A 52-year-old female with no PMH or FH of autoimmune diseases and no substance use history presented with worsening dyspnea, dry cough and brownish nasal discharge over several weeks. On physical examination, vital signs were T 97.3oF, BP 111/45 mmHg, HR 56/min, RR 13/min, SpO2 98%. Physical findings were notable for tenderness over the nasal bridge and lower anterior chest wall, lungs clear to auscultation and no palpable neck lymphadenopathy. Sinus CT was negative for sinonasal pathology. CXR revealed multiple suspicious pulmonary nodules with patchy opacities. Chest CT additionally revealed ~5cm cavitary necrotic mass in the RLL. She underwent robotic thoracoscopic wedge resection of the mass with significant symptomatic relief, but biopsy was inconclusive. Three months later, repeat chest CT revealed new ~5cm necrotic mass in the RLL and LLL, several ~1.5cm nodules in the RUL, LUL, RLL and LLL, and ~1cm lymphadenopathies in the mediastinum, right hilum, right paratracheal and supraclavicular areas. She underwent repeat thoracoscopic wedge resection of only the LLL mass. Biopsy revealed microabscesses, geographic bluish necrosis with multinucleated giant cells and necrotizing vasculitis all consistent with granulomatosis with polyangiitis. Workup also revealed elevated ESR and CRP, but negative cANCA and pANCA, normal CBC, BMP, LFT and urinalysis. Initial therapy consisted of methylprednisolone 1000mg daily for 3 days, followed by rituximab 375mg/m2 weekly for 4 weeks, then prednisone 60mg daily with tapering over the next several months, followed by methotrexate 15mg weekly for the next several months. She responded well to therapy, and her subsequent ESR and CRP measurements remained relatively low over time.
Granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis) is characterized by small to medium vessel vasculitis most commonly affecting the ENT with an estimated frequency of 90%, but also affects the lungs, kidneys and other organs. ANCA is positive in over 90% of patients with active disease involving the lungs and/or kidneys. Chest imaging commonly identifies multiple pulmonary nodules with patchy opacities, but intrathoracic and extrathoracic lymphadenopathies are quite uncommon. This case illustrates the possibility of pseudomalignant lung granulomas with intrathoracic and extrathoracic lymphadenopathies in ANCA-negative GPA.
Findings of multiple lung nodules with lymphadenopathies are oftentimes associated with true malignancies. However, here the CXR and CT findings of malignant-appearing lung granulomas and lymphadenopathies were not true malignancies. Furthermore, this case underscores the importance of full diagnostic workup including tissue biopsy in cases of malignant-appearing pulmonary lesions of unclear etiology to avoid a premature presumptive diagnosis of lung cancer. Overall, the frequency and degree of lymphadenopathy in ANCA-negative GPA with lung involvement is unclear and warrants further investigation.
To cite this abstract:Ukadike, KC; Andron, RI; Williams, KD . PSEUDOMALIGNANT GRANULOMAS IN ANCA-NEGATIVE GRANULOMATOSIS WITH POLYANGIITIS. Abstract published at Hospital Medicine 2017, May 1-4, 2017; Las Vegas, Nev. Abstract 762. Journal of Hospital Medicine. 2017; 12 (suppl 2). https://www.shmabstracts.com/abstract/pseudomalignant-granulomas-in-anca-negative-granulomatosis-with-polyangiitis/. Accessed September 15, 2019.