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Spontaneously Regressed Granulomatosis With Polyangiitis: A Case Report

Ota, H. 2021Other/Unknown

Ota, H., Sato, C., Igarashi, A., Inoue, S., Masuda, S., Ishizu, A., & Watanabe, M. (2021). Spontaneously regressed granulomatosis with polyangiitis: A case report. Respiratory investigation, 59(3), 372–376. https://doi.org/10.1016/j.resinv.2020.12.002

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Abstract

A 71-year-old woman presented with chest pain, cough, and back pain. A chest roentgenogram showed multiple nodular shadows in both lungs. She was diagnosed with granulomatosis with polyangiitis (GPA). The multiple nodular shadows in both lungs regressed spontaneously in a few months. There are few reports of spontaneous regression of GPA, and the underlying mechanism is unclear. Neutrophil extracellular traps (NETs) have been recently shown to be involved in GPA. NETs may also be related to the natural regression of GPA.

Case Details

Disease Location

Lung

Personal Characteristics

71-year-old woman

Clinical Characteristics

Chief complaints of cough, chest pain, and back pain. A chest roentgenogram showed multiple nodular shadows in both lungs. Despite oral antibiotic therapy (cefcapene pivoxil hydrochloride hydrate, amoxicillin/clavulanate), imaging findings showed no improvement. Chest computed tomography (CT) showed multiple subpleural nodular shadows in both lungs. C-reactive protein level was elevated. Urinalysis showed microscopic hematuria. Thoracoscopic partial right lung resection was performed. Histopathological results showed infiltration of inflammatory cells, primarily plasma cells. Subsequent re-analysis of the lung biopsy specimens showed granulomatous vasculitis with necrosis. The organic symptoms of the lungs and kidneys and histological findings of necrotizing granulomatous vasculitis were consistent with granulomatosis with polyangiitis. Neutrophil extracellular traps (nets) were detected in the basophilic areas with cell lysis within the necrotic foci, whereas nets were absent in the lace-like sparse area without cell lysis. Nets in the basophilic area of cell lysis were susceptible to dnase i digestion

Remission Characteristics

A few nodular shadows showed regression at the time of lung biopsy. During subsequent outpatient follow-up, the multiple nodular shadows appeared to regress further.

Treatment & Mechanisms

Proposed Remission Mechanisms

Presumably had nets highly susceptible to dnase i digestion within the pulmonary necrotizing granulomas, and some of these nets had already been digested at the time of biopsy

Clinical Treatment

Biopsy

Non-Clinical Treatment

None reported