d. Empiric initial therapy consists of intravenous pulse methylprednisolone (500 to 1000 mg/day for three days) and consideration of plasmapheresis, especially if the patient has hemoptysis.
he nephrotic syndrome is unusual and is most likely to occur in patients with less severe kidney function impairment
progressive loss of kidney function
extensive crescent formation (extracapillary proliferation in Bowman's space)
type of immunologic process
degree of crescent formation
circumferential crescents in more than 80 percent of the glomeruli tend to present with advanced kidney failure that may not respond well to therapy
Rents (focal gaps or discontinuities of the capillary wall) are induced in the glomerular capillary wall, resulting in the movement of plasma products, including fibrinogen, into Bowman's space with subsequent fibrin formation,
Many of these diseases can often be diagnosed from the history, clinical findings, and laboratory testing.
This transition is important clinically because fibrous crescents represent a stage of the disease that is not likely to respond to immunosuppressive therapy.
In one series of over 630 patients, 60 percent had pauci-immune crescentic GN, 24 percent had immune complex-mediated GN, and 15 percent had anti-GBM disease
nd 45 percent had immune complex-mediated GN
Among patients between 1 and 20 years old,
; antinuclear antibodies, a "full house" immunofluorescence staining for immunoglobulin G (IgG), IgA, immunoglobulin M (IgM), C3 and C1q, with mesangial plus subendothelial deposits in lupus nephritis; and circulating cryoglobulins and intraluminal "thrombi" in mixed cryoglobulinemia.
The majority of patients with renal-limited vasculitis are ANCA positive, and many have or will develop the systemic symptoms of granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA)
Some studies suggest that antibodies directed against lysosome-associated membrane protein 2 (LAMP-2) may be present in over 90 percent of ANCA-positive patients with pauci-immune necrotizing crescentic GN,
Some cases of ANCA-positive disease are induced by drugs (eg, propylthiouracil, hydralazine, allopurinol, penicillamine, minocycline, rifampicin, levamisole).
does not fit into any of the identifiable categories, and a pauci-immune disease that is ANCA negative. The former is rare , while the latter accounts for less than 5 percent of cases of crescentic GN.