Gene/Protein Disease Symptom Drug Enzyme Compound
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Granulomatous interstitial nephritis (GIN) is a rare histologic diagnosis. This series reports the presenting features, associated conditions, treatment, and outcome of patients with a diagnosis of GIN in Glasgow during a 15-yr period and compares this with the available literature. Eighteen cases were identified: Five cases were associated with sarcoidosis, two were associated with tubulointerstitial nephritis and uveitis, two were associated with medication, and nine were idiopathic. Patients presented with advanced renal failure (median estimated creatinine clearance 21 ml/min) and minimal proteinuria (urine albumin-to-creatinine ratio 9.9 mg/mmol). Sixteen patients were treated with prednisolone for a mean of 25 mo. Six patients relapsed with reduction in prednisolone dosage, and four patients required steroid-sparing agents. During the mean follow-up of 45 mo, renal function improved or stabilized in 17 patients; the rate of improvement in renal function was most marked in the first year after diagnosis with a gain in function of +1.9 ml/min per mo. The median estimated creatinine clearance at final visit was 56 ml/min. One patient required renal replacement therapy at diagnosis but recovered renal function with treatment. No patient required long-term renal replacement therapy. There was no correlation between the degree of fibrosis or inflammation on biopsy and renal outcome, and the features on biopsy did not help to determine the cause of GIN. GIN is a treatable cause of renal failure that highlights the value of renal biopsy in patients who present with renal failure even when there is minimal proteinuria. The rarity of GIN demonstrates the need for systematic data collection.
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PMID:Granulomatous interstitial nephritis. 1769 17

Granulomatous interstitial nephritis (GIN) is an uncommon form of acute interstitial nephritis. We report a young male who presented to us with a rapidly progressing renal failure and massive proteinuria. A renal biopsy revealed GIN, and we were able to demonstrate the presence of tuberculous DNA in the biopsy specimen. The patient was started on anti-tuberculous therapy and steroids besides 11 sessions of hemodialysis. He recovered and is currently doing well. This case highlights an uncommon manifestation of renal tuberculosis, namely massive proteinuria, acute renal failure, and granulomatous interstitial lesions.
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PMID:Granulomatous interstitial nephritis due to tuberculosis-a rare presentation. 1973 86

Granulomatous interstitial nephritis (GIN) is an uncommon pathologic lesion encountered in 0.5% to 5.9% of renal biopsies. Drugs, sarcoidosis, and infections are responsible for most cases of GIN. Malignancy is not an established cause of GIN. Here, we report a series of 5 patients with GIN secondary to chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). Patients were mostly elderly white males with an established history of CLL/SLL who presented with severe renal impairment (median peak serum creatinine, 7.3 mg/dL), leukocyturia, and mild proteinuria. One had nephromegaly. In 2 patients, the development and relapse of renal insufficiency closely paralleled the level of lymphocytosis. Kidney biopsy in all patients showed GIN concomitant with CLL/SLL leukemic interstitial infiltration. Granulomas were nonnecrotizing and epithelioid and were associated with giant cells. One biopsy showed granulomatous arteritis. One patient had a granulomatous reaction in lymph nodes and skin. Steroids with/without CLL/SLL-directed chemotherapy led to partial improvement of kidney function in all patients except 1 who had advanced cortical scarring on biopsy. In conclusion, we report an association between CLL/SLL and GIN. Patients typically present with severe renal failure due to both GIN and leukemic interstitial infiltration, which tends to respond to steroids with/without CLL/SLL-directed chemotherapy. The pathogenesis of GIN in this clinical setting is unknown but may represent a local hypersensitivity reaction to the CLL/SLL tumor cells.
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PMID:Granulomatous interstitial nephritis secondary to chronic lymphocytic leukemia/small lymphocytic lymphoma. 2579 22

Sarcoidosis is a systemic disease with multiorgan involvement which can cause renal failure through several different mechanisms. Granulomatous interstitial nephritis is an important albeit less frequent cause of clinically significant renal disease. Herein, we present the case of a 46 year old woman with a history of sarcoidosis whom we evaluated for rapidly worsening kidney function and proteinuria. Renal biopsy revealed granulomatous interstitial nephritis. After therapy with adalimumab, her renal function improved with a significant reduction in proteinuria. Repeat kidney biopsy showed resolution of renal granulomata. To our knowledge, this is the first report of successful treatment of granulomatous interstitial nephritis with adalimumab.
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PMID:Treatment of sarcoid granulomatous interstitial nephritis with adalimumab. 2594 11

Human immunodeficiency virus (HIV) infection can cause a broad spectrum of renal diseases. However, there is paucity of Indian data on the patterns of renal lesions in HIV-seropositive patients. The aim of the present study was to delineate the spectrum of renal lesions in HIV/acquired immunodeficiency syndrome patients. In this prospective study, all HIV-positive patients of both genders aged >18 years were screened for renal disease. Patients with proteinuria of more than 1 g/24 h were subjected to renal biopsy. A total of 293 HIV-positive patients were screened; of these, 136 (46.4%) patients found to have renal involvement. Dipstick-positive proteinuria of 1+ or more was observed in 112 (38.2%) patients, and 16 (14.2%) patients had proteinuria of more than 1 g/24 h. Renal biopsy in 14 cases revealed glomerulonephritis (GN) in 12 (85.7%) (isolated GN in 4 [28.5%] and GN mixed with chronic TIN in 8 [57.1%]) patients. These include mesangioproliferative GN in 5 (35.7%), membranoproliferative GN in 2 (14.2%), focal segmental glomerulosclerosis in 2 (14.2%), diffuse proliferative GN in 2 (14.2%), and diabetic nephropathy in 1 (7.1%) patients. Chronic interstitial nephritis was noted in 10 (71.42%) (superimposed on GN in 8 [57.1%], isolated in 2 [14.2%]) patients. Granulomatous interstitial nephritis was seen in 3 (24.1%) cases. GN and chronic interstitial nephritis were noted in 85.7% and 71.42% of patients, respectively, mostly superimposed on each other. Mesangioproliferative GN was the most common glomerular lesion, but classical HIV-associated nephropathy was not observed.
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PMID:Kidney Disease in Human Immunodeficiency Virus-seropositive Patients: Absence of Human Immunodeficiency Virus-associated Nephropathy was a Characteristic Feature. 2876 Dec 28

Amyloidosis is a rare disease characterized by extracellular deposition of fibrils. Among the most common forms of systemic amyloidosis with renal involvement are AL-amyloidosis based on plasma cell dyscrasia and AA-amyloidosis in chronic inflammatory diseases. Depending on the affected renal compartment, the clinical appearance of renal amyloidosis varies. The pattern of renal amyloid deposition can be glomerular, interstitial, tubular or even vascular. Renal amyloid deposits are detected by renal biopsy. Patients with glomerular deposits typically show severe nephrotic syndrome with volume overload. Patients with predominantly tubulo-interstitial or vascular deposits typically exhibit lower proteinuria and progressive renal impairment. Treatment strategies for renal amyloidosis are primarily based on the treatment of the underlying disease including chemotherapy or stem cell transplantation in AL-amyloidosis or treatment of chronic inflammatory diseases in AA-amyloidosis. Granulomatous interstitial nephritis is the most common renal lesion occurring in sarcoidosis. Therapy of granulomatous interstitial nephritis is mainly based on the use of glucocorticoids.
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PMID:[Renal involvement in amyloidosis and sarcoidosis]. 2935 90

Granulomatous interstitial nephritis (GIN) is a rare entity identified in <1% of native kidney biopsies. The most frequent aetiology is drug-related, followed by systemic granulomatous conditions. Among drugs implicated in GIN, antibiotics and non-steroidal anti-inflammatory drugs (NSAIDs) are the most frequent. We report the case of a 45-year-old white man referred to a nephrology consult due to chronic kidney disease. He had a history of arterial hypertension with 10 years of evolution, hyperuricaemia, medicated with allopurinol and NSAID abuse for at least 20 years. Urine sediment was blunt, without proteinuria. Renal ultrasound was normal. A kidney biopsy revealed well-defined epithelioid granulomas with glomerular wrinkling and collapse. Infectious and systemic conditions were excluded, favouring the hypothesis of drug-induced GIN, probably related to NSAIDs. Kidney biopsy remains the gold standard for the diagnosis of GIN. Facing a patient with renal failure without significant proteinuria or active sediment, one should look for causes of tubulointerstitial injury.
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PMID:Granulomatous interstitial nephritis: a rare diagnosis with an overlooked culprit. 3140 69