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Table 3 Most common extrapulmonary manifestations of PID (adapted from JC Aldave, JFM) [27]

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

MOST COMMON EXTRAPULMONARY MANIFESTATIONS OF PID

N (Composition of panel)

Votes in agreement (%)

Degree of agreement

 Recurrent or complicated sinusitis

43 (AI, AP, PI, PP)

100.0

Unanimity

 Recurrent or complicated otitis

43 (AI, AP, PI, PP)

93.0

Consensus

 Other extrapulmonary infections requiring admission

43 (AI, AP, PI, PP)

88.4

Consensus

 Ectodermal dysplasia

43 (AI, AP, PI, PP)

72.1

Majority

 Chronic diarrhea or malabsorption

43 (AI, AP, PI, PP)

93.0

Consensus

 Difficult-to-treat giardiasis

43 (AI, AP, PI, PP)

93.0

Consensus

 Autoimmune cytopenias or other autoimmunity

43 (AI, AP, PI, PP)

93.0

Consensus

 Lymphadenopathies and hepatosplenomegaly

43 (AI, AP, PI, PP)

97.7

Consensus

 Related cancers, especially associated with viruses

43 (AI, AP, PI, PP)

83.7

Consensus

 Family history of ID or consistent manifestations

43 (AI, AP, PI, PP)

88.4

Consensus

 Post-vaccinal infections

43 (AI, AP, PI, PP)

79.1

Majority

  1. AI: adult immunologists; AP: adult pulmonologists; ID: immunodeficiency; PI: pediatric immunologists; PID: primary immunodeficiency disease; PP: pediatric pulmonologists