Rapidly progressive glomerulonephritis diagnostic study of choice
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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 ,
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..
Biopsy
Determination of ANCAs can aid in the diagnosis, but positivity is not conclusive and negative ANCAs are not sufficient to reject the diagnosis. Cytoplasmic staining ANCAs that react with the enzyme proteinase 3 (cANCA) in neutrophils (a type of white blood cell) are associated with Granulomatosis with polyangiitis. If the patient has renal failure or cutaneous vasculitis, these are the most logical organs to obtain a biopsy from. Rarely, thoracoscopic lung biopsy is required.
Diagnostic Criteria
A diagnosis of Granulomatosis with polyangiitis can be made when three out of the six criteria are established. They are:[1]
- a histopathology that shows granuloma
- the upper respiratory tract is involved
- there is a stenosis that is present in larynx, trachea, and the bronchioles
- the pulmonary system is involved
- the presence of anti-neutrophil cytoplasmic antibodies
- the presence of glomerulonephritis
In 1990, the American College of Rheumatology accepted classification criteria for Granulomatosis with polyangiitis. They were not intended for diagnosis, but for inclusion in randomized controlled trials. Two or more positive criteria have a sensitivity of 88.2% and a specificity of 92.0% of describing Granulomatosis with polyangiitis.[2]
- Nasal or oral inflammation:
- painful or painless oral ulcers or
- purulent or bloody nasal discharge
- Lungs: abnormal chest X-ray with:
- nodules,
- infiltrates or
- cavities
- Kidneys: urinary sediment with:
- microhematuriaor
- red cell casts
- Biopsy: granulomatous inflammation
- within the arterial wall or
- in the perivascular area
According to the Chapel Hill Consensus Conference (CHCC) on the nomenclature of systemic vasculitis (1992), establishing the diagnosis of Granulomatosis with polyangiitis demands:[3]
- a granulomatous inflammation involving the respiratory tract, and
- a vasculitis of small- to medium-sized vessels.
Several investigators have compared the ACR and Chapel Hill criteria.[4]
References
- ↑ Noone D, Hebert D, Licht C (2016). "Pathogenesis and treatment of ANCA-associated vasculitis-a role for complement". Pediatr Nephrol. ( ): . doi:10.1007/s00467-016-3475-5. PMID 27596099.
- ↑ Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum 1990;33:1101-7. PMID 2202308.
- ↑ Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG,et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference.Arthritis Rheum 1994;37:187-92. PMID 8129773.
- ↑ Bruce IN, Bell AL. A comparison of two nomenclature systems for primary systemic vasculitis. Br J Rheumatol1997;36:453-8. PMID 9159539.