SESSION TITLE: Pulmonary Manifestations of Systemic Disease Student/Resident Case Report Posters II
SESSION TYPE: Student/Resident Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: We present a case of bronchiolitis obliterans-organizing pneumonia (BOOP)-like variant of granulomatosis with polyangiitis (GPA), a rare diagnosis requiring careful histopathologic review and clinical judgement.
CASE PRESENTATION: A 77 year old female with history of beta thalassemia minor was transferred from an outside hospital for tertiary workup of nasal stuffiness for 7 months, photophobia for 1 month. Of late she was experiencing fever, chills, fatigue, shortness of breath and hematuria. Labwork at presentation is listed in the table. CT thorax showed multiple spiculated nodules bilaterally, largest being 2.2 x 4.1 cm in the left upper lobe (see image). Ultrasound guided lung biopsy of the right lower lobe nodule showed fibroblasts and fibrinous exudates infiltrated by fibroblasts, giving it an appearance of organizing pneumonia. Capillaritis, multinucleated giant cells, necrotizing vasculitis, and hemosiderin-laden macrophages indicative of previous hemorrhage were also seen. Given the predominant organizing pneumonia-like pattern, this may represent a BOOP-like variant of GPA, which typically lacks the large areas of geographic necrotizing granulomatous inflammation. She was pulse dosed with steroids. In the following eight months after the diagnosis was established, she received six cycles of cyclophosphamide and is currently on weekly rituximab with excellent clinical and radiographic response. She now requires hemodialysis due to rapid renal failure secondary to GPA.
DISCUSSION: Very limited literature exists on the BOOP-like variant of GPA. There is only one clinicopathologic study by Uner et al from our institute in 1996 (1). They did not find any clinical features, presenting symptoms or laboratory abnormalities that distinguish BOOP-like variant of GPA from classic GPA. The authors concluded that the importance of BOOP-like variant of GPA is not in defining a new entity but in emphasizing a histologic variant that needs to be recognized. Another plausible theory to support the rarety of this entity is that the presence of BOOP may reflect a sampling problem, especially if the periphery of the nodule was biopsied instead of the center of the lesion. There has been one report on a possible association between this variant and orally administered mesalazine (2).
CONCLUSIONS: BOOP-like variant of GPA is rare. This variant should be part of the differentials in patients who have histological manifestations consistent with BOOP (now known as cryptogenic organizing pneumonia) including evidence of capillaritis, hemosiderin-laden macrophages in the presence of necrotizing vasculitis.
Reference #1: Uner AH, Rozum-Slota B, Katzenstein AL. Bronchiolitis obliterans organizing pneumonia (BOOP)-like variant of Wegener’s granulomatosis. A clinicopathologic study of 16 cases. Am J Surg Pathol 1996; 20:794 - 801.
Reference #2: Abrari A, Bakshi V. The BOOP trojan horse in Wegener's granulomatosis: a reminder to pathologists and clinicians. BMJ Case Rep 2011;2011:bcr1120103483
DISCLOSURE: The following authors have nothing to disclose: Shalin Kothari, Wajihuddin Syed, Matthew Hess
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