Diffuse Lung Disease |

Rapidly Progressive Pattern of Granulomatosis With Polyangiitis (Wegener’s) - Clinical Case FREE TO VIEW

Claudia Toma, MD; Ionela Belaconi, MD; Stefan Dumitrache-Rujinski, MD; Liliana Grigoriu, MD; Alina Croitoru, MD; Miron Bogdan, PhD
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Carol Davila University of Medicine and Pharmacy, Bucharest, Romania

Chest. 2014;145(3_MeetingAbstracts):223A. doi:10.1378/chest.1832567
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SESSION TITLE: Diffuse Lung Disease Case Report Posters

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PM

INTRODUCTION: Granulomatosis with polyangiitis (Wegener’s), abbreviated as GPA, is an antineutrophil cytoplasmic antibody (ANCA) - associated vasculitides which have mainly upper and lower airways, and renal involvement.

CASE PRESENTATION: We present the case of a 70 years-old female patient, nonsmoker, with no previous significant medical history, admitted for a history of 2 weeks of cough, sputum production, and arthralgia which associated hemoptysis in the last 4 days. Physical exam revealed no fever, pale skin, bilateral basal crackles on pulmonary auscultation, SaO2 95% (air ambient and rest), normal blood pressure and pulse, no peripheral edema, no signs of peripheral thrombosis. Routine laboratory showed moderate iron deficiency anemia (Hemoglobin 7.8g/dL, Hematocrit 24.7%, and serum iron level 27mcg/dL), elevated ESR (88mm/H), microscopic proteinuria and hematuria on urianalysis. Chest radiography and CT-scan revealed bilateral patchy consolidation with air bronchogram and no necrosis. Lung function tests showed only a mild restrictive pattern (mild reduction in VC and FEV1) with normal DLco (85.7% predicted). Bronchoscopy showed no bronchial lesions and BAL had 93.4% neutrophils and severe alveolar hemorrhage syndrome. The clinical status of the patient deteriorates rapidly in 4 days from hospital admission and respiratory failure developed. Urgent thoracotomy with lung biopsy was performed, and immediately after the biopsy the immunosuppressive treatment with pulse therapy with Methylprednisolone and Cyclophosphamide was given to the patient. The lung biopsy showed polyangiitis with granulomatosis, confirming the diagnosis of GPA. PR3-ANCA (c-ANCA) antibodies came positive. After treatment, patient’s clinical status improved in a few days and was discharged from the hospital after 10 days. She continued the immunosuppressive treatment as outpatient.

DISCUSSION: GPA can be a severe disease with a fatal outcome without a rapid diagnosis and a correct management, usual in a specialized center.

CONCLUSIONS: Prompt diagnosis of GPA is very important due to the fact that the immunosuppressive therapy may save the life of the patient.

Reference #1: Hogan SL, Falk RJ, Chin H, et al. Predictors of relapse and treatment resistance in antineutrophil cytoplasmic antibody-associated small-vessel vasculitis. Ann Intern Med 2005; 143:621.

Reference #2: Hogan SL, Falk RJ, Nachman PH, Jennette JC. Various forms of life in antineutrophil cytoplasmic antibody-associated vasculitis. Ann Intern Med 2006; 144:377.

Reference #3: J. Charles Jennette, M.D., and Ronald J. Falk, M.D. N Engl J Med 1997; 337:1512-1523

DISCLOSURE: The following authors have nothing to disclose: Claudia Toma, Ionela Belaconi, Stefan Dumitrache-Rujinski, Liliana Grigoriu, Alina Croitoru, Miron Bogdan

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