Disorders of the Pleura |

ANCA Negative Limited GPA (Wegener's) of the Lung in the Setting of Severe Ulcerative Colitis FREE TO VIEW

Imam Shaik, MD; Jyoti Matta, MD; Rumana Khan, MD; Valentin Marian, MD; Saira Shah, MD
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Jersey City Medical Center, Bayonne, NJ

Chest. 2014;146(4_MeetingAbstracts):465A. doi:10.1378/chest.1994564
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SESSION TITLE: Miscellaneous Student/Resident Case Report Posters I

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: The diagnosis of ANCA negative limited form of GPA requires high suspicion with histopathologic confirmation in appropriate clinical setting. Approximately 10 percent of patients with systemic GPA and up to 40 percent with limited GPA will be ANCA negative. Here in we report a rare case of ANCA negative limited GPA with involvement of Lung in a patient previously diagnosed with Ulcerative colitis (UC).

CASE PRESENTATION: A 25-year-old African American Male with refractory Ulcerative colitis presented with one-week history of abdominal pain, bloody diarrhea and occasional fevers. His work up showed diffuse large bowel colitis and pulmonary nodules on the CT scan, endoscopies revealed diffuse colonic inflammation with crypt abscesses on biopsy. The work up for infection, pulmonary embolism, and septic emboli was negative. The CT-guide biopsy of pulmonary nodules revealed small vessel vasculitis with necrosis and granulomas. All the cultures were negative including acid fast bacilli and fungi after six-weeks. The work up for ANCAs, ASCA and other autoimmune conditions was negative except for elevated ESR and CRP. The diagnosis of limited GPA was made based on ACR 1990 criteria.

DISCUSSION: The association of Ulcerative colitis and GPA is extremely rare. Though respiratory tract and kidneys involve most of the cases, potentially every organ can involve. In our case, the patient had multiple endoscopies and colonoscopies, all suggestive of pancolitis He was treated with sulfasalazine, mesalamine, infliximab and corticosteroids with limited response. Few case reports described granulomatous inflammation in colectomy specimens, which were often negative in mucosal biopsies as in our case. Late onset of GPA in patients with UC thought to be a secondary complication to drugs. The colonic manifestations could actually be a part of diffuse vasculitis of the gastrointestinal system vs. co-occurrence. On the other hand, UC develops in genetically susceptible individuals who have abnormalities of humoral and cell-mediated immunity and perhaps is not totally unreasonable to speculate that another autoimmune inflammatory condition that affects the small vessels, such as GPA, could be a different phenotypical expression of the same immune or genetic dysregulation.

CONCLUSIONS: Up to 80 percent of patients with limited GPA eventually develop glomerulonephritis. The early diagnosis and therapy may be life saving and organ sparing.

Reference #1: 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.

Reference #2: Sokol RJ, Farrell MK, McAdams AJ. An unusual presentation of Wegener’s granulomatosis mimicking inflammatory bowel disease. Gastroenterology 1984; 87: 426-32.

Reference #3: Domschke W (University of Munster, Munster, Germany). Colitis as the initial presentation of Wegener’s granulomatosis (Case Report). J Intern Med 1997; 242: 513-517.

DISCLOSURE: The following authors have nothing to disclose: Imam Shaik, Jyoti Matta, Rumana Khan, Valentin Marian, Saira Shah

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