2
Key Points
➤ Since the publication of the previous practice parameter in 2015,
many advances in the care and diagnosis of patients with primary
immune deficiency disorders (now also known as inborn errors of
immunity [IEI]) have occurred.
➤ This practice parameter will discuss and highlight some of these
changes, including the use of genetic testing in the diagnosis and
in guiding treatment of IEI, newborn screening for severe T-cell
lymphopenia, and the use of targeted therapies and precision medicine,
based on the identification of the immunopathology of the disorder.
GRADE Strength of Recommendation Classifications and
Interpretation
Strength of
Recommendation
Description Implications
S = Strong Recommended • Desirable effects outweigh undesirable effects.
• Most patients would want this course of
action.
• Most clinicians would implement these
recommendations in patient care.
• Most policy makers would agree to follow
these recommendations.
C = Conditional
Suggested • Most patients would want this course of
action, but many would not.
• Most clinicians would consider this course of
action but would review the case to see if other
options are also appropriate and involve the
patient in shared decision making.
• Policy makers will likely require additional
information from many stakeholders.
GRADE Classification of Guideline Recommendations
Certainty of Evidence Interpretation
H = High Further research is very unlikely to change the confidence in
the recommendation.
M = Moderate Further research is likely to affect the confidence of the
balance of effects and may change the recommendation.
L = Low Further research is likely to change the recommendation.
VL = Very low e estimate of the effect is very uncertain.
