2003;8:107–10. (Level 4)   Chapter 13: Rapidly progressive glomerulonephritic syndrome RPGN and CKD RPGN(rapidly progressive glomerulonephritis)is defined by the World Health Organization (WHO) as “a syndrome of diseases presenting with insidiously developing hematuria and proteinuria and rapidly progressive renal failure,” and in Japan as “a syndrome of diseases in which renal failure subacutely develops for several weeks to months associated with urine abnormalities indicative of glomerulonephritis”

(Table 8). RPGN includes a wide variety Stattic of rapidly progressive renal diseases (ANCA-positive RPGN, lupus nephritis, anti-GBM antibody glomerulonephritis, etc.) and the definition does not require reference to the renal pathology, which often shows necrotizing and crescentic glomerulonephritis. The prognosis is poor as the initial therapy is delayed, thus it is important to make a diagnosis as early as possible according to the “diagnostic criteria for the early detection of RPGN” (Tables 8, 9). Table 9 Diagnostic criteria for early detection of rapidly progressive glomerulonephritis (1) Urine abnormalities (esp. hematuria, proteinuria, casts) (2) eGFR <60 mL/min/1.73 m2 (3) Elevated CRP and ESR * If the above criteria are fulfilled, referral to a nephrology clinic is recommended after confirming the absence of renal cortex atrophy

by ultrasonography, if available. If infection or exacerbation of chronic nephritis is suspected, serum creatinine should be reexamined and the eGFR value calculated after 1 or 2 weeks There is an increasing number of cases of SHP099 RPGN that initially only show asymptomatic urine findings. With the occurrence of a recently

appearing urine abnormality, RPGN should be considered even if the renal function appears to be almost normal eGFR should be calculated by the equation used for the Japanese Regarding the relationship between RPGN and CKD, of note is that differentiating RPGN from CKD (chronic glomerulonephritic syndrome) is not possible with only one visit. Therefore, the possibility of RPGN should be considered even if the patient’s serum creatinine level remains slightly above or even within the reference Selleckchem Abemaciclib values, because serum creatinine does not necessarily reflect renal function within next that low range of values. Thus, it is important to re-examine the renal function within several weeks. Some of the patients with RPGN will be followed as CKD after their initial therapy. Such patients may be managed according to the clinical practice guidelines for CKD in addition to maintenance immunosuppressive therapy. RPGN may develop de novo, or as an exacerbation of chronic glomerulonephritis during the course of CKD. Small kidney size generally suggests the presence of CKD, but the fact that RPGN can develop from CKD cannot be ignored. Are corticosteroids recommended as initial therapy for RPGN? Corticosteroids are widely used as initial therapy for various causes of RPGN.