Disease Information for Nephritis, familial interstitial

Diagnosis: Fever; Transient maculopapular rash; Acute renal insufficiency; Pyuria (including eosinophiluria), white blood cell casts, and hematuria; Acute interstitial nephritis accounts for 10-15% of cases of intrinsic renal failure; An interstitial inflammatory response with edema and possible tubular cell damage is the typical pathologic finding; Cell-mediated immune reactions prevail over humoral responses; T lymphocytes can cause direct cytotoxicity or release lymphokines that recruit monocytes and inflammatory cells; Although drugs account for over 70% of cases, acute interstitial nephritis also occurs in infectious diseases, immunologic disorders, or as an idiopathic condition; The most common drugs are penicillins and cephalosporins, sulfonamides and sulfonamide-containing diuretics, NSAIDs, rifampin, phenytoin, and allopurinol; Infectious causes include streptococcal infections, leptospirosis, cytomegalovirus, histoplasmosis, and Rocky Mountain spotted fever; Immunologic entities are more commonly associated with glomerulonephritis, but systemic lupus erythematosus, Sjögren"s syndrome, sarcoidosis, and cryoglobulinemia can cause interstitial nephritis; Clinical features can include fever (> 80%), rash (25- 50%), arthralgias, and peripheral blood eosinophilia (80%); The urine often contains red cells (95%), white cells, and white cell casts; Proteinuria can be a feature, particularly in NSAID-induced interstitial nephritis but is usually modest; Eosinophiluria can be detected by Wright"s or Hansel"s stain; Acute interstitial nephritis often carries a good prognosis; Recovery occurs over weeks to months, but acute dialytic therapy may be necessary in up to one-third of all patients before resolution; Patients rarely progress to ESRD; Those with prolonged courses of oliguric failure and advanced age have a worse prognosis; Treatment consists of supportive measures and removal of the inciting agent; If renal failure persists after these steps, a short course of corticosteroids can be given; Short-term, high-dose methylprednisolone 4 days or prednisone 2 weeks followed by a prednisone taper can be used in these more severe cases of drug-induced interstitial nephritis; [CMDXRX2005]

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