, Narayana Medical College, Chintareddypalem, Nellore - 524 003, Andhra Pradesh
India
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DOI: 10.4103/1319-2442.284036

PMID: 32394934

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    Abstract
   Introduction
   Case Report
   Discussion
   Conclusion
    References
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