Granulomatous-Lymphocytic Interstitial Lung Disease in a Patient With Common Variable Immunodeficiency

Shah, Jessica; Amin, Sanjiv B.; Verma, Nupur; Mohammed, Tan-Lucien

doi:10.1067/j.cpradiol.2017.04.007

Cited by 7 publications

(5 citation statements)

References 8 publications

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“…No significant chemotherapy-related complications occurred. Similar results in terms of both effectiveness and safety were achieved in 4 case reports described between 2015 and 2017, by using the same therapeutic protocol including RTX and azathioprine [109–112]. In 2017 Jolles et al described the use of 2-[(18)F]-fluoro-2-deoxy- d -glucose positron emission tomography and computed tomography (FDG PET-CT) scanning for the monitoring of response in a CVID patient with GLILD, treated with 2 infusions of RTX at dose of 1 g (with an interval of 1 month) in combination with long-term standard-dose (1–2 g/day) mycophenolate [113].…”

Section: Introductionsupporting

confidence: 71%

Immunosuppressive therapy with rituximab in common variable immunodeficiency

et al. 2019

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Common variable immunodeficiency (CVID) is the most frequent symptomatic primary antibody deficiency in adulthood and is characterized by the marked reduction of IgG and IgA serum levels. Thanks to the successful use of polyvalent immunoglobulin replacement therapy to treat and prevent recurrent infections, non-infectious complications, including autoimmunity, polyclonal lymphoproliferation and malignancies, have progressively become the major cause of morbidity and mortality in CVID patients. The management of these complications is particularly challenging, often requiring multiple lines of immunosuppressive treatments. Over the last 5–10 years, the anti-CD20 monoclonal antibody (i.e., rituximab) has been increasingly used for the treatment of both autoimmune and non-malignant lymphoproliferative manifestations associated with CVID. This review illustrates the evidence on the use of rituximab in CVID. For this purpose, first we discuss the mechanisms proposed for the rituximab mediated B-cell depletion; then, we analyze the literature data regarding the CVID-related complications for which rituximab has been used, focusing on autoimmune cytopenias, granulomatous lymphocytic interstitial lung disease (GLILD) and non-malignant lymphoproliferative syndromes. The cumulative data suggest that in the vast majority of the studies, rituximab has proven to be an effective and relatively safe therapeutic option. However, there are currently no data on the long-term efficacy and side effects of rituximab and other second-line therapeutic options. Further randomized controlled trials are needed to optimize the management strategies of non-infectious complications of CVID.

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Section: Introductionsupporting

confidence: 71%

Immunosuppressive therapy with rituximab in common variable immunodeficiency

et al. 2019

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“…Pulmonary function tests (PFT) as assessment tools were reported in 40 studies ( 5 , 13 , 15 – 18 , 20 – 24 , 27 , 28 , 30 – 36 , 38 – 45 , 49 , 52 – 54 , 56 , 57 , 59 , 60 , 62 – 64 , 69 ), and included spirometry, measurement of diffusion capacity and assessment of static lung volumes (i.e., total lung capacity (TLC), and residual volume (RV)). Two studies reported PFT abnormalities as potential diagnostic criteria for CVID-ILD in addition to CT ( 64 , 65 ).…”

Section: Resultsmentioning

confidence: 99%

“…The diagnosis of CVID-ILD was confirmed by biopsy in 31 case reports and series ( 12 – 22 , 26 – 45 ) and was used as an obligatory inclusion criterium for CVID-ILD patients in eleven studies ( 5 , 24 , 25 , 54 , 56 – 60 , 62 , 63 ). Transbronchial biopsy (TBB) was described in 17 studies involving 57 patients, 28 of whom had definitive results, while the remaining patients underwent a supplemental biopsy modality to confirm the diagnosis ( 16 – 18 , 22 , 30 , 31 , 34 , 38 , 42 , 44 , 50 , 58 , 59 , 62 – 64 , 69 ). The most common diagnostic findings were non-necrotizing granulomatous and lymphocytic inflammation.…”

Section: Resultsmentioning

confidence: 99%

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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“…A con dent diagnosis of GL-ILD often requires a histological evaluation [4] combined with cultures and special stains to exclude infection [4,5]. However, surgical lung biopsies in patients with CVID can be associated with an increased risk of decompensation, infection and surgical complications [6].…”

Section: Introductionmentioning

confidence: 99%

Granulomatous Lymphocytic Interstitial Lung Disease (GL-ILD): CT Imaging Features and Clinical Findings to Expedite a Timely Diagnosis

Wynn¹,

Berkowitz²,

Little³

et al. 2021

Preprint

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Background: Establishing a diagnosis of Granulomatous Lymphocytic-Interstitial Lung Disease (GL-ILD) is difficult due to the overlapping CT imaging and histologic features, and are often confused with infection, sarcoidosis and/or follicular bronchiolitis. Research Question: Identify specific CT imaging features and clinical characteristics to suggest a confident diagnosis of Granulomatous Lymphocytic-Interstitial Lung Disease (GL-ILD).Study Design and Methods: IRB-approved retrospective case series study involving the review of the electronic medical record and CT chest imaging of eight patients with GL-ILD.Results: Bronchocentric airway-centered lower lobe predominant (part solid nodules more common than ground glass or solid nodules) ranging from 5 to 10 mm in size were found consistently in a majority of the eight patients with GL-ILD. Interpretation: In combination with the above pulmonary nodular pattern, mediastinal and hilar lymphadenopathy in conjunction, either splenomegaly or splenectomy and/or abdominal lymphadenopathy are excellent CT imaging features to prospectively suggest the diagnosis of GL-ILD in a patient with CVID.

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Granulomatous-Lymphocytic Interstitial Lung Disease in a Patient With Common Variable Immunodeficiency

Cited by 7 publications

References 8 publications

Immunosuppressive therapy with rituximab in common variable immunodeficiency

Immunosuppressive therapy with rituximab in common variable immunodeficiency

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

Granulomatous Lymphocytic Interstitial Lung Disease (GL-ILD): CT Imaging Features and Clinical Findings to Expedite a Timely Diagnosis

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