Acute laryngeal dyspnea as first presentation of granulomatosis with polyangiitis.

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.

[1]  N. Rasmussen.  L24. Local treatments of subglottic and tracheal stenoses in granulomatosis with polyangiitis (Wegener's). , 2013, Presse medicale.

[2]  B. Baslund,et al.  Incidence of granulomatosis with polyangiitis (Wegener's) in Greenland and the Faroe Islands: epidemiology of an ANCA-associated vasculitic syndrome in two ethnically distinct populations in the North Atlantic area. , 2013, Clinical and experimental rheumatology.

[3]  P. Macchiarini,et al.  New treatment of subglottic stenosis due to Wegener's granulomatosis , 2012, Acta oto-laryngologica.

[4]  R. Franco.  Intralesional Corticosteroid Injection and Dilatation Provides Effective Management of Subglottic Stenosis in Wegener's Granulomatosis , 2011 .

[5]  W. Gross,et al.  Clinical manifestations and treatment of Wegener's granulomatosis. , 2010, Rheumatic diseases clinics of North America.

[6]  E. Rhee,et al.  Rituximab as maintenance therapy for anti-neutrophil cytoplasmic antibody-associated vasculitis. , 2010, Clinical journal of the American Society of Nephrology : CJASN.

[7]  P. Moss,et al.  Patients with Wegener’s granulomatosis demonstrate a relative deficiency and functional impairment of T‐regulatory cells , 2010, Immunology.

[8]  G. Hoffman,et al.  Surgical interventions and local therapy for Wegener's granulomatosis , 2010, Current opinion in rheumatology.

[9]  W. Lukas,et al.  Co-trimoxazole and prevention of relapses of PR3-ANCA positive vasculitis with pulmonary involvement , 2009, European journal of medical research.

[10]  E. Pallisa,et al.  Clinical features and therapeutic management of subglottic stenosis in patients with Wegener’s granulomatosis , 2008, Lupus.

[11]  B. Thiers.  ANCA Are Detectable in Nearly All Patients with Active Severe Wegener's Granulomatosis , 2008 .

[12]  V. Lund,et al.  Patterns of presentation and diagnosis of patients with Wegener's granulomatosis: ENT aspects , 2006, The Journal of Laryngology & Otology.

[13]  R. Hogg,et al.  Wegener’s granulomatosis and subglottic stenosis: management of the airway , 2004, The Journal of Laryngology & Otology.

[14]  C. Hallahan,et al.  Clinical features and therapeutic management of subglottic stenosis in patients with Wegener's granulomatosis. , 1996, Arthritis and rheumatism.

[15]  B. Haynes,et al.  Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. , 1983, Annals of internal medicine.