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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2020  |  Volume : 31  |  Issue : 4  |  Page : 856-859
Granulomatous Interstitial Nephritis Due to Renal-limited Sarcoidosis

1 Medical Student, King Saud University, Riyadh, Saudi Arabia
2 Department of Medicine, King Abdulaziz Medical City; King Saud Bin Abdulaziz University of Health Sciences; King Abdullah International Medical Research Center, Riyadh, Saudi Arabia
3 Department of Medicine, King Abdulaziz Medical City, Riyadh, Saudi Arabia
4 Department of Pathology, King Abdulaziz Medical City, Riyadh, Saudi Arabia

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Date of Web Publication15-Aug-2020


Sarcoidosis can present as acute kidney injury (AKI) due to granulomatous interstitial nephritis (GIN). AKI caused by sarcoid GIN without extra-renal manifestations is extremely rare. We report a case of a 42-year-old man with a history of unexplained weight loss admitted with progressively worsening kidney function. Physical examination did not show any abnormality. Laboratory investigations were normal except for high calcium level with no evidence of organ involvement of sarcoidosis. A renal biopsy showed GIN with non-caseating granulomata. Prednisolone was initiated and renal function improved. This is a case of an extremely rare AKI caused by sarcoid GIN without extra-renal manifestations which responded to prednisolone.

How to cite this article:
Alangari FS, Binsalih SA, Heijani B, Aloudah N. Granulomatous Interstitial Nephritis Due to Renal-limited Sarcoidosis. Saudi J Kidney Dis Transpl 2020;31:856-9

How to cite this URL:
Alangari FS, Binsalih SA, Heijani B, Aloudah N. Granulomatous Interstitial Nephritis Due to Renal-limited Sarcoidosis. Saudi J Kidney Dis Transpl [serial online] 2020 [cited 2022 Nov 29];31:856-9. Available from: https://www.sjkdt.org/text.asp?2020/31/4/856/292321

   Introduction Top

Renal involvement as an initial manifestation of sarcoidosis is a rare entity. The prevalence of renal failure in case patients with sarcoi- dosis ranges from 0.7% to 4.3%.[1] Sarcoidosis is a granulomatous multi-system disease that is characterized by non-caseating granulomatous inflammation with tissue destruction in the involved organs.[2] Sarcoidosis most commonly affects lungs, however, other organs including kidneys are rarely affected.[3] Granulomatous interstitial nephritis (GIN) secondary to sarcoidosis is usually clinically silent but may occasionally present as acute kidney injury.[4]

We present a case of granulomatous interstitial nephritis due to renal-limited sarcoidosis.

   Case Report Top

A 42-years old male presented with a history of unintentional weight loss of 5 kg associated with decreased appetite over the last few weeks prior to his presentation. He also gave a history of intermittent subjective fever and sweating, especially at night, which responded well to acetaminophen. The patient also described a history of dry, non-productive cough that was not associated with shortness of breath or hemoptysis. The review of systems revealed a history of general fatigue and muscle aches. There was no joint pain, tenderness or swelling. The patient denied any previous medical history, medication intake, contact with sick patients or animals, and raw milk ingestion. Moreover, there was no history of limbs weakness, altered sensation, visual disturbance, vomiting, chest pain or palpitation, body swelling, burning micturition, hematuria or skin rash. His family history was significant for diabetes mellitus, hypertension, and ischemic heart disease. His sister has multiple sclerosis.

The patient is married, with three children. He denied any history of smoking. For few months prior to presentation, the patient had been under severe stress due to financial and social issues.

At presentation, the patient was conscious, oriented, afebrile, hemodynamically stable, not in respiratory distress, his blood pressure was 125/80 mm Hg, heart rate of 75 beats per minute, temperature of 36.8°C. The abdomen was soft, lax, with no palpable organomegaly or ascites. There was no skin rash, oral or genitalia ulcerations, lymphadenopathy or signs of arthritis.

White blood cell count was 8.8 K/uL, hemoglobin 13.3 g/dL, mean corpuscular volume (MCV): 81 fL, platelet count was 384 K/uL, erythrocyte sedimentation rate (was 49 mm/hour, creatinine: 115 μmol/L, calcium: 2.75 mmol/L, phosphorus: 1.25 mmol/L, alanine aminotransferase, 42 U/L, albumin: 45 g/L and the urine was negative for protein, red blood cells, casts, nitrite and leukocytes. Thyroid stimulating hormone, and serum intact parathyroid hormone levels were normal. Brucella titer was negative.

After three weeks, the laboratory investigation were repeated and creatinine level was 152 μmol/L, Ca: 3 mmol/L (and urine analysis showed only trace protein. Protein electrophoresis, C3, and C4 were normal. Serum antinuclear antibodies anti-double stranded- DNA, and urine culture were negative. The patient was still complaining of body ache, low grade fever with unremarkable clinical examination.

Chest X-ray was normal and renal ultrasound showed normal sized kidneys and there was no evidence of organ or lymph node enlargement. CT of the chest, abdomen and pelvis was unremarkable. Ultrasound of the neck, thyroid, parathyroid, and genitalia US were all normal.

The patient underwent a percutaneous kidney biopsy. Microscopic examination revealed well preserved, normal looking glomeruli. The interstitium showed heavy interstitial lympho- cytic infiltrate and non-caseating granulomata with giant cells. Tubular injury was noted [Figure 1]. Ziehl-Neelsen stain for acid fast bacilli was negative [Figure 2]. The histolo- gical diagnosis was granulomatous interstitial nephritis (GIN), consistent with sarcoidosis. In the absence of any other organ involvement, clinical diagnosis of renal-limited sarcoidosis was made.
Figure 1: Core biopsy of kidney (20×, H&E) showing heavy interstitial lymphocytic infiltrate (open arrow) and non-caseating granuloma (inset, open arrow) and giant cell (solid blue arrow), with relatively well preserved normal looking glomeruli. Tubular injury is noted.

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Figure 2: PAS stained slides showing granulomatous inflammation with multiple multinucleated giant cells (red arrows). Right upper insert showing AFB stain which is negative.

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The differential diagnoses included tuberculosis, brucellosis, lymphoma and hyper- thyroidism. There was no radiological evidence suggestive of pulmonary tuberculosis. Computerized axial tomograms of chest abdomen and pelvis revealed no chest findings suggestive of pulmonary tuberculosis. There were no cervical, axillary, hilar or abdominal lymphadenopathy. Brucella serology and thyroid stimulating hormone was normal.

The patient was treated with prednisone 1 mg/kg/day (total dose) and azathioprine 50 mg oral once daily. Azathioprine had to be stopped after four weeks due to bone marrow suppression. The patients symptoms quickly resolved with return of appetite, disappearance of fever and fatigue.

After 12 months of treatment the creatinine level was 106 μmol/L, the calcium became normal, the patient improved clinically, and he continued on prednisolone 5 mg/daily.

   Discussion Top

GIN occurs in many conditions including infections,[5] Wegner’s vasculitis, Crohn’s disease and sarcoidosis,[2],[6] Sarcoidosis is a rare multi system granulomatous disorder with a prevalence of 10–20 cases per 100,000 population in the United States of America.[7] Pulmonary involvement is the most frequent finding in sarcoidosis, however, extra pulmonary disorders including skin, eyes and lymph nodes are also common.[8]

Our case initially presented with vague symptoms and unexplained weight loss and progressively worsening kidney function. None of the clinical examinations or imaging tests showed any evidence of pulmonary, cutaneous or ocular involvement with sarcoi- dosis.

GIN is a rare histological diagnosis found in 0.5–0.9% of native renal biopsies,[9] however, it is rarely diagnosed as this pathology is usually clinically silent and does not affect the renal function.[10] Kikuchi et al in their similar case report published in 2015[11] reported that till that time approximately 20 cases of GIN without extra renal sarcoid manifestations have been reported in the literature. The pathological mechanism contributing to AKI from GIN in sarcoidosis is thought to be due to non-caseating granulomatous inflammation, that is composed of a central follicle of macrophages, epithelioid cells, and multi- nucleated giant cells.[12],[13]

In agreement with similar cases reported in the literature,[14] when our patient developed renal failure, there was no radiographic evidence of systemic sarcoidosis and diagnosis was suggested only by renal biopsy and the response to steroid therapy. On the other hand, and in contrast to El-Reshaid case, our case had hypercalcemia.[14]

For patients with decreased estimated glome- rular filtration rate (eGFR), corticosteroid therapy is considered the treatment of choice for sarcoid-related GIN, often resulting in significant improvement in kidney function.[15] In our case the patient was treated with corticosteroids with excellent short-term response and absent renal or systemic evidence of relapse after two years of follow-up. Such complete remission of the disease was described in nearly half of the reported cases of renal sarcoidosis.[15]

Conflict of interest: None declared.

   References Top

Mahévas M, Lescure FX, Boffa JJ, et al. Renal sarcoidosis: clinical, laboratory, and histologic presentation and outcome in 47 patients. Medicine (Baltimore) 2009;88(2):98-106.  Back to cited text no. 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. 2
Rao DA, Dellaripa PF. Extrapulmonary manifestations of sarcoidosis. Rheum Dis Clin North Am 2013;39(2):277-97.  Back to cited text no. 3
Casella FJ, Allon M. The kidney in sarcoidosis. J Am Soc Nephrol 1993;3(9):1555-62.  Back to cited text no. 4
Zumla A, James DG. Granulomatous infections: etiology and classification. Clin Infect Dis 1996;23(1):146—58.  Back to cited text no. 5
Kiely PD, Rees DH, Axford JS. Crohn’s and sarcoidosis: different manifestations of the same disease process? Br J Clin Pract 1994; 48(5):274-5.  Back to cited text no. 6
Thomas KW, Hunninghake GW. Sarcoidosis. JAMA 2003;289(24):3300-3.  Back to cited text no. 7
Baughman RP, Teirstein AS, Judson MA, et al. Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med 2001;164(10 Pt 1):1885-9.  Back to cited text no. 8
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(4):297-302.  Back to cited text no. 9
Casella FJ, Allon M. The kidney in sarcoidosis. J Am Soc Nephrol 1993;3(9):1555-62.  Back to cited text no. 10
Kikuchi H, Mori T, Rai T, Uchida S. Acute kidney injury caused by sarcoid granulomatous interstitial nephritis without extrarenal manifestations. CEN Case Rep 2015;4(2):212-7.  Back to cited text no. 11
Manjunath V, Moeckel G, Dahl NK. Acute kidney injury in a patient with sarcoidosis: hypercalciuria and hypercalcemia leading to calcium phosphate deposition. Clin Nephrol 2013;80(2) :151—5.  Back to cited text no. 12
Kobak S. Sarcoidosis: a rheumatologist’s perspective. Ther Adv Musculoskelet Dis 2015;7(5) :196—205.  Back to cited text no. 13
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 (1):48-52.  Back to cited text no. 14
Rajakariar R, Sharples EJ, Raftery MJ, Sheaff M, Yaqoob MM. Sarcoidtubulo-interstitial nephritis: long-term outcome and response to corticosteroid therapy. Kidney Int 2006;70(1): 165-9.  Back to cited text no. 15

Correspondence Address:
Salih A Binsalih
Department of Medicine, King Abdulaziz Medical City, P. O. Box 22490, Mail code 1443, Riyadh 11426
Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1319-2442.292321

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  [Figure 1], [Figure 2]

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