Rituximab for granulomatous lymphocytic interstitial lung disease in a patient with common variable immunodeficiency. Is single therapy enough?

Arraya, Mauricio; Castro, Yolanda; Navarro, J.; Sarmiento, E.; Cruz, Elena de la; Campoverde, Javier Carbone

doi:10.4172/1758-4272.1000159

Cited by 4 publications

(5 citation statements)

References 8 publications

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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 most common diagnostic findings were non-necrotizing granulomatous and lymphocytic inflammation. The use of video-assisted thoracoscopic surgery (VATS) was reported in 17 studies ( 15 , 16 , 21 , 26 , 33 , 34 , 36 , 39 – 41 , 45 , 47 , 57 , 60 , 62 , 63 , 69 ). The results from 81 patients demonstrated the characteristic histological findings of CVID-ILD.…”

Section: Resultsmentioning

confidence: 99%

“…Thirty-one case reports and case series reported the use of CT in the diagnostic work up (12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36)(37)(38)(39)(40)(41)(42)(43)(44)(45). Thirteen observational studies relied primarily on CT as their only criteria for CVID-ILD (2,23,27,(46)(47)(48)(49)(50)(51)(52)(53)(54)(55), while 13 studies required either histological PRISMA flow diagram of study selection process.…”

Section: Radiologymentioning

confidence: 99%

“…The diagnosis of CVID-ILD was confirmed by biopsy in 31 case reports and series (12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36)(37)(38)(39)(40)(41)(42)(43)(44)(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][63][64]69).…”

Section: Biopsymentioning

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: Radiologymentioning

confidence: 99%

Section: Biopsymentioning

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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“…All described treatment courses of rituximab monotherapy for CVID+PGD were effective ( Table 6). In 20 of the 22 patients with CVID+PGD were rituximab was part of treatment regimen, granulomatous disease was only present in the lungs (Supplemental Table 4) (49,(59)(60)(61)(62)(63)(64)(66)(67)(68)(69). In the majority of the included cases the dose of rituximab as part of combination therapy with azathioprine was consistent, namely 375 mg/m2 (49,59,60,62).…”

Section: Rituximab With or Without Azathioprinementioning

confidence: 99%

What Works When Treating Granulomatous Disease in Genetically Undefined CVID? A Systematic Review

et al. 2020

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BackgroundGranulomatous disease is reported in at least 8–20% of patients with common variable immunodeficiency (CVID). Granulomatous disease mainly affects the lungs, and is associated with significantly higher morbidity and mortality. In half of patients with granulomatous disease, extrapulmonary manifestations are found, affecting e.g. skin, liver, and lymph nodes. In literature various therapies have been reported, with varying effects on remission of granulomas and related clinical symptoms. However, consensus recommendations for optimal management of extrapulmonary granulomatous disease are lacking.ObjectiveTo present a literature overview of the efficacy of currently described therapies for extrapulmonary granulomatous disease in CVID (CVID+EGD), compared to known treatment regimens for pulmonary granulomatous disease in CVID (CVID+PGD).MethodsThe following databases were searched: Embase, Medline (Ovid), Web-of-Science Core Collection, Cochrane Central, and Google Scholar. Inclusion criteria were 1) CVID patients with granulomatous disease, 2) treatment for granulomatous disease reported, and 3) outcome of treatment reported. Patient characteristics, localization of granuloma, treatment, and association with remission of granulomatous disease were extracted from articles.ResultsWe identified 64 articles presenting 95 CVID patients with granulomatous disease, wherein 117 different treatment courses were described. Steroid monotherapy was most frequently described in CVID+EGD (21 out of 53 treatment courses) and resulted in remission in 85.7% of cases. In CVID+PGD steroid monotherapy was described in 15 out of 64 treatment courses, and was associated with remission in 66.7% of cases. Infliximab was reported in CVID+EGD in six out of 53 treatment courses and was mostly used in granulomatous disease affecting the skin (four out of six cases). All patients (n = 9) treated with anti-TNF-α therapies (infliximab and etanercept) showed remission of extrapulmonary granulomatous disease. Rituximab with or without azathioprine was rarely used for CVID+EGD, but frequently used in CVID+PGD where it was associated with remission of granulomatous disease in 94.4% (17 of 18 treatment courses).ConclusionAlthough the number of CVID+EGD patients was limited, data indicate that steroid monotherapy often results in remission, and that anti-TNF-α treatment is effective for granulomatous disease affecting the skin. Also, rituximab with or without azathioprine was mainly described in CVID+PGD, and only in few cases of CVID+EGD.

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Rituximab Monotherapy Is Effective as First-Line Treatment for Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in CVID Patients

Tessarin,

Baronio,

Gazzurelli

et al. 2023

J Clin Immunol

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Granulomatous lymphocytic interstitial lung disease (GLILD) represents a fatal immune dysregulatory complication in common variable immunodeficiency (CVID). Evidence-based diagnostic guidelines are lacking, and GLILD treatment consists in immunosuppressive drugs; nonetheless, therapeutical strategies are heterogeneous and essentially based on experts’ opinions and data from small case series or case reports.We aimed to evaluate the efficacy and safety of first-line Rituximab monotherapy for CVID-related GLILD, by assessing symptoms and quality of life alterations, immunological parameters, pulmonary function tests, and lung computed tomography.All six GLILD patients received Rituximab infusions as a first-line treatment. Rituximab was administered at 375 mg/m2 monthly for six infusions followed by maintenance every 3 months; none of the patients experienced severe adverse events. Symptom burden and quality of life significantly improved in treated patients compared to a control group of CVID patients without GLILD. Rituximab treatment indirectly caused a trend toward reduced T-cell activation and exhaustion markers sCD25 and sTIM-3. Lung function improved in treated patients, with statistically significant increases in TLC and DLCO. Lung CT scan findings expressed by means of Baumann scoring system displayed a reduction in the entire cohort.In conclusion, first-line monotherapy with Rituximab displayed high efficacy in disease remission in all treated patients, with improvement of symptoms and amelioration of quality of life, as well as restoration of PFTs and lung CT scan findings.

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Rituximab for granulomatous lymphocytic interstitial lung disease in a patient with common variable immunodeficiency. Is single therapy enough?

Cited by 4 publications

References 8 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

What Works When Treating Granulomatous Disease in Genetically Undefined CVID? A Systematic Review

Rituximab Monotherapy Is Effective as First-Line Treatment for Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in CVID Patients

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