10 Warning Signs of Primary and Secondary Immunodeficiencies
The Jeffrey Modell Foundation (JMF) proposed 10 warning signs of primary immunodeficiencies (PID). The study presented below examined whether they are applicable to secondary immunodeficiencies (SID) and whether there are any additional signs that could help in diagnosing SID.
Study Methodology and Population
A total of 162 patients with immunodeficiency — primary (27.1%; n = 98; 49% girls; average age 8.2 years) or secondary (17.7%; n = 64; 25% girls; average age 5.7 years) — and 200 healthy patients (55.2%; 49% girls; average age 5.5 years) were included in a prospective study from June to December 2020. Their parents filled out a questionnaire regarding family and personal medical history and 10 warning signs of PID according to JMF, where each positive answer = 1 point to the total score.
10 Warning Signs of PID according to JMF:
- ≥ 2 months of oral antibiotic treatment (ATB) with insufficient effect
- recurrent skin or organ abscesses
- failure to thrive in childhood (weight gain or normal growth)
- persistent thrush in the oral cavity or recurring fungal skin infections
- need for intravenous administration of ATB
- ≥ 2 deep-seated infections including septicemia
- family history of PID
- ≥ 4 ear infections in one year
- ≥ 2 serious sinus infections in one year
- ≥ 2 pneumonias in one year
In patients with PID, the most common type was a humoral immunodeficiency. In 50% of SID cases, the cause was a chromosomal anomaly; in 10% malnutrition; in 5% uremia; and in 5% metabolic disease. 30% of patients were taking antiepileptic drugs.
Findings
The average score in the questionnaire was significantly higher in the PID (3.36 ± 1.65) and SID (3.72 ± 1.12) groups than in the healthy control group (0.34 ± 0.61; p < 0.05). A total of 2 PID patients without the mentioned warning signs were examined for chronic diarrhea. In the same group (not in the SID group or control group), the warning signs of consanguineous parents and the occurrence of tuberculosis in the family were statistically significant.
The PID group experienced higher rates of these symptoms compared to the SID and control groups: ≥ 4 ear infections within 1 year, ≥ 2 serious sinus infections within 1 year, and family history of PID (p < 0.001).
Both the PID and SID groups showed higher incidence of the following symptoms compared to the control group: ≥ 2 months of ineffective oral antibiotic treatment, recurrent skin or organ abscesses, persistent oral thrush or fungal skin infections, and ≥ 2 deep-seated infections including septicemia (p < 0.001).
In SID patients, significantly more frequent signs were failure to thrive in childhood, need for intravenous ATB administration, and ≥ 2 pneumonias in one year.
Parental consanguinity was 7.27 times more frequent in the PID group (95% confidence interval [CI] 3.87–13.64) and 3.37 times more frequent in the SID group (95% CI 1.60–7.09) compared to the control group.
Conclusion
Recognizing warning signs is key for the early diagnosis of primary immunodeficiencies. According to the cited study, these warning signs can also be used for the early diagnosis of secondary immunodeficiencies (SID). Additionally, parental consanguinity, chronic diarrhea, and the occurrence of tuberculosis in the family can be considered additional warning signs of PID.
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Source: Eldeniz F. C., Gul Y., Yorulmaz A. et al. Evaluation of the 10 warning signs in primary and secondary immunodeficient patients. Front Immunol 2022; 13: 900055, doi: 10.3389/fimmu.2022.900055.
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