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Table 1 Respiratory manifestations indicating a suspicion of PID in primary care and pulmonology clinics

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

Respiratory Manifestions with suspicion of PID in PC

N (Composition of panel)

Votes in agreement (%)

Degree of agreement

Adult patient

 Recurrent bronchial infections (≥ 2/year), with cough and purulent expectoration

23* (AI, AP)

87.0

Consensus

 Idiopathic bronchiectasis

23* (AI, AP)

100.0

Unanimity

 Recurrent pneumonias**

23* (AI, AP)

100.0

Unanimity

 Chronic bronchial infection

23* (AI, AP)

91.3

Consensus

 Need for prolonged antibiotic treatment for respiratory infections

23* (AI, AP)

95.7

Consensus

 Pediatric patient

 Recurrent pneumonias**

19 (PI, PP)

100.0

Unanimity

 Idiopathic bronchiectasis

19 (PI, PP)

100.0

Unanimity

Respiratory manifestations with suspicion of PID in pulmonolgy unit

N (Composition panel)

Votes in agreement (%)

Degree of agreement

Infections

 Adult and pediatric patients

  Recurrent bronchial infections (≥ 2/year), with cough and purulent expectoration

43 (AI, AP, PI, PP)

86.0

Consensus

  Idiopathic bronchiectasis

43 (AI, AP, PI, PP)

100.0

Unanimity

  Recurrent pneumonias**

43 (AI, AP, PI, PP)

97.7

Consensus

  Chronic bronchial infection

43 (AI, AP, PI, PP)

90.7

Consensus

  Need for prolonged antibiotic treatment for respiratory infections

43 (AI, AP, PI, PP)

93.0

Consensus

  Pulmonary abscess and pneumatocele

43 (AI, AP, PI, PP)

90.7

Consensus

  Infections caused by rare or opportunistic microorganisms

43 (AI, AP, PI, PP)

100.0

Unanimity

 Infants

  Severe infantile bronchiolitis or pneumonia

43 (AI, AP, PI, PP)

93.0

Consensus

 NON-INFECTIOUS

 Adult and pediatric patients

  Granulomatous-lymphocytic interstitial lung disease

43 (AI, AP, PI, PP)

100.0

Unanimity

  Bronchiolitis obliterans

43 (AI, AP, PI, PP)

72.1

Majority

  Lymphoproliferative syndrome

43 (AI, AP, PI, PP)

90.7

Consensus

  Alveolar proteinosis

43 (AI, AP, PI, PP)

86.0

Consensus

  Recurrent serositis

43 (AI, AP, PI, PP)

76.7

Majority

  Thymic disorders: thymoma (adult), thymic aplasia (infant)

43 (AI, AP, PI, PP)

100.0

Unanimity

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