Pulmonary Disease Burden in Primary Immune Deficiency Disorders: Data from USIDNET Registry

Patrawala, Meera; Cui, Ying; Peng, Limin; Fuleihan, Ramsay; Garabedian, Elizabeth; Patel, Kiran; Guglani, Lokesh

doi:10.1007/s10875-019-00738-w

Cited by 18 publications

(19 citation statements)

References 42 publications

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“…Our observations may support the hypothesis that, despite IgRT, some patients develop chronic lung disease, which matches earlier reports [15]. Moreover, serious pulmonary complications may progress despite adequate serum IgG concentration.…”

Section: Discussionsupporting

confidence: 93%

“…It is worth highlighting that we diagnosed ILD in 1 patient with XLA, although according to the literature, ILD also appears to be very rare or absent in this condition [4]. Interestingly, the incidence of pulmonary complications appears to be higher in CVID than XLA, and it is probably related to factors other than antibody deficiency [15,23].…”

Section: Discussionmentioning

confidence: 67%

“…CVID was associated with ILD, bronchiectasis, and mixed variant. Although CVID is the most prevalent symptomatic PID, and chronic lung disease is among its most common complications, very little attention has been paid to the paediatric population so far [15]. Ramzi et al [16] in their meta-analysis reported that the overall prevalence of bronchiectasis in CVID patients was 34%, while Van de Hen et al [17] highlighted the occurrence of bronchiectasis in 20% of children with CVID, ILD in 15%, and 5% patients showed a mixed pattern.…”

Section: Discussionmentioning

confidence: 99%

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Interstitial lung disease and bronchiectasis in children with primary immunodeficiencies: a single-centre experience

Tąpolska-Jóźwiak¹,

Szczawińska-Popłonyk²,

Bręborowicz³

2021

polp

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Introduction: Primary immunodeficiencies (PIDs) are a heterogeneous group of inherited disorders in which one or several components of the immune system are involved. Pulmonary complications remain the most common cause of morbidity and mortality in patients with PIDs. The term chronic lung disease refers to interstitial lung disease (ILD) and bronchiectasis. Infectious as well as non-infectious factors may contribute to the development of chronic lung disease. In contrast to adults with PIDs, reports about chronic lung disease in children are limited. Prevention and early diagnosis of pulmonary complications improve patients' prognosis and quality of life. Aim of the study: To evaluate ILD and bronchiectasis in immunocompromised children. Material and methods:The study was conducted through a retrospective review of the data of 44 children with different PIDs, taking into consideration the following: pulmonary infections, autoimmunity, presence of comorbidities, and therapy performed before the diagnosis of ILD/bronchiectasis. Results: Interstitial lung disease was diagnosed in 25 cases (56.82%) and bronchiectasis in 8 cases (18.18%); both diseases were observed in 11 cases (25.00%). The earliest radiological features of ILD were detected in patients with combined PIDs. Bronchiectasis had already been reported in children above 5 years of age with a defect in antibody production. Bacterial infections requiring intravenous antibiotic treatment occurred significantly more frequently in the medical history of children with bronchiectasis (p < 0.001), whereas children with the ILD component showed a higher prevalence of pulmonary fungal infections (p = 0.034). Conclusions: Regular clinical evaluation, supported by additional tests results including radiological findings, is crucial for the early diagnosis of chronic lung disease.

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Section: Discussionsupporting

confidence: 93%

Section: Discussionmentioning

confidence: 67%

Section: Discussionmentioning

confidence: 99%

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Interstitial lung disease and bronchiectasis in children with primary immunodeficiencies: a single-centre experience

Tąpolska-Jóźwiak¹,

Szczawińska-Popłonyk²,

Bręborowicz³

2021

polp

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“…Patients often require hospitalization and intravenous antibiotics. Bronchiectases are a long-term sequel of recurrent lung infections in CVID[ 22 ]. Still a significant percentage of patients are diagnosed when bronchiectases are already present[ 23 ].…”

Section: Clinical Featuresmentioning

confidence: 99%

From infections to autoimmunity: Diagnostic challenges in common variable immunodeficiency

Więsik-Szewczyk

Jahnz-Różyk

2020

WJCC

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Common variable immunodeficiency (CVID) is the most common clinically significant primary antibody deficiency diagnosed in adults. The early symptoms are not specific. They include common infections, mainly of the respiratory tract, caused by typical microorganisms, so cases can be missed in primary care. In the majority of patients increased susceptibility to infections coexists with signs or symptoms of autoimmunity, inflammation or polyclonal lymphoproliferation, which can divert diagnosis from immune deficiency. The overall incidence of malignancy is increased in CVID and certain cancers are significantly more common. Lymphomas and gastric carcinoma are the most frequently reported malignancies in CVID, so a high index of suspicion is recommended. Diagnostic delay in CVID is seen worldwide. The main goal of this paper is to increase the awareness about CVID among health care professionals. We aim to present features which can be helpful in CVID diagnosis in order to shorten the “latency” of proper management of CVID patients. We review clinical symptoms, complications and laboratory abnormalities of CVID. Immunoglobulin replacement therapy is regarded as the cornerstone of pharmacological intervention. New modes of Ig application, mainly subcutaneously and via the hyaluronidase-facilitated subcutaneous route, help to adjust therapy to patients’ needs and preferences. Still there remain unmet needs. It remains to be seen whether CVID complications can be avoided by earlier diagnosis, treatment and thorough monitoring in the context of increased risk of malignancy. Development of patient tailored protocols depending on the clinical phenotype and risk factors might be more appropriate. The most important consideration is to diagnose suspected cases and stratify patients in a precise and timely way. Work is needed to define features predictive of unfavorable prognosis.

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“…An analysis of 1,937 PID patients registered in the U.S. Immunodeficiency Network (USIDNET) found that 61.7% of patients with CVID were reported as having pulmonary disease. 19 The study further narrowed pulmonary disease by type and revealed that the highest prevalence of airway disease was in CVID patients. Nearly 58% of CVID patients were reported to have airway disease (which included Table 1 Diagnosis of common variable immunodeficiency disorders 14,32…”

Section: Common Variable Immunodeficiency and Bronchiectasismentioning

confidence: 94%

Common Variable Immunodeficiency and Other Immunodeficiency Syndromes in Bronchiectasis

McShane

2021

Semin Respir Crit Care Med

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Immunodeficiency represents a vast number of diseases and syndromes. Both primary and secondary forms of immunodeficiency are important contributors to the development of bronchiectasis. Primary immune deficiencies, in particular, are increasingly identified and defined as contributors. Specific immune deficiencies that are closely associated with bronchiectasis and as discussed in this article are common variable immunodeficiency, specific antibody deficiency, immunodeficiencies involving immunoglobulin E, DOCK8 immunodeficiency, phosphoglucomutase 3 deficiency, activated phosphoinositide 3-kinase delta syndrome, and X-linked agammaglobulinemia. Each of these primary immune deficiencies has unique nuances. Vigilance for these unique signs and symptoms is likely to improve recognition of specific immunodeficiency in the idiopathic bronchiectasis patient. Secondary forms of immunodeficiency occur as a result of a separate disease process. Graft versus host disease, malignancy, and human immunodeficiency virus are three classic examples discussed in this article. An awareness of the potential for these disease settings to lead to bronchiectasis is necessary to optimize patient care. With understanding and mindfulness toward the intricate relationship between bronchiectasis and immunodeficiency, there is an opportunity to elucidate pathophysiologic underpinnings between these two syndromes.

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Pulmonary Disease Burden in Primary Immune Deficiency Disorders: Data from USIDNET Registry

Cited by 18 publications

References 42 publications

Interstitial lung disease and bronchiectasis in children with primary immunodeficiencies: a single-centre experience

Interstitial lung disease and bronchiectasis in children with primary immunodeficiencies: a single-centre experience

From infections to autoimmunity: Diagnostic challenges in common variable immunodeficiency

Common Variable Immunodeficiency and Other Immunodeficiency Syndromes in Bronchiectasis

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