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Paraneoplasic ANCA Vasculitis and Cancer of Unknown Origin FREE TO VIEW

Erick Rendón Ramírez, MD; Jasel Tapia-Orozco, MD; Sergio Sanchez-Salazar, MD; Alexis Herrera-Guerra, MD; Erick Renpenning-Carrasco, MD; Claudia Rivera-Uribe, MD; Roberto Mercado-Longoria, MD; Dania Flores-Quintanilla, MD; Perla Colunga-Pedraza, MD; Hector Ibarra-Sifuentes, MD
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Pulmonary and Critical Care. Hospital Universitario UANL, Monterrey, Nuevo León, Mexico; Internal Medicine, Hospital Universitario UANL, Monterrey, Nuevo León, Mexico

Chest. 2015;148(4_MeetingAbstracts):576A. doi:10.1378/chest.2265732
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SESSION TITLE: Lung Cancer Student/Resident Case Report Posters

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Malignancy-associated vasculitis is a rare condition(0.4-4.2% of all vasculitis cases) with an even less common association between solid tumors and vasculitis. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is an organ and life-threatening chronic inflammatory small-vessel vasculitis with systemic manifestations. Its association with malignancy has seldom been described.

CASE PRESENTATION: A 26-year-old man presented with dyspnea, anasarca and abdominal pain. Pleural and pericardic effusion, multiple bilateral lung nodules, and septal thickening were found on lung CT. Sputum, bronchoalveolar lavage, and pleural effusion studies, cultures and cancer inmunological markers were negative. p-ANCA 1:5120, and myeloperoxidase 145.68 IU were positive. Virus and toxicological studies, were negative. Lung biopsy showed a poorly differentiated signet-ring-cell adenocarcinoma negative for vasculitis with no primary tumor. Renal biopsy revealed rapidly progressive glomerulonephritis negative for neoplasm and immune complexes. Hemodialysis were given, and 1g methylprednisolone and cyclophosphamide for three consecutive days. Paclitaxel and carboplatin were then started. In the year after diagnosis, the patient received 10 cycles of the Gramont regimen with a partial response.

DISCUSSION: AAV and malignancy have been reported in a few case report. Solans-Laqué et al. reported a prevalence of 1.2% related to a solid tumor. Clinical and laboratory findings in paraneoplasic vasculitis are nonspecific. Cutaneous involvement is the most frequent sign (>75%), followed by joint symptoms and kidney involvement. Additional findings include positive antinuclear antibodies (ANA) in 53%, rheumatoid factor, and cryoglobulins. ANCA can be detected in up to 20%, mostly associated with positive ANA. Renal involvement and CT lung findings made us suspect an AAV. Most patients have a complete resolution of vasculitis after therapy for the underlying malignancy. Meanwhile they have been managed with conventional therapy for the vasculitic disorder. The majority of deaths have been associated with the severity of the neoplasm rather than to vasculitic complications.

CONCLUSIONS: We present the first reported case of AAV associated with signet-ring adenocarcinoma related to Cancer of unknown origin. High titers of ANCA not related to positive ANA have not been reported

Reference #1: Mahr A, Heijl C, Le Guenno G, Faurschou M. ANCA-associated vasculitis and malignancy: Current evidence for cause and consequence relationships. Best Practice & Research Clinical Rheumatology. 2013;27(1):45-56

Reference #2: Solans-Laqué R, Bosch-Gil JA, Pérez-Bocanegra C, Selva-O'Callaghan A, Simeón-Aznar CP, Vilardell-Tarres M. Paraneoplastic vasculitis in patients with solid tumors: report of 15 cases. The Journal of Rheumatology. 2008;35(2):294-304

Reference #3: Kurzrock R, Cohen PR, Markowitz A. Clinical manifestations of vasculitis in patients with solid tumors. Arch Intern Med. 1994;154:334-40

DISCLOSURE: The following authors have nothing to disclose: Erick Rendón Ramírez, Dania Flores-Quintanilla, Jasel Tapia-Orozco, Sergio Sanchez-Salazar, Alexis Herrera-Guerra, Erick Renpenning-Carrasco, Claudia Rivera-Uribe, Perla Colunga-Pedraza, Hector Ibarra-Sifuentes, Roberto Mercado-Longoria

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