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tubulointerstitial nephropathy

Diseases of the renal tubules & interstitium. Etiology: 1) acute interstitial nephritis 2) secondary tubulointerstitial injury - glomerulonephritis - vasculitis - hypertensive nephrosclerosis 3) immunologic (autoimmune disease) - Sjogren syndrome - sarcoidosis - systemic lupus erythematosus - anti-tubular basement membrane antibody - tubulointerstitial nephritis with uveitis - IgG4 disease 4) infectious a) risk factors - pre-existing kidney disease - immunologic disease b) viral - polyoma BK virus (post renal transplantation) - JC virus - cytomegalovirus (CMV) - adenovirus - Epstein-Barr virus - hepatitis C virus - antiviral tenofovir used to treat HIV1, hepatitis B [1] c) bacterial: - chronic pyelonephritis - Rickettsia - leptospirosis - Brucellosis - Legionella d) Mycobacterial - Mycobacterium tuberculosis - Mycobacterium avium complex e) helminth infection: schistosomiasis f) mycosis (fungal infection) g) protozoan infection: toxoplasmosis 5) inherited a) polycystic kidney disease b) medullary sponge disease c) uromodulin-related kidney disease - medullary cystic kidney disease type 2 - familial juvenile hyperuricemic nephropathy - sickle cell disease d) nephronophthisis 6) malignancy - multiple myeloma - leukemia - lymphoma 7) pharmaceuticals a) analgesics (analgesic nephropathy) - phenacetin, aspirin, NSAIDs, COX2 inhibitors b) caffeine in combination with analgesics c) calcineurin inhibitors - cyclosporine, tacrolimus d) antivirals - tenofovir [1] e) lithium carbonate f) proton pump inhibitors [1] g) aristolochic acid nephropathy (Chinese herb) - Balkin endemic nephropathy h) prolonged exposure to any medication that can cause acute interstitial nephritis - allopurinol, cephalosporins, fluoroquinolones, H2 blockers, indinavir, mesalamine, penicillins, proton pump inhibitors, rifampin, sulfonamides 8) metabolic - heavy metals (lead, mercury, cadmium) - hyperuricemia - cystinosis - hyperoxaluria, nephrocalcinosis - phosphate nephropathy 9) obstructive uropathy a) prostatic hyperplasia b) urinary reflex nephropathy c) nephrolithiasis d) malignancies: - cervical cancer - prostate cancer - bladder cancer Pathology: - antigens on tubular proteins or tubular epithelial cells may initiate renal injury - renal tubules, interstitium &/or genitourinay tract may be affected with relative sparing of the glomeruli - may result from renal glomerular disease (glomerulonephritis) or renal vascular disease - degree of tubulointerstitial fibrosis correlates with progression to end-stage renal disease - lymphocytic infiltration, interstitial fibrosis, renal tubular atrophy, arteriosclerosis, diffuse glomerulosclerosis Genetics: - autosomal dominant form - family history of end-stage renal disease [1] Clinical manifestations: - slowly progressive chronic renal failure - Fanconi syndrome - renal tubular acidosis - polyuria, isosthenuria, nocturia Laboratory: - complete blood count (CBC) - anemia (injury to erythropoietin-producing cells) - eosinophilia - serum chemistries a) serum urea nitrogen increased b) serum creatinine increased c) serum potassium: hyperkalemia d) serum bicarbonate decreased (RTA) e) serum phosphate low - glomerular filtration rate: decline in GFR - urine osmolality: isosthenuria - urine protein below nephrotic range (< 2 g/24 hours) - urinalysis - pyuria, eosinophiluria - occasional granular casts - Fanconi syndrome - glucosuria despite normal serum glucose - aminoaciduria Special laboratory: - renal ultrasound - atrophic, echogenic kidneys consistent with chronic renal disease - exclude obstructive uropathy - renal biopsy Radiology: - computed tomography as needed based upon renal ultrasound Management: - treatment generally results in limited improvement - treatment of underlying cause(s) may slow progression of disease - avoid nephrotoxic agents & use of intravenous contrast agents

Interactions

disease interactions

Specific

Balkan nephropathy Fanconi renotubular syndrome interstitial nephritis renal tubular disease

General

nephropathy

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 19 American College of Physicians, Philadelphia 2009, 2012, 2015, 2021
  2. Bleyer AJ, Hart PS, Kmoch S. Hereditary interstitial kidney disease. Semin Nephrol. 2010 Jul;30(4):366-73. Review. PMID: 20807609 Free PMC Article
  3. Bollee G, Dahan K, Flamant M et al Phenotype and outcome in hereditary tubulointerstitial nephritis secondary to UMOD mutations. Clin J Am Soc Nephrol. 2011 Oct;6(10):2429-38. PMID: 21868615 Free PMC Article