Table 1.

Clinical characteristics of 34 patients with GPA-related SGS.

VariableNo. Patients
Female23
Male11
Age, yrs18–78
Median age, yrs46
  SGS diagnosis
  SGS as the only symptom of GPA2
  SGS diagnosed in clinical remission of GPA15
  SGS + activity of GPA in other organs17
BVAS, organ involvement34
  015
  1: sinus9
  2: ENT, sinus1
  3: sinus, kidney3
  4: sinus, ocular, kidney4
  5: sinus, skin, lung1
  9: sinus, ocular, lung, kidney1
Trachea biopsy26/34
GPA confirmation16/26
Noncharacteristic pattern10/26
ANCA present24/34
Clinical symptoms
  Dyspnea34
  Cough11/34
  Hoarseness10/34
Pulmonary function tests
  Desaturation in walking test4/26
  Hypoxemia, < 70 mmHg7/34
  Obstruction of spirometry, FEV1/FVC < 0.717/26
Treatment type
  Only IDIT13
  IDIT + IST, activity in other organs17
  IDIT + IST, persistent symptoms of SGS4
  Overall34
  • GPA: granulomatosis with polyangiitis; SGS: subglottic stenosis; BVAS: Birmingham Vasculitis Activity Score; ENT: ear, nose, throat; ANCA: antineutrophil cytoplasmic antibodies; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; IDIT: intratracheal dilation-injection technique; IST: immunosuppressive therapy.