GRANULOMATOSIS WITH POLYANGITIS PRESENTING AS A LARGE BLEEDING PLEURAL BASED MASS

Mili Shah, PGY 3/MD1, Vikrant Tambe, Assistant Clinical Instructor 2, Bashar Sharma, Jil Yeager, 1SUNY Upstate Medical University, Syracuse, NY; 2Syracuse, NY

Meeting: Hospital Medicine 2019, March 24-27, National Harbor, Md.

Abstract number: 972

Categories: Adult, Clinical Vignettes, Hospital Medicine 2019

Keywords: , , ,

Case Presentation: 69-year-old man presented to his PCP with complaints of acute onset abdominal pain. After admission he was initially managed conservatively but since the pain did not resolve a CT abdomen was obtained and this revealed incidental finding of multiple lung nodules. This was followed up with a CT Thorax which revealed a dominant 3.9 x 1.7 cm right lower lobe (RLL) pleural based mass with multiple smaller nodules bilaterally. Initial suspicion was of a metastatic malignancy and the patient underwent an EBUS with biopsy of the RLL mass. The biopsy showed small fragments of benign bronchial wall with non-specific fibrosis and mild eosinophilic infiltrate. He was discharged with a plan to keep a close Pulmonary follow up and CT scan. About a week post EBUS he presented to the ER with complaints of hemoptysis of about 300-400 cc of fresh blood. He was hypoxic and hypotensive requiring of pressor support to maintain his blood pressure, he was eventually intubated and admitted to the MICU. D-dimer was elevated and hence a CTA was done and did not show any evidence of pulmonary embolism but showed increase in the lesions seen in the previous CT scan. Repeat EBUS revealed a large clot in his RLL with blood slowly oozing around it and later underwent IR guided embolization of a small pseudoaneurysm in the RLL mass. On further review it was found that the patient had a recurrent history of sinus infections, so an autoimmune workup was initiated for concern of vasculitis. The patient was found to have a positive c-ANCA titer of 1:360, elevated ESR of 66, and elevated CRP of 361. ANA and ANA specificity are negative and complements C3/C4 levels were normal. He was diagnosed to have ANCA associated vasculitis, specifically Granulomatosis with polyangiitis and started on high dose steroids by rheumatology with subsequent improvement. He later was weaned off the ventilator and extubated. After discharge he received Rituxan as an outpatient with further improvement in his lung nodules.

Discussion: The lung is the most commonly involved organ in primary vasculitis especially in Wegner’s granulomatosis. Pulmonary involvement per say may give rise to solitary or multiple nodules which often cavitate and can be surrounded by thick walls of inflammatory tissue. Pleural effusions and inflammatory pleural pseudotumors can also be sometimes seen. In a cohort of patients with Wegener’s granulomatosis 34% of patient had asymptomatic pulmonary nodule.

Conclusions: In cases with atypical presentation, even in the absence of other clinical sign and symptoms, ANCA determination is recommended in patient with multiple pulmonary lesions, in order to employ appropriate treatment and avoid complications.

To cite this abstract:

Shah, M; Tambe, V; Sharma, B; Yeager, J. GRANULOMATOSIS WITH POLYANGITIS PRESENTING AS A LARGE BLEEDING PLEURAL BASED MASS. Abstract published at Hospital Medicine 2019, March 24-27, National Harbor, Md. Abstract 972. https://www.shmabstracts.com/abstract/granulomatosis-with-polyangitis-presenting-as-a-large-bleeding-pleural-based-mass/. Accessed December 6, 2019.

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