Skip to main content

Table 2 Common respiratory manifestations by PID type

From: Primary immunodeficiency diseases in lung disease: warning signs, diagnosis and management

Common respiratory manifestations by PID type

N (Composition of panel)

Votes in agreement (%)

Degree of agreement

Predominantly T cell deficiency (combined and PID-related syndromes)

 Recurrent bronchitis

43 (AI, AP, PI, PP)

72.1

Majority

 Idiopathic bronchiectasis

43 (AI, AP, PI, PP)

76.7

Majority

 Recurrent pneumonias*

43 (AI, AP, PI, PP)

88.4

Consensus

 Repeated pneumonia in child

43 (AI, AP, PI, PP)

88.4

Consensus

 Chronic bronchial infection

43 (AI, AP, PI, PP)

79.1

Majority

 Prolonged antibiotic treatment with poor response

43 (AI, AP, PI, PP)

86.0

Consensus

 Abscess and pneumatocele

43 (AI, AP, PI, PP)

55.8

Discrepancy

 Infections caused by rare microorganisms

43 (AI, AP, PI, PP)

100.0

Unanimity

 Pneumonitis or bronchitis with hospitalization in infants

43 (AI, AP, PI, PP)

86.0

Consensus

 Interstitial lung disease

43 (AI, AP, PI, PP)

93.0

Consensus

 Bronchiolitis obliterans

43 (AI, AP, PI, PP)

67.4

Majority

 Alveolar proteinosis

43 (AI, AP, PI, PP)

76.7

Majority

 Absent thymus or aplasia

43 (AI, AP, PI, PP)

95.3

Consensus

Antibody deficiencies

 Recurrent bronchitis

43 (AI, AP, PI, PP)

90.7

Consensus

 Idiopathic bronchiectasis

43 (AI, AP, PI, PP)

97.7

Consensus

 Recurrent pneumonias*

43 (AI, AP, PI, PP)

97.7

Consensus

 Repeated pneumonia in child

43 (AI, AP, PI, PP)

97.7

Consensus

 Chronic bronchial infection

43 (AI, AP, PI, PP)

90.7

Consensus

 Prolonged antibiotic treatment with poor response

43 (AI, AP, PI, PP)

97.7

Consensus

 Pneumonia due to encapsulated bacteria

43 (AI, AP, PI, PP)

97.7

Consensus

 Interstitial lung disease

43 (AI, AP, PI, PP)

79.1

Majority

 Bronchiolitis obliterans

43 (AI, AP, PI, PP)

60.5

Discrepancy

 Pulmonary lymphoma

43 (AI, AP, PI, PP)

69.8

Majority

 Thymoma

43 (AI, AP, PI, PP)

88.4

Consensus

Immune deregulation

 Recurrent bronchitis

43 (AI, AP, PI, PP)

69.8

Majority

 Idiopathic bronchiectasis

43 (AI, AP, PI, PP)

74.4

Majority

 Chronic bronchial infection

43 (AI, AP, PI, PP)

65.1

Majority

 Pneumonia due to encapsulated bacteria

43 (AI, AP, PI, PP)

67.4

Majority

 Interstitial lung disease

43 (AI, AP, PI, PP)

81.4

Consensus

Phagocyte disorders

 Recurrent pneumonias*

43 (AI, AP, PI, PP)

90.7

Consensus

 Prolonged antibiotic treatment with poor response

43 (AI, AP, PI, PP)

88.4

Consensus

 Abscess and pneumatocele

43 (AI, AP, PI, PP)

97.7

Consensus

 Infections caused by rare microorganisms

43 (AI, AP, PI, PP)

97.7

Consensus

 Alveolar proteinosis

43 (AI, AP, PI, PP)

69.8

Majority

Innate immunity disorder

 Recurrent pneumonias*

43 (AI, AP, PI, PP)

97.7

Consensus

 Pneumonia due to encapsulated bacteria

43 (AI, AP, PI, PP)

95.3

Consensus

 Abscess and pneumatocele

43 (AI, AP, PI, PP)

83.7

Consensus

 Infections caused by rare microorganisms

43 (AI, AP, PI, PP)

90.7

Consensus

Complement deficiency

 Recurrent bronchitis

43 (AI, AP, PI, PP)

65.1

Majority

 Idiopathic bronchiectasis

43 (AI, AP, PI, PP)

72.1

Majority

 Recurrent pneumonias*

43 (AI, AP, PI, PP)

83.7

Consensus

 Repeated pneumonia in child

43 (AI, AP, PI, PP)

83.7

Consensus

 Chronic bronchial infection

43 (AI, AP, PI, PP)

67.4

Majority

Autoinflammatory disease

 Recurrent serositis

43 (AI, AP, PI, PP)

100.0

Unanimity

  1. AI: adult immunologists; AP: adult pulmonologists; PI: pediatric immunologists; PID: primary immunodeficiency disease; PP: pediatric pulmonologists
  2. * One pneumonia per year for more than one year