Unusual Cause of Stridor in an Adult Man, Selective Immunoglobulin A Deficiency

The common causes of stridor in adults are abscesses or swelling of the upper airway, tumors, paralysis, or malfunction of vocal cords. Tracheitis due to immunoglobulin deficiency may be a rare explanation for stridor in adults, although occasionally reported in children. We report an adult man having stridor secondary to isolated immunoglobulin A deficiency. We did an in-depth review of the literature to seek out no reported cases of stridor thanks to immunoglobulin deficiency in adults. This case underlines the very fact that a standard symptom like stridor rarely occurs thanks to uncommon causes. This case is exclusive because it reports the presence of stridor associated with isolated immunoglobulin A deficiency in an adult patient. The explanation for the stridor was an intraluminal narrowing of the upper part of the extrathoracic trachea.

Selective immunoglobulin A deficiency(SIgAD)primarily leads to recurrent infections and autoimmune diseases:A retrospective study of Chinese patients in the past 40 years

Selective immunoglobulin A deficiency(SIgAD)is considered to be the most common human primary immune-deficiency disease in the world.However,the incidence in China is obviously lower than Caucasian races.The definition of SIgAD has changed over time with the progress of people’s understanding.The scientific community did not reach a consensus on the definition until 1999.As a result,many previously reported cases need to be excluded under the current definition.SIgAD can lead to several spectra of diseases including infections and autoimmune diseases.We retrospectively summarized the SIgAD patients in Peking Union Medical College Hospital(PUMCH),and summarized the Chinese SIgAD reported in China and abroad in past 40 years.Fourty three SIgAD patients were confirmed in the study,in which 9 were healthy without clinical symptoms.Of the 34 patients with clinical symptoms,recurrent infections were found in 29(85.3%)patients;13(38.2%)patients were with autoimmune diseases;6(17.6%)cases had allergic symptoms;3 patients(8.8%)were with tumors,only one case(2.9%)had a family history.Compared with other countries,sIgAD patients in China showed similar symptoms,but the rate of recurrent infections and autoimmune diseases were higher than some other countries;most of the allergic symptoms are drug allergy,different with the allergic sequelae reported in other countries,such as asthma,rhinitis,food allergy and atopic dermatitis;and it is rare to have family history in Chinese patients.We also figured out that more female SIgAD patients tend to have more autoimmune diseases than men(P=0.039).

Prevalence of selective immunoglobulin A deficiency in Greek children and adolescents with type 1 diabetes

Background:The association of selective immunoglobulin A(IgA)deficiency with type 1 diabetes(T1D)remains unclear.This study was to evaluate serum IgA concentrations in Greek children and adolescents with T1D.Methods:In two hundred individuals with T1D,serum IgA concentrations were quantitatively determined using nephelometry.Results:Immunoglobulin A deficiency was detected in 6(3.0%)of 200 patients who were subjected to immunological evaluation.Recurrent infections were not recorded,but human papilloma virus infection was clinically suspected and confirmed by laboratory examination in a 5-year-old girl.In regard to coincidence of selective IgA deficiency with autoimmune diseases,celiac disease was detected in a girl and juvenile idiopathic arthritis in a boy.Serum IgA concentrations differed significantly when patients were grouped according to age at the beginning of the study(P<0.001),age at diagnosis of T1D(P=0.015)and coincidence of celiac disease(CD)(P=0.038).However,when the age of the patients was adjusted,difference in serum IgA concentrations was not statistically significant despite CD was present or not.Moreover,serum IgA concentrations were positively correlated with serum IgG(P<0.001)and IgE(P=0.001)concentrations and negatively correlated with serum antigliadin antibody IgG(P=0.035)concentrations.There was no association or correlation of serum IgA concentrations with glycemic control.Conclusion:The prevalence of selective IgA deficiency in Greek children and adolescents with T1D is high(3.0%).The correlation of serum IgA concentrations with serum IgG,IgE and anti-gliadin antibody IgG concentrations needs further investigation.