Saudi Journal of Kidney Diseases and Transplantation

CASE REPORT
Year
: 2011  |  Volume : 22  |  Issue : 6  |  Page : 1208--1210

Isolated sarcoid renal granulomatous tubulointerstitial disease


Amal Abdel Ghani1, Salah Al Waheeb2, Ekhlas Al Homoud3,  
1 Nephrology Unit, Mubarak Al Kabeer Hospital, Ministry of Health, Kuwait
2 Department of Histopathology, Mubarak Al Kabeer Hospital, Ministry of Health, Kuwait
3 Department of Medicine, Mubarak Al Kabeer Hospital, Ministry of Health, Kuwait

Correspondence Address:
Amal Abdel Ghani
Nephrology Department, Mubarak Al Kabeer Hospital, P.O. Box 43787, Code 3205, Hawally
Kuwait

Abstract

A 37-year-old lady presented with hypercalcemia and acute renal impairment. She had no previous medical problems apart from the use of non steroidal anti-inflammatory drugs for nonspecific body pains. Her abdominal ultrasound scan as well as urine studies were nonspecific. Further workup for hypercalcemia (skeletal survey, high resolution computed tomography (CT) of the chest and abdomen, purified protein derivative (PPD) test, serum protein electrophoresis, tumor markers, immunology screening, and Bence Jones proteinuria) was negative. Serum angiotensin converting enzyme was high. Renal biopsy showed extensive lymphocytes and multinucleated giant cells infiltration forming interstitial non necrotizing granulomata. Immune staining as well as staining for acid fast bacilli was negative. The possibility of sarcoid renal granulomata was raised and the patient was started on oral prednisolone with subsequent normalization of renal functions and serum calcium after one month of treatment.



How to cite this article:
Ghani AA, Waheeb SA, Homoud EA. Isolated sarcoid renal granulomatous tubulointerstitial disease.Saudi J Kidney Dis Transpl 2011;22:1208-1210


How to cite this URL:
Ghani AA, Waheeb SA, Homoud EA. Isolated sarcoid renal granulomatous tubulointerstitial disease. Saudi J Kidney Dis Transpl [serial online] 2011 [cited 2019 Dec 6 ];22:1208-1210
Available from: http://www.sjkdt.org/text.asp?2011/22/6/1208/87235


Full Text

 Introduction



Granulomatous interstitial nephritis (GIN) is a rare histologic diagnosis that is present in 0.5-0.9% of native renal biopsies [1],[2] and 0.6% of renal transplant biopsies. [3] GIN has been associated with medications, infections, sarcoidosis, crystal deposits, paraproteinemia, Wegener's granulomatosis, and also is seen in an idiopathic form. [4] GIN may be the first manifesation of systemic diseases such as sarcoidosis. [5]

 Case Report



A 37-year-old lady presented to our nephrology service with lethargy, generalized body aches, hair fall, arthralgia, and weight loss of three months duration for which she was using non steroidal anti-inflammatory drugs. There were no arthritis, no deformities, and no mucocutaneous manifestations detected on physical examination and she was vitally stable. Her past medical history was unremarkable.

Her laboratory investigations on presentation revealed blood urea nitrogen (BUN) of 15.7 mmol/L, serum creatinine 480 μmol/L, serum bicarbonate 17.8 mmol/L, serum corrected calcium 3.17 mmol/L, serum phosphorus 1.7 mmol/ L, and serum alkaline phosphatase 68 IU/L. Urinalysis showed no active sediments. Her sonography showed normal kidneys; skeletal survey was negative, high resolution computed tomography (CT) of the chest and abdomen was normal, and tuberculin skin test was negative. Further blood analysis was normal for protein electrophoresis, tumor markers, immnology screening, culture and sensitivity, but showed elevated serum angiotensin converting enzyme levels. Urine was negative for acid fast bacilli by staining and culture and there was no detectable Bence Jones proteinuria. A renal biopsy showed normal glomeruli with extensive tubulointerstitial infiltration with lymphocytes and multinucleated giant cells forming interstitial non necrotizing granulomata [Figure 1], [Figure 2], [Figure 3], [Figure 4] and [Figure 5]. Immune staining as well as staining for acid fast bacilli was negative. The possibility of sarcoid granuloma was raised and the patient was started on oral prednisolone 40 mg/d. After one week of steroid treatment, renal functions showed improvement with BUN of 11 mmol/L, serum creatinine 243 μmol/L, and serum corrected calcium 2.79 mmol/L.

The patient's case was reviewed after one month of treatment with marked improvement of her renal functions with BUN of 4.6 mmol/L, serum creatinine 85 μmol/L, serum corrected calcium 2.3 mmol/L. Treatment was continued for six months, then tapered off slowly with complete recovery of her renal functions and normalization of serum calcium.{Figure 1}{Figure 2}{Figure 3}{Figure 4}{Figure 5}

 Discussion



Sarcoidosis is a multi-system disease in which the kidney may be involved. [3] It is characterized by the formation of non-caseating granulomata in various organs. The sarcoid granuloma, which is the hallmark of the disease, was first described by Schaumann in 1933 as a rounded cellular collection of epitheloid cells, multinucleated giant cells surrounded by a rim of lymphocytes and macrophages in association with various degrees of fibrosis without caseation. [4]

The first case of isolated renal sarcoidosis was described in 1976 by Spargo. [6] Up till now, only 100 cases of isolated renal sarcoidosis have been described in the medical literature. [7] Sarcoidosis can affect the kidney via hypercalcemia caused by the overproduction of vitamin D by lymphocytes; granulomatous infiltration of the interstitium; a variety of glomerulopathies; miscellaneous causes such as AA amyloidosis and renal artery stenosis (sarcoid angitis). [8] Hypercalcemia can affect the kidney through its hemodynamic effect leading to a decrease in the glomerular filtration rate, impairment of urinary concentration ability, and formation of nephrocalcinosis (<5%) and nephrolithiasis (<10%) resulting in obstructive uropathy. [8]

Steroids are the main stay of treatment as it blocks the extra renal calcitriol production. It should be prescribed for six months to one year and it usually results in an excellent response. [9] Other lines of treatment include aminoquinolone derivatives, calcium binding agents, cyclophosphamide, thalidomide, pentoxyphylline, infleximab, cyclosporine, methotrexate, and azathioprine. [10],[11]

We conclude that isolated renal sarcoidosis should be suspected in any case of acute renal failure with hypercalcemia and granulomatous interstitial infiltrations even in the absence of any systemic manifestation of sarcoidosis.

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