RATIONALE: Bronchial involvement alone is a rare initial manifestation of granulomatosis with polyangiitis (GPA). Herein, we report a case of refractory GPA with obstructive pneumonia caused by bronchial involvement. PATIENT CONCERNS: A 65-year-old man complained of a 2-week cough and fever. DIAGNOSES: Considering the presence of opacities and multiple consolidations in both lungs due to obstruction or stenosis on the bronchus, which did not respond to antibiotics, and proteinase-3-antineutrophil cytoplasmic autoantibody positivity, he was diagnosed with GPA. Positron emission tomography- computed tomography scan revealed no abnormal findings in the upper respiratory tract. INTERVENTIONS: He was treated with prednisolone (PSL, 50 mg/d) and intravenous cyclophosphamide. OUTCOMES: His general and respiratory symptoms improved. However, 8 weeks after PSL treatment at 20 mg/d, he developed a relapse of vasculitis along with sinusitis and hypertrophic pachymeningitis. Hence, PSL treatment was resumed to 50 mg/d, and weekly administration of rituximab was initiated. Consequently, the symptoms gradually mitigated. LESSONS: GPA with bronchial involvement is often intractable and requires careful follow-up, which should include upper respiratory tract and hypertrophic pachymeningitis assessment.
RATIONALE: Bronchial involvement alone is a rare initial manifestation of granulomatosis with polyangiitis (GPA). Herein, we report a case of refractory GPA with obstructive pneumonia caused by bronchial involvement. PATIENT CONCERNS: A 65-year-old man complained of a 2-week cough and fever. DIAGNOSES: Considering the presence of opacities and multiple consolidations in both lungs due to obstruction or stenosis on the bronchus, which did not respond to antibiotics, and proteinase-3-antineutrophil cytoplasmic autoantibody positivity, he was diagnosed with GPA. Positron emission tomography- computed tomography scan revealed no abnormal findings in the upper respiratory tract. INTERVENTIONS: He was treated with prednisolone (PSL, 50 mg/d) and intravenous cyclophosphamide. OUTCOMES: His general and respiratory symptoms improved. However, 8 weeks after PSL treatment at 20 mg/d, he developed a relapse of vasculitis along with sinusitis and hypertrophic pachymeningitis. Hence, PSL treatment was resumed to 50 mg/d, and weekly administration of rituximab was initiated. Consequently, the symptoms gradually mitigated. LESSONS: GPA with bronchial involvement is often intractable and requires careful follow-up, which should include upper respiratory tract and hypertrophic pachymeningitis assessment.
Authors: C A Langford; M C Sneller; C W Hallahan; G S Hoffman; W A Kammerer; C Talar-Williams; A S Fauci; R S Lebovics Journal: Arthritis Rheum Date: 1996-10
Authors: G S Hoffman; G S Kerr; R Y Leavitt; C W Hallahan; R S Lebovics; W D Travis; M Rottem; A S Fauci Journal: Ann Intern Med Date: 1992-03-15 Impact factor: 25.391
Authors: J C Jennette; R J Falk; P A Bacon; N Basu; M C Cid; F Ferrario; L F Flores-Suarez; W L Gross; L Guillevin; E C Hagen; G S Hoffman; D R Jayne; C G M Kallenberg; P Lamprecht; C A Langford; R A Luqmani; A D Mahr; E L Matteson; P A Merkel; S Ozen; C D Pusey; N Rasmussen; A J Rees; D G I Scott; U Specks; J H Stone; K Takahashi; R A Watts Journal: Arthritis Rheum Date: 2013-01
Authors: Charlotte Girard; Pierre Charles; Benjamin Terrier; Guillaume Bussonne; Pascal Cohen; Christian Pagnoux; Vincent Cottin; Jean-François Cordier; Loïc Guillevin Journal: Medicine (Baltimore) Date: 2015-08 Impact factor: 1.817