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. 2020 Jun 19;55(2):174-183.
doi: 10.14744/TurkPediatriArs.2020.46656. eCollection 2020.

Evaluation of pulmonary findings in patients with humoral immunodeficiency

Zuhal Karalı  1 , Yasin Karalı  1 , Şükrü Çekiç  1 , Zeynep Yazıcı  2 , Yakup Canıtez  1 , Nihat Sapan  1 , Sara Şebnem Kılıç Gültekin  1
Affiliations

Evaluation of pulmonary findings in patients with humoral immunodeficiency

Zuhal Karalı et al. Turk Pediatri Ars. .

Abstract
in English, Turkish

Aim: To determine the frequency of sinopulmonary infections, detect changes in the respiratory system, and measure functional capacity of the lungs in our patients with humoral immunodeficiency.

Material and methods: Fifty-six patients with humoral immunodeficiency were enrolled in this study. The clinical, laboratory, and radiologic data, and pulmonary function tests of the subjects were evaluated from their file records, retrospectively.

Results: The distribution of our patients was as follows: 25 patients had common variable immune deficiency, three patients had X-linked agammaglobulinemia, five patients had hyper immunoglobulin M syndrome, 19 patients had deficiency of immunoglobulin G subset, and four patients had selective immunoglobulin A deficiency. The most common symptom of the patients was chronic cough (n=47, 83.9%). The most common pathologies on high-resolution computed tomography of the chest were atelectasis and bronchiectasis (27.7%). The most common pathology in pulmonary function tests was the presence of moderate obstructive patterns along with restrictive patterns (n=6,12.5%). The FEV 1, FVC, and FEF 25-75 values were significantly lower in patients with common variable immunodeficiency compared with the patients who had IgG subset deficiencies (p=0.001, p=0.01, p=0.01). Among the patients who were treated with intravenous immunoglobulin, the age at the diagnosis of immunodeficiency was higher in patients with bronchiectasis (14.2±8.4 years) compared with those without bronchiectasis (10.1±11.4 years) (p=0.04).

Conclusion: Clinical findings are not sufficient to monitor the structural and functional changes in the respiratory system, and patients should be evaluated using high-resolution computed tomography of the chest and pulmonary function tests.

Amaç: Humoral immün yetmezlik tanılı olgularımızda, sinopulmoner enfeksiyonların sıklığı ve bunların sonucunda solunum sisteminde meydana gelen değişikliklerin saptanması ve fonksiyonel akciğer kapasitelerinin ölçülmesi amaçlanmıştır.

Gereç ve yöntemler: Çalışmaya humoral immün yetmezlik tanılı 56 olgu alındı. Olguların dosya kayıtlarından klinik, laboratuvar, radyolojik görüntüleme bulguları ve solunum fonksiyon testi verileri geriye dönük olarak incelendi.

Bulgular: Olguların tanılara göre dağılımı; yaygın değişken immün yetmezlik tanılı 25 olgu, X’e bağlı agamaglobulinemi tanılı üç olgu, Hiper immunoglobulin M sendromu tanılı beş olgu, İgG alt grup eksikliği tanılı 19 olgu ve selektif immunoglobulin A eksikliği tanılı dört olgu şeklindedir. Olguların 37’si (%66,1) erkek 19’u (%33,9) kadın, yaş ortalaması 14,1±10,6 yıldı. Olguların en sık yakınması kronik öksürük idi (n=47, %83,9). Akciğerlerin yüksek çözünürlüklü bilgisayarlı tomografilerinde en sık görülen patoloji atelektazi ve bronşiektaziydi (%27,7). Solunum fonksiyon testlerinde en sık görülen anormallik orta obstrüktif ve orta restriktif patern birlikteliğiydi (n=6,%12,5). Solunum fonksiyon testi bulguları ile radyolojik bulguların her zaman tutarlı olmadığı görüldü. İntravenöz immünglobulin alan olgular arasında yaygın değişken immün yetmezlik tanılı olgularda, immünglobulin G alt grup eksikliği olan olgulara göre solunum fonksiyon testlerinde; FEV 1, FVC ve FEF 25–75 değerlerinin anlamlı düşük olduğu saptandı (p=0,001, p=0,01, p=0,01). İntravenöz immünglobulin tedavisi alan olgularda; bronşiektazisi olanların immün yetmezlik tanı yaşı (14,2±8,4 yıl), olmayanlara (10,1±11,4 yıl) göre anlamlı olarak büyüktü (p=0,04).

Çikarimlar: Klinik bulgular solunum sistemindeki yapısal ve fonksiyonel değişiklikleri izlemek için yeterli değildir ve olgular gereğinde akciğer yüksek çözünürlüklü bilgisayarlı tomografi ve solunum fonksiyon testleri ile değerlendirilmelidir.

Keywords: Humoral immunodeficiency; pulmonary findings; radiologic evaluation.

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Conflict of interest statement

Conflict of Interest: The authors have no conflicts of interest to declare.

Figures

Figure 1
Figure 1
Distribution of the findings on high resolution computed tomography of the lung by order of frequency
Figure 2
Figure 2
Appearances of the findings on high-resolution computed tomography of the lung. (a) Cystic bronchiectasis. (b) Bronchiectasis and atelectasis. (c) Ground-glass opacity. (d) Parenchymal nodule
See this image and copyright information in PMC

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