The efficacy and safety of systemic corticosteroids as first line treatment for granulomatous lymphocytic interstitial lung disease

Smits, Bas M.; Goldacker, Sigune; Seneviratne, Suranjith L.; Malphettes, Marion; Longhurst, Hilary; Mohamed, Omar E; Witt-Rautenberg, C; Leeman, Lucy; Schwaneck, Eva Christina; Raymond, I; Meghit, K; Uhlmann, Annette; Winterhalter, Ch; Montfrans, Joris van; Klima, Miriam; Workman, Sarita; Fieschi, Claire; Lorenza, Landi; Boyle, S; Onyango‐Odera, Shamim; Price, S; Schmalzing, Marc; Aurillac, V; Prasse, Antje; Hartmann, Ieneke J. C.; Meerburg, Jennifer J.; Corput, Mariette Kemner-van de; Tiddens, Harm A.W.M.; Grimbacher, Bodo; Kelleher, Peter; Patel, Smita Y.; Korganow, A-S; Viallard, J.-F.; Tony, Hans‐Peter; Bethune, Claire; Schulze‐Koops, Hendrik; Witte, Torsten; Huissoon, Aarnoud; Baxendale, Helen; Grigoriadou, Sofia; Oksenhendler, Éric; Burns, Siobhan O.; Warnatz, Klaus

doi:10.1016/j.jaci.2022.12.813

Cited by 10 publications

(9 citation statements)

References 32 publications

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“…Thirty-one case reports and case series reported the use of CT in the diagnostic work up ( 12 – 22 , 26 – 45 ). Thirteen observational studies relied primarily on CT as their only criteria for CVID-ILD ( 2 , 23 , 27 , 46 – 55 ), while 13 studies required either histological confirmation ( 5 , 24 , 25 , 56 – 63 ) or an impairment in pulmonary function ( 64 , 65 ) in addition to the detection of relevant CT abnormalities. As CT imaging was the basis for the diagnosis of CVID-ILD, CT imaging was abnormal in all patients.…”

Section: Resultsmentioning

confidence: 99%

“…One recent paper by Smits et al. was added to the review despite being published after our search was completed, as it provides additional insight into the diagnostic criteria used for CVID-ILD ( 55 ). The authors recruited patients from the STILPAD study in which appropriate radiographic signs of CVID-ILD were sufficient for diagnosis.…”

Section: Resultsmentioning

confidence: 99%

“…The authors recruited patients from the STILPAD study in which appropriate radiographic signs of CVID-ILD were sufficient for diagnosis. According to the authors’ classification, a ‘possible’ diagnosis was made if patients presented radiographic signs of CVID-ILD only, whereas a ‘probable’ diagnosis required either a probability score >50% and radiographic signs of CVID-ILD or histological confirmation of CVID-ILD ( 55 ).…”

Section: Resultsmentioning

confidence: 99%

“…Other potential biomarkers have been used to developed diagnostic prediction models and to help avoid biopsy (as discussed further below). Furthermore, different blood biomarkers have been reported to be associated with CVID-ILD progression, and these include increased level of B cell-activating factor (BAFF), IgM in serum, the soluble form of the interleukin-2 receptor (sIL-2R) and neopterin ( 17 , 48 , 55 , 70 ). These data are summarised in Table 3 .…”

Section: Resultsmentioning

confidence: 99%

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Diagnostic testing for interstitial lung disease in common variable immunodeficiency: a systematic review

et al. 2023

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IntroductionCommon variable immunodeficiency related interstitial lung disease (CVID-ILD, also referred to as GLILD) is generally considered a manifestation of systemic immune dysregulation occurring in up to 20% of people with CVID. There is a lack of evidence-based guidelines for the diagnosis and management of CVID-ILD.AimTo systematically review use of diagnostic tests for assessing patients with CVID for possible ILD, and to evaluate their utility and risks.MethodsEMBASE, MEDLINE, PubMed and Cochrane databases were searched. Papers reporting information on the diagnosis of ILD in patients with CVID were included.Results58 studies were included. Radiology was the investigation modality most commonly used. HRCT was the most reported test, as abnormal radiology often first raised suspicion of CVID-ILD. Lung biopsy was used in 42 (72%) of studies, and surgical lung biopsy had more conclusive results compared to trans-bronchial biopsy (TBB). Analysis of broncho-alveolar lavage was reported in 24 (41%) studies, primarily to exclude infection. Pulmonary function tests, most commonly gas transfer, were widely used. However, results varied from normal to severely impaired, typically with a restrictive pattern and reduced gas transfer.ConclusionConsensus diagnostic criteria are urgently required to support accurate assessment and monitoring in CVID-ILD. ESID and the ERS e-GLILDnet CRC have initiated a diagnostic and management guideline through international collaboration.Systematic review registrationhttps://www.crd.york.ac.uk/prospero/, identifier CRD42022276337.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Diagnostic testing for interstitial lung disease in common variable immunodeficiency: a systematic review

et al. 2023

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“…After assessment for respiratory mycobacteria, macrolides may be considered, both to limit infectious exacerbation of chronic pulmonary disease as well as potential anti-inflammatory benefit. Recent evidence has shown that systemic corticosteroid treatment is a possible first-line therapy for CVID ILD, with improvement of CT scores and PFTs and long-term remission is 42% of patients without major side effects [60 ▪▪ ]. Rituximab has shown efficacy for CVID ILD treatment, either as a single agent or part of combination therapy with an additional immunomodulator such as azathioprine or mycophenolate [15,57,61 ▪▪ ].…”

Section: Introductionmentioning

confidence: 99%

Interstitial lung diseases in inborn errors of immunity

Sullivan,

Maniam,

Maglione

2023

Current Opinion in Allergy &Amp; Clinical Immunology

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Purpose of review Our goal is to review current understanding of interstitial lung disease (ILD) affecting patients with inborn errors of immunity (IEI). This includes understanding how IEI might predispose to and promote development or progression of ILD as well as how our growing understanding of IEI can help shape treatment of ILD in these patients. Additionally, by examining current knowledge of ILD in IEI, we hope to identify key knowledge gaps that can become focus of future investigative efforts. Recent findings Recent identification of novel IEI associated with ILD and the latest reports examining treatment of ILD in IEI are included. Of noted interest, are recent clinical studies of immunomodulatory therapy for ILD in common variable immunodeficiency. Summary ILD is a frequent complication found in many IEI. This article provides a guide to identifying manifestations of ILD in IEI. We review a broad spectrum of IEI that develop ILD, including antibody deficiency and immune dysregulation disorders that promote autoimmunity and autoinflammation. This work integrates clinical information with molecular mechanisms of disease and diagnostic assessments to provide an expedient overview of a clinically relevant and expanding topic.

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Dysregulated Lymphocyte Antigen Receptor Signaling in Common Variable Immunodeficiency with Granulomatous Lymphocytic Interstitial Lung Disease

et al. 2023

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The efficacy and safety of systemic corticosteroids as first line treatment for granulomatous lymphocytic interstitial lung disease

Cited by 10 publications

References 32 publications

Diagnostic testing for interstitial lung disease in common variable immunodeficiency: a systematic review

Diagnostic testing for interstitial lung disease in common variable immunodeficiency: a systematic review

Interstitial lung diseases in inborn errors of immunity

Dysregulated Lymphocyte Antigen Receptor Signaling in Common Variable Immunodeficiency with Granulomatous Lymphocytic Interstitial Lung Disease

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