Table 2

Cumulative manifestations during follow-up

EGPA manifestations during the whole follow-upn=150 (=100%)
Ear–nose–throat (ENT)140 (93.3)
 Paranasal sinusitis115 (76.7)
 Nasal mucosa inflammation87 (58)
 Nasal polyps66 (44)
 Middle ear affection18 (12)
Constitutional symptoms117 (78)
Peripheral nervous system115 (76.7)
 Mononeuritis multiplex84 (56)
 Polyneuropathy
  Sensoric112 (74.7)
  Motoric96 (64)
Lungs (without asthma bronchiale)92 (61.3)
 Migratory infiltrates (x-ray)87 (58)
 Eosinophilc infiltrates (BAL)47 (31.3)
 Alveolar haemorrhage (BAL)15 (10)
 Heamoptysis9 (6)
 Nodules (x-ray/CT scan)10 (6.7)
 Pleural effusion11 (7.3)
Joints and muscles92 (61.3)
 Arthritis/arthralgia77 (51.3)
 Myalgia49 (32.7)
 Myositis20 (13.3)
Skin74 (49.3)
 Purpura52 (34.7)
 Erythema nodosum26 (17.3)
 Livedo4 (2.7)
Heart70 (46.7)
 Pericardial effusion43 (28.7)
 Arrhythmia34 (22.7)
 Heart failure31 (20.7)
 Angina pectoris11 (7.3)
 Coronariitis (angiography)4 (2.7)
Gastrointestinal43 (28.7)
Kidney28 (18.7)
 Proteinuria >1 g/day5 (3.3)
 Glomerulonephritis8 (5.3)
Central nervous system23 (15.3)
 Apoplexia/vasculitis on angiography13 (8.7)
 Central affection of cranial nerves10 (6.7)
 Eosinophilia in CSF3 (2)
 Psychosis2 (1.3)
Eyes18 (12)
 Episcleritis13 (8.7)
  • Only manifestations related to eosinophilic granulomatosis and polyangiitis (EGPA) were counted, that is, if the causative relationship was proven or highly likely due to exclusion of other possible causes. As some diagnostic procedures were performed in every patient (eg, ENT examination) but others only in case of symptoms (coronarangiography), some manifestations might be underestimated.

  • BAL, Broncho-Alveolar-Lavage; CSF, Cerebro-Spinal Fluid.