Rapidly progressive glomerulonephritis diagnostic study of choice
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Rapidly progressive glomerulonephritis Microchapters |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Ali Poyan Mehr, M.D. [2] Associate Editor(s)-in-Chief: Krzysztof Wierzbicki M.D. [3], Amandeep Singh M.D.[4], Ahmed Elsaiey, MBBCH [5]
Overview
Rapid diagnosis of rapidly progressive glomerulonephritis is very crucial to save kidneys function
Diagnostic study of choice:
Renal biopsy:
- Renal biopsy will provide the most accurate reslt.
- Renal biopsy will give accurate information about the extent of the disease and therapy can be planned accordingly.
results of Renal biopsy:
- Light microscopy reveals
- Diffuse inflammation in glomeruli with rupture and damage to glomerular basement membrane.
- Crescents are present in the Bowmans space.
- Renal vessels can show transmural vasculitis, with necrosis and lymphocyte infiltrates.
- Tubular necrosis may also be present.
- Interstitial granulomas in the glomeruli indicate Wegener’s granulomatosis.
Immunoflourescence
- In type I RPGN- diffuse and linear deposition of IgG along the GBM.
- In ttype II RPGN- diffuse and irregular deposition of IgG and C3 in the mesangial matrix.
- In type III RPGN- no finding.
Diabetic nephropathy and fibrillary glomerulonephritis can be easily distinguished from anti-GBM disease on both clinical and histopathologic grounds. Circulating anti-GBM antibodies are absent in both disorders, and crescents are not seen in diabetic nephropathy. The history of diabetes, the finding of diabetic glomerulosclerosis on light microscopy, and, in fibrillary glomerulonephritis, the presence of the characteristic fibrils on electron microscopy are also helpful.
Serologic studies
- Complete blood cell count (CBC) withdifferential,
- Anemia can be seen in patienst with renal failure or gastrointestinal tract bleeding.
- Eosinophilia of 13% or greater suggest Churg-Strauss disease.
- Serum electrolytes
- BUN(blood urea nitrogen)
- Serum creatinine
- Lactate dehydrogenase (LDH)
- Creatine phosphokinase (CPK),
- The most common abnormality is an increased serum creatinine level
- Urinalysis with microscopy: Proteinuria equal to or greater than 2-3 g in 24 hours.
- Microscopic hematuria
- Red cell casts indicates glomerular inflammation
- Erythrocyte sedimentation, elevated with active disease.
- C-reactive protein: levels are elevated and correspond with disease activity.
- Antinuclear antibody (ANA).High ANA titer is present in systemic lupus erythematosus.
- ANCA with ELISA subtyping: More than 80% of patients with microscopic polyangiitis are ANCA-positive, and most of these demonstrate pANCA with MPO specificity. Of patients with granulomatosis with polyangiitis, 90% are ANCA-positive and most have cANCA with PR3 specificity, especially in pulmonary involvement. However, ANCA type and specificity is not pathognomonic for each of these clinical syndromes because some patients with granulomatosis with polyangiitisare pANCA-positive and some patients with microscopic polyangiitis are cANCA-positive. Simon and colleagues investigated the presence of anti-pentraxin 3 (PTX3)- autoantibodies (aAbs) in the sera of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) patients and found that anti-PTX3 aAbs were present in nearly 40% of patients studied including in patients without detectable MPO and PR3 ANCA.
- Urine and serum protein electrophoresis,helpful in light-chain disease or multiple myeloma..
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