Pulmonary Complications of Primary Immunodeficiencies

Routes, John M.

doi:10.1016/b978-1-4160-4710-0.00082-1

Cited by 2 publications

(2 citation statements)

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“…BE is a known complication of combined variable immunodeficiency and detected in more than 20% of patients, 7 corresponding to the prevalence of 29% in our study cohort. However, our findings suggest that BE often develops also in CHH patients with inapparent immunodeficiency, implicating the presence of other contributing factors.…”

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confidence: 57%

High prevalence of bronchiectasis in patients with cartilage-hair hypoplasia

Kostjukovits

Klemetti

Föhr

et al. 2017

Journal of Allergy and Clinical Immunology

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High prevalence of bronchiectasis in patients with cartilage-hair hypoplasia To the Editor: Cartilage-hair hypoplasia (CHH, MIM #250250) is a metaphyseal chondrodysplasia with variable immunodeficiency and increased risk of malignancy. We previously showed that in patients with CHH with chronic respiratory symptoms, the prevalence of bronchiectasis (BE) is 52%. 1 The prevalence of BE and the contributing risk factors in the overall CHH population remain unknown. High-resolution computed tomography (HRCT) is the criterion standard investigation for assessing BE; however, radiation exposure is a concern. Pulmonary changes in patients with CHH have not been examined systematically and the applicability of lung magnetic resonance imaging (MRI) in assessing their lung pathology is unclear. We investigated the prevalence of BE in a random cohort of 34 Finnish patients with genetically confirmed CHH (6 men, 28 women; median age, 39 years, range, 13-68 years). All patients or caregivers gave their written informed consent. The Institutional Research Ethics Committee approved the study. We collected clinical data, obtained blood tests, and performed lung HRCT (34 of 34) and MRI (16 of 34) (for detailed imaging protocols, see Appendix E1 in this article's Online Repository at www. jacionline.org). We analyzed the clinical and immunologic correlates with BE with the Fisher exact test, the Mann-Whitney test, and multiple logistic regression analysis. Performance of MRI compared with HRCT was determined by the Spearman rank correlation coefficient (rho). Table I presents the patients' clinical and laboratory characteristics. HRCT showed BE in 10 of 34 patients (29%), aged 29 to 68 years. BE was unilateral (n 5 2) or bilateral (n 5 8) and was located most commonly in the lower lobes and right middle lobe, but was also found in all other lobes (Fig 1). Additional findings on HRCT were acute inflammation (n 5 3), fibrosis-like changes (n 5 6), and nonspecific subpleural nodules of less than 0.5 cm in size (n 5 8). MRI showed lung abnormalities in 8 of 16 patients and had a good correlation with HRCT (overall r, 0.820; P < .001) (see Table E1 in this article's Online Repository at www.jacionline.org). Patients with BE had significantly higher serum IgG and white blood cells, total lymphocyte, and CD16/56 1 cell counts (Table I). However, these parameters correlated significantly with age (see Fig E1 in this article's Online Repository at www.jacionline.org). Patients with BE tended to have more sinus infections, pneumonia, and chronic cough (defined as ongoing daily cough lasting > _1 year). BE was diagnosed in 4 of 6 patients with a history of smoking, but when controlled for age, this association became insignificant. In the multiple logistic regression analysis, only higher CD16/56 1 cell counts remained associated with the presence of BE (P 5 .026; B

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confidence: 57%

High prevalence of bronchiectasis in patients with cartilage-hair hypoplasia

Kostjukovits

Klemetti

Föhr

et al. 2017

Journal of Allergy and Clinical Immunology

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“…Routinely monitor the patient's nutritional status and basic chemistries, guided by severity of enteropathies 8. Malignancy rates are as high as 20% in patients with CVID, with the most frequent being lymphoma, gastric cancer, and breast cancer 1,10. Regular screening and monitoring for these complications based on individual disease manifestation are essential to patient care 8…”

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confidence: 99%

Piecing together the subtle clues of common variable immunodeficiency

Kochanoff

2023

Jaapa

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A 45-year-old woman presented to the immunology clinic with complaints of lifelong fatigue and frequent, prolonged infections often requiring antibiotics for resolution.History Ten years earlier, during a workup for an ovarian cyst, the patient had an incidental finding of scattered lymphadenopathy throughout the abdomen and pelvis on CT scan. This prompted an excisional left inguinal lymph node biopsy, which, like the ovarian cyst, was benign. She was referred to hematology/oncology and allergy/immunology for further evaluation. During these consultations, diagnostic evaluations were negative for myeloproliferative disease, hepatitis A and B, HIV, Epstein-Barr virus (EBV), and autoimmune conditions. The only abnormal finding was a low quantitative serum immunoglobulin G (IgG). No cause for lymphadenopathy or low IgG was determined.Eight years later, the patient presented to the ED with profuse vaginal bleeding and petechiae diagnosed as an acute episode of immune thrombocytopenia (ITP). ITP often is triggered by a viral infection that sets off an autoimmune

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Pulmonary Complications of Primary Immunodeficiencies

Cited by 2 publications

References 134 publications

High prevalence of bronchiectasis in patients with cartilage-hair hypoplasia

High prevalence of bronchiectasis in patients with cartilage-hair hypoplasia

Piecing together the subtle clues of common variable immunodeficiency

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