SESSION TYPE: ILD Case Report Posters
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Granulomatosis with polyangiitis (GPA) primarily affects the kidneys and upper and lower airways; however, involvement of other organ systems may also occur.
CASE PRESENTATION: A 67 year old male presented for chest imaging due to prior pleural plaques. He was a former boilermaker with a 54-pack-year smoking history. On initial evaluation he had mild bilateral serous otitis media but no dyspnea. He previously had an acute illness characterized by fatigue, shortness of breath, and fevers that resolved with antimicrobial therapy. Computed tomography scan of the chest revealed multiple new parenchymal cavitary nodules. A core transthoracic needle biopsy showed capillaritis, focal vasculitis and alveolar hemorrhage. C- and PR3-ANCA were elevated. Patient was given methotrexate and prednisone with resolution of parenchymal nodules three months later. However, six months later, while still on immunosuppressive therapy, he developed cough, dyspnea, epistaxis, and acute renal failure with new patchy areas of dense nodular consolidations. Bronchoalveolar lavage showed no evidence of infection. He was hospitalized for 14 days and treated with methylprednisolone, cyclophosphamide, and plasmapheresis. He was discharged on cyclophosphamide and prednisone. Three months later the patient was readmitted with progressive edema and worsened dyspnea despite normalized renal function and undetectable ANCA. Ultrasound revealed deep vein thrombosis. He developed atrial flutter with 2:1 conduction which resolved with amiodarone. Several months later cytoxan was converted to cellcept and prednisone therapy was gradually weaned. He has gradually improved and ANCA level remained undetectable over two years with progressively decreasing immunosuppressive therapy.
DISCUSSION: This case illustrates several important features of GPA. GPA can present with minimal to no symptoms; and clinical or radiographic progression can occur while on therapy. Remission can be difficult to maintain, and relapses occur in over half of patients, typically following cessation of immunosuppressive therapy. Reported risk factors for recurrence include involvement of lung and upper respiratory tract, PR3 positivity, and lack of renal involvement. Cardiac complications occur in up to 44% of patients and consist of pericarditis, arteritis, myocarditis, valvulitis, conduction disturbances and arrhythmias. Up to 16% of patients with c-ANCA vasculitis develop venous thromboembolic events during an active phase of disease.
CONCLUSIONS: GPA can present with minimal to no symptoms and can progress or relapse despite therapy. It is important to consider GPA in a differential diagnosis of arrhythmias and venous thromboembolic disease.
1) Hogan et al. Predictors of relapse and treatment resistance in c-ANCA-associated small-vessel vasculitis. Ann Intern Med. 2005;143(9):621-31.
DISCLOSURE: The following authors have nothing to disclose: Lioudmila Karnatovskaia, Margaret Johnson
No Product/Research Disclosure InformationMayo Clinic, Jacksonville, FL