Pulmonologist perspectives regarding diagnosis and management of primary immunodeficiency diseases

Akhter, Javeed; Seeborg, Filiz O.; Boyle, Marcia; Scalchunes, Christopher; Hernandez-Trujillo, Vivian

doi:10.2500/aap.2016.37.4007

Cited by 4 publications

(3 citation statements)

References 1 publication

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“…Moreover, even after a diagnosis of PAD, pulmonologist are usually the first specialist to whom patients are referred. An early detection of the underlying immune defect and the consequent establishment of appropriate treatment strategies may significantly reduce the occurrence of new infections and of long-term lung damage [29,38,39]. On the contrary, diagnostic delay may be responsible for some degree of permanent impairment in lung function in up to 54% of patients [38,40].…”

Section: Pulmonary Complications Of Padsmentioning

confidence: 99%

The broad spectrum of lung diseases in primary antibody deficiencies

et al. 2018

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Human primary immunodeficiency diseases (PIDs) represent a heterogeneous group of more than 350 disorders. They are rare diseases, but their global incidence is more relevant than generally thought. The underlying defect may involve different branches of the innate and/or adaptive immune response. Thus, the clinical picture may range from severe phenotypes characterised by a broad spectrum of infections to milder infectious phenotypes due to more selective (and frequent) immune defects. Moreover, infections may not be the main clinical features in some PIDs that might present with autoimmunity, auto-inflammation and/or cancer. Primary antibody deficiencies (PADs) represent a small percentage of the known PIDs but they are the most frequently diagnosed, particularly in adulthood. Common variable immunodeficiency (CVID) is the most prevalent symptomatic PAD.PAD patients share a significant susceptibility to respiratory diseases that represent a relevant cause of morbidity and mortality. Pulmonary complications include acute and chronic infection-related diseases, such as pneumonia and bronchiectasis. They also include immune-mediated interstitial lung diseases, such as granulomatous-lymphocytic interstitial lung disease (GLILD) and cancer. Herein we will discuss the main pulmonary manifestations of PADs, the associated functional and imaging findings, and the relevant role of pulmonologists and chest radiologists in diagnosis and surveillance.

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Section: Pulmonary Complications Of Padsmentioning

confidence: 99%

The broad spectrum of lung diseases in primary antibody deficiencies

et al. 2018

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“…Evaluation of attitudes and practices related to diagnosis and management of PID among family practice physicians and pulmonologists was conducted among the members of the American Medical Association and the American Osteopathic Association [15,16]. During the survey, information was collected on primary patient care settings, number of PID diagnoses being followed, awareness of PID identification testing and treatment strategies, use of Ig replacement therapy, hygiene-based interventions, and vaccination recommendations.…”

Section: Discussionmentioning

confidence: 99%

Physicians’ awareness concerning primary immunodeficiencies in the Ternopil Region of Ukraine

Boyarchuk¹,

Lewandowicz-Uszyńska²,

Kinash³

et al. 2018

polp

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Introduction: Primary immunodeficiencies (PID) are genetic defects of the immune system that result in chronic, serious, and often life-threatening infections if they are not diagnosed and treated. Worldwide, from 70 to 90% of PID sufferers remain undiagnosed because of poor awareness. Early diagnosis is very important for adequate prevention and management of PID infectious complications and may improve the life quality of patients with PID. Aim of the study: The aim of this study was to assess the awareness of physicians of different specialties about the signs of primary immunodeficiency in children and adults. Material and methods: A survey among physicians of different specialties on awareness of PID was conducted. The study involved physicians of the Ternopil region of Ukraine. Of 103 participants 42 were paediatricians, 25 general practitioners/family physicians, eight internists, 13 paediatric surgical specialists, and 15 physicians of paediatric sub-specialties. The survey consisted of a questionnaire containing 25 questions. Results: Sixty-one (59.2%) physicians gave more than 50% of correct answers. The percentage of correct answers to the questions about warning signs of PID in children was 66.1% among paediatricians and 64% among general practitioners, in adults-66.7% and 72%, respectively. The lowest knowledge of PID was about the specific signs of PID, more often concerning verification of Nijmegen breakage syndrome, ataxia-telangiectasia, and DiGeorge syndrome. Conclusions: This study has revealed poor awareness about PID among physicians in the Ternopil region of Ukraine. There was no significant difference in the percentages of correct answers among the first-contact physicians (paediatricians, general practitioners/family physicians) and paediatric sub-specialists. There is a significant need for educational programs to improve physicians' knowledge on PID. Implementation of the model of combined physician education and public awareness may improve detection of PID in children in the early stages.

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“…In addition to primary care clinicians, pulmonologists frequently encounter patients with recurrent respiratory infections. Orange et al 18 report the result of a survey of lung specialists designed to identify opportunities to improve diagnosis and clinical management of patients with primary immunodeficiency among pulmonologists. Also, with regard to clinical management of this disease, Bonilla 19 extends our knowledge by reviewing intravenous and subcutaneous IgG replacement therapy.…”

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confidence: 99%

A Janus tale of the two faces of corticosteroid therapy: A potential for adverse effects versus a steroid-sparing benefit of certain therapies

Bellanti¹,

Settipane²

2016

allergy asthma proc

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Pulmonologist perspectives regarding diagnosis and management of primary immunodeficiency diseases

Abstract: Differences in the diagnosis and treatment of patients with PIDD between these two groups of specialists revealed areas in which PIDD-focused educational initiatives may be helpful for pulmonologists.

Cited by 4 publications

References 1 publication

The broad spectrum of lung diseases in primary antibody deficiencies

The broad spectrum of lung diseases in primary antibody deficiencies

Physicians’ awareness concerning primary immunodeficiencies in the Ternopil Region of Ukraine

A Janus tale of the two faces of corticosteroid therapy: A potential for adverse effects versus a steroid-sparing benefit of certain therapies

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