Organising pneumonia in common variable immunodeficiency

Boujaoude, Ziad; Arya, Rohan; Rafferty, William; Dammert, Pedro

doi:10.1136/bcr-2013-008905

Cited by 7 publications

(11 citation statements)

References 9 publications

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“…Treatment with glucocorticoids led to a partial response in 13% (four individuals) and failed to maintain remission in 7% (two individuals) of patients (18,29). There are, however, also literature reports about the positive effects of glucocorticoids (16,17,20,21). 23% (seven individuals) of all patients using glucocorticoids had resolution of symptoms.…”

Section: Not Mentionedmentioning

confidence: 99%

“…Ten additional studies included glucocorticoid treatment as one of several therapies (Tables 2 and 3). Five of these reported no effect of glucocorticoids (26,27,31,(36)(37)(38), one reported relapse after initial remission (29) and four reported treatment success (16,17,20,21). The article by Kanathur et al is particularly interesting as it describes a case in which glucocorticoids initially failed to have any effect at all but FIGURE 2 | Comparison of the available qualitative and quantitative outcomes of studies that reported on patients (N) treated with steroids, rituximab monotherapy and rituximab combination therapy.…”

Section: Glucocorticoidsmentioning

confidence: 99%

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Treatment Strategies for GLILD in Common Variable Immunodeficiency: A Systematic Review

et al. 2021

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IntroductionBesides recurrent infections, a proportion of patients with Common Variable Immunodeficiency Disorders (CVID) may suffer from immune dysregulation such as granulomatous-lymphocytic interstitial lung disease (GLILD). The optimal treatment of this complication is currently unknown. Experienced-based expert opinions have been produced, but a systematic review of published treatment studies is lacking.GoalsTo summarize and synthesize the published literature on the efficacy of treatments for GLILD in CVID.MethodsWe performed a systematic review using the PRISMA guidelines. Papers describing treatment and outcomes in CVID patients with radiographic and/or histologic evidence of GLILD were included. Treatment regimens and outcomes of treatment were summarized.Results6124 papers were identified and 42, reporting information about 233 patients in total, were included for review. These papers described case series or small, uncontrolled studies of monotherapy with glucocorticoids or other immunosuppressants, rituximab monotherapy or rituximab plus azathioprine, abatacept, or hematopoietic stem cell transplantation (HSCT). Treatment response rates varied widely. Cross-study comparisons were complicated because different treatment regimens, follow-up periods, and outcome measures were used. There was a trend towards more frequent GLILD relapses in patients treated with corticosteroid monotherapy when compared to rituximab-containing treatment regimens based on qualitative endpoints. HSCT is a promising alternative to pharmacological treatment of GLILD, because it has the potential to not only contain symptoms, but also to resolve the underlying pathology. However, mortality, especially among immunocompromised patients, is high.ConclusionsWe could not draw definitive conclusions regarding optimal pharmacological treatment for GLILD in CVID from the current literature since quantitative, well-controlled evidence was lacking. While HSCT might be considered a treatment option for GLILD in CVID, the risks related to the procedure are high. Our findings highlight the need for further research with uniform, objective and quantifiable endpoints. This should include international registries with standardized data collection including regular pulmonary function tests (with carbon monoxide-diffusion), uniform high-resolution chest CT radiographic scoring, and uniform treatment regimens, to facilitate comparison of treatment outcomes and ultimately randomized clinical trials.

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Section: Not Mentionedmentioning

confidence: 99%

Section: Glucocorticoidsmentioning

confidence: 99%

Treatment Strategies for GLILD in Common Variable Immunodeficiency: A Systematic Review

et al. 2021

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“…Organising pneumonia, formerly known as bronchiolitis obliterans organising pneumonia, has been described as a frequent presentation in PADs [93][94][95][96][97]. It is a nonspecific reactive inflammation resulting from different causes of epithelial lung injury (infections, inflammation and fibrosis) and characterised by plugs of granulation tissue and spirals of fibroblasts, known as Masson bodies, in the alveolar spaces [16,98].…”

Section: Asthma and Copdmentioning

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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“…6,13-15 Additionally, granulomatous lung disease is found in many cases 16-18 and organizing pneumonia (OP) in some. 19-21 Granulomatous-lymphocytic interstitial lung disease (GLILD) has been used as an encompassing term for this combination of pathologic findings in CVID. 6 …”

Section: Introductionmentioning

confidence: 99%