60
Classification of PPID
Disclaimer
is Guideline attempts to define principles of practice that should produce high-quality patient
care. It is applicable to specialists, primary care, and providers at all levels. is Guideline
should not be considered exclusive of other methods of care reasonably directed at obtaining the
same results. e ultimate judgment concerning the propriety of any course of conduct must be
made by the clinician aer consideration of each individual patient situation.
Neither IGC, the medical associations, nor the authors endorse any product or service associated
with the distributor of this clinical reference tool.
Source
Bonilla FA, Khan DA, Ballas ZK, et al. Practice parameter for the diagnosis and
management of primary immunodeficiency. J Allerg y Clin Immunol. 2015;136(5):
1186-205.e1-78.
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AAAAIPRI16023
Immunodeficiency associated with autoantibodies
Acquired angioedema
Anti–C1 inhibitor
Neutropenia/Felty syndrome
Anti–G-CSF
Cryptococcal meningitis/PAP
Anti–GM-CSF
Disseminated varicella-zoster/APECED
Anti–IFN-α/β
Disseminated infections (virus, bacteria, fungi)
Anti–IFN-γ
Recurrent bacterial skin infections/sepsis
Anti–IL-6
Disseminated Burkholderia gladioli infection
Anti–IL-12p70
CMCC/APECED
Anti–IL-17, anti–IL-22
a
e classification is based on the format used by the WHO/IUIS (Al-Herz W, Bousfiha A,
Casanova JL, et al. Front Immunol. 2014;5:162.) e authors have attempted to use the Human
Genome Organization name for each gene current at the time of publication of this document. e
reader should be aware that this nomenclature is fluid, and some names might have changed.
Table 11. Classification of Primary Immunodeficiencies
a
(cont'd)
Defect or disease(s) Gene(s)
