2006, Number 3
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Med Int Mex 2006; 22 (3)
Microscopic polyangiitis and rapid progression glomerulonephrtitis: importance of timely diagnosis and treatment. A report of a case
Flores PG, Ávila BRR, Moncada TE
Language: Spanish
References: 21
Page: 253-256
PDF size: 222.90 Kb.
ABSTRACT
Microscopic polyangiitis is a necrotizing vasculitis affecting small vessels, with renal and lung involvement and the presence of anti-neutrophil cytoplasmic antibodies (ANCA). This paper describes the case of a 53 year old male with fever, myalgias, arthralgias, sore throat, and purulent rinorrhea. He was treated with antibiotics several times without symptom remission. He was admitted to the hospital five months later because of acute renal failure and deterioration of his general status. Physical examination revealed lower limb edema and generalized arthalgias. Laboratory reported serum creatinine of 20.6 mg/dL, urea 368 mg/dL, hemoglobin 8.3 g/dL, and a highly positive pANCA. Urinary sediment showed glomerular damage and a crescentic and necrotizing proliferative glomerulonephritis on the kidney biopsy. During hospitalization the patient developed hemoptysis, dyspnea and fever. The chest X ray was compatible with pulmonary hemorrhage. A diagnosis of microscopic polyangiitis was established based on clinical, laboratory and biopsy data. The patient was treated with steroids and cyclophosphamide. He is now asymptomatic and on hemodyalisis. Initial symptoms of microscopic polyangiitis are usually nonspecific and last for several months before the diagnosis is made. A longer time to diagnosis is associated with a 70% increased risk of end stage renal failure and permanent dyalisis requirement due to a rapidly progressive glomerulonephritis. Prompt diagnosis and treatment are imperative for renal function recovery.
REFERENCES
Fernández PP, Álvarez RS, Jones CM. Poliangeítis microscópica. Piel 2003;18(5):259-63.
Matteson EL. Historical perspective on the classification of vasculitis. Arthritis Care Res, 2000;13(2):122-7.
Watts RA, Lane SE, Bentham G, Scott DG. Epidemiology of systemic vasculitis. Arthritis Rheum 2000;43(2):414-9.
Jennette JC, Thomas DB. Crescentic glomerulonephritis. Nephrol Dial Transplant 2001;16(s6):80-2.
Agard C, Mouthon L, Mahr A, Guillevin L. Microscopic polyangiitis and polyarteritis nodosa: How and when do they start? Arthritis Rheum 2003;49(5):709-15.
Watts RA, Scott DG, Lane SE. Epidemiology of Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome. Clev Clin J Med 2002;69(S2):84-7.
Bajema IM, Hagen EC, Hermans J, et al. Kidney biopsy as a predictor for renal outcome in ANCA-associated necrotizing glomerulonephritis. Kidney Int 1999;56(5):1751-89.
Hauer HA, Bajema IM, van Houwenlingen HC, et al. Renal histology in ANCA-associated vasculitis: Differences between diagnostic and serologic subgroups. Kidney Int 2002;61:80-89.
Vizjak A, Rott T, Koselj-Kajtna M, et al. Histologic and immunohistologic study and clinical presentation of ANCA-associated glomerulonephritis with correlation to ANCA antigen specificity. Am J Kidney Dis 2003;41(3):539-49.
Mansi IA, Opran A, Rosner F. ANCA-associated small-vessel vasculitis. Am Fam Physician 2002;65(8):1615-21.
Kamesh L, Harper L, Savage C. ANCA-Positive vasculitis. J Am Soc Nephrol 2002,13:1953-60.
Harper L, Savage C. Pathogenesis of ANCA-associated systemic vasculitis. J Pathol 2000;190:349-59.
Hauer HA, Hagen EC, de Heer E, et al. Glomerulonephritis in the vasculitides: Advances in immunopathology. Curr Opin Rheumathol 2003;15:17-21.
Muller Kobold AC, van den Geld YM, Linburg PC, et al. Pathophysiology of ANCA-associated glomerulonephritis. Nephrol Dial Transplant 1999;14:1366-75.
Guillevin L, Durand-Gasselin B, Cevallos R, et al. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum 1999;42:421-30.
Russel KA, Fass DN, Specks U. Antineutrophil cytoplasmic antibodies reacting with the pro form of proteinase 3 and disease activity in patients with Wegener’s granulomatosis and microscopic polyangiitis. Arthritis Rheum 2001;44:463-8.
Vergunst CE, van Gurp E, Hagen EC, et al. An index for renal outcome in ANCA-associated glomerulonephritis. Am J Kidney Dis 2003;41(3):532-8.
Gayraud M, Guillevin L, le Toumelin P, et al. Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome. Arthritis Rheum 2001;44(3):666-75.
Jayne DR. Conventional treatment and outcome of Wegener’s granulomatosis and microscopic polyangiitis. Clev Clin J Med 2002;69(s2):110-5.
Guillevin L. Treatment of polyarteritis nodosa and microscopic polyangiitis with poor prognosis factors: A prospective trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in sixty-five patients. Arthritis Rheum 2003;4 (1):93-100.
Plassance M, Goldsmith DJ. Spontaneous and protracted partial remission of microscopic polyangiitis. AmJ Kidney Dis 2002;39(5):1113-7.