open access

Vol 83, No 3 (2015)
CASE REPORTS
Submitted: 2015-04-30
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Acute laryngeal dyspnea as first presentation of granulomatosis with polyangiitis

Anna Maria Pajor, Sylwia Kwiatkowska, Jadwiga Kroczyńska-Bednarek, Wojciech J. Piotrowski
DOI: 10.5603/PiAP.2015.0035
·
Pneumonol Alergol Pol 2015;83(3):216-219.

open access

Vol 83, No 3 (2015)
CASE REPORTS
Submitted: 2015-04-30

Abstract

Granulomatosis with polyangiitis (GPA) is a multi-organ disease which mostly affects lungs, kidney, and head and neck region. We report a rare case of acute laryngeal dyspnea and rapidly progressive pulmonary changes as first manifestations of disease. A 53 year-old woman presented with symptoms of two-week dyspnea, which aggravated rapidly in the preceding hours. Laryngological examination revealed subglottic infiltrations and vocal fold oedema which required urgent tracheotomy. During few days she developed gingival ulcerations and pulmonary infiltration with negative serum c-ANCA titers. The histopathological examination of subglottic and gingival biopsies and the clinical picture established the diagnosis of GPA. She was treated with prednisone and cyclophosphamide with recovery; however, during over 3 years of follow-up, pulmonary symptoms relapsed and subglottic stenosis persisted. The difficulties in diagnosis and treatment in this unusual presentation of GPA are outlined with conclusion that in patients with subglottic infiltration, which develops rapidly, even when this is a sole presentation of the disease, and when c-ANCA are negative, GPA should always be considered.

Abstract

Granulomatosis with polyangiitis (GPA) is a multi-organ disease which mostly affects lungs, kidney, and head and neck region. We report a rare case of acute laryngeal dyspnea and rapidly progressive pulmonary changes as first manifestations of disease. A 53 year-old woman presented with symptoms of two-week dyspnea, which aggravated rapidly in the preceding hours. Laryngological examination revealed subglottic infiltrations and vocal fold oedema which required urgent tracheotomy. During few days she developed gingival ulcerations and pulmonary infiltration with negative serum c-ANCA titers. The histopathological examination of subglottic and gingival biopsies and the clinical picture established the diagnosis of GPA. She was treated with prednisone and cyclophosphamide with recovery; however, during over 3 years of follow-up, pulmonary symptoms relapsed and subglottic stenosis persisted. The difficulties in diagnosis and treatment in this unusual presentation of GPA are outlined with conclusion that in patients with subglottic infiltration, which develops rapidly, even when this is a sole presentation of the disease, and when c-ANCA are negative, GPA should always be considered.

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Keywords

granulomatosis with polyangiitis, subglottic stenosis, pulmonary symptoms

About this article
Title

Acute laryngeal dyspnea as first presentation of granulomatosis with polyangiitis

Journal

Advances in Respiratory Medicine

Issue

Vol 83, No 3 (2015)

Pages

216-219

DOI

10.5603/PiAP.2015.0035

Bibliographic record

Pneumonol Alergol Pol 2015;83(3):216-219.

Keywords

granulomatosis with polyangiitis
subglottic stenosis
pulmonary symptoms

Authors

Anna Maria Pajor
Sylwia Kwiatkowska
Jadwiga Kroczyńska-Bednarek
Wojciech J. Piotrowski

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