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Primary interstitial kidney disease comprises a diverse group of disorders that elicit interstitial inflammation associated with tubular damage. Traditionally, interstitial nephritis has been classified morphologically and clinically into acute and chronic forms. Acute interstitial nephritis (AIN) generally induces rapid deterioration in kidney function with a marked interstitial inflammatory response characterized by mononuclear cell infiltration with or without eosinophils, interstitial edema, and varying degrees of tubular cell injury. This process typically spares both glomerular and vascular structures, and is discussed more fully in Chapter 33. By contrast, chronic interstitial nephritis (CIN) follows a more indolent course and is characterized by interstitial fibrosis, tubular atrophy, and most often an interstitial mononuclear cell infiltrate. Over time, glomerular and vascular structures are involved, with progressive parenchymal fibrosis and sclerosis. Overlap can occur between these two clinical conditions; AIN sometimes presents as a more insidious disease with progression to chronic kidney disease (CKD). Similarly, some forms of CIN are associated with acute tubular cell injury.
The histopathology of CIN is remarkably consistent despite the varied causes ( Box 43.1 ). CIN is characterized by the development of tubular atrophy with interstitial fibrosis typically with associated predominantly mononuclear cell inflammation with or without inflammation in atrophied tubules ( Fig. 43.1 ). Certain forms of CIN may have minimal inflammation (e.g., lead, lithium) or have interstitial granulomas (e.g., sarcoidosis). Glomerular and vascular structures may be relatively preserved early in the course of disease but ultimately become involved in progressive sclerosis and fibrosis. Ongoing development of tubulointerstitial fibrosis is observed in all forms of progressive kidney disease, including primary tubulointerstitial, glomerular, and vascular disorders, as a final common pathway to kidney failure.
Analgesics
Heavy metals (lead, cadmium, mercury)
Lithium
Chinese herbs (aristolochic acid)
Calcineurin inhibitors (cyclosporine, tacrolimus)
Cisplatin
Nitrosoureas
Herbicides (glyphosate, paraquat, others)
Polycystic kidney disease
Medullary cystic disease–juvenile nephronophthisis
Hereditary nephritis
Karyomegalic interstitial nephritis
Hypercalcemia/nephrocalcinosis
Hypokalemia
Hyperuricemia
Hyperoxaluria
Cystinosis
Kidney allograft rejection
Systemic lupus erythematosus
Sarcoidosis
Granulomatosis with polyangiitis (Wegener granulomatosis)
Vasculitis
Sjögren syndrome
Tubulointerstitial nephritis and uveitis (TINU) syndrome
IgG4 disease
Anti-low-density lipoprotein receptor-related protein 2 (LRP2) nephropathy
Multiple myeloma
Light chain disease
Dysproteinemias
Lymphoproliferative disease
Sickle cell disease
Kidney
Systemic
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