Intravenous Immunoglobulin Monotherapy for Granulomatous Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency

Hasegawa, Mizue; Sakai, Fumikazu; Okabayashi, Asako; Sato, Akitoshi; Yokohori, Naoko; Katsura, Hideki; Asano, Chihiro; Kamata, Toshihide; Koh, Eitetsu; Sekine, Yasuo; Hiroshima, Kenzo; Ogura, Takashi; Takemura, Tamiko

doi:10.2169/internalmedicine.7757-16

Cited by 12 publications

(14 citation statements)

References 15 publications

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“…4,31,[39][40][41] In case reports or studies of small numbers of patients with GLILD, corticosteroids, TNF-a inhibitors, MMF, sirolimus, cyclosporin, rituximab monotherapy, abatacept, and immunoglobulin replacement therapy have all been reported to improve GLILD. 3,[42][43][44][45][46][47][48][49][50] In our cohort, we found that a minimum of 6 months of immunoglobulin replacement therapy did not result in improvement of GLILD. Corticosteroid therapy, which is the most commonly used and recommended first-line therapy, 51 was used in 20 of our patients (51%) before referral to our center and was ineffective in treating GLILD.…”

Section: Discussionmentioning

confidence: 51%

Rituximab and antimetabolite treatment of granulomatous and lymphocytic interstitial lung disease in common variable immunodeficiency

Verbsky

Hintermeyer

Simpson

et al. 2021

Journal of Allergy and Clinical Immunology

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Section: Discussionmentioning

confidence: 51%

Rituximab and antimetabolite treatment of granulomatous and lymphocytic interstitial lung disease in common variable immunodeficiency

Verbsky

Hintermeyer

Simpson

et al. 2021

Journal of Allergy and Clinical Immunology

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“…Development of lymphoproliferative disease in CVID has been suggested to be associated with viral infections [10]. Infectious association potentially explains the response to Ig replacement therapy alone in patient 2 in this series and another reported case [11]. It is unclear if increased doses of Ig, above normal replacement targets, would improve response.…”

Section: Discussionmentioning

confidence: 87%

Granulomatous lymphocytic interstitial lung disease: limiting immunosuppressive therapy—a single‐centre experience

Beaton

Gillis

Morwood

et al. 2020

Respirology Case Reports

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Granulomatous lymphocytic interstitial lung disease (GLILD) is characterized by lymphocytic and granulomatous pulmonary infiltration occurring in common variable immunodeficiency (CVID). It is associated with increased mortality compared with CVID patients without GLILD. There are no treatment guidelines due to the low prevalence and the heterogeneity of the condition. A case review of three patients diagnosed with GLILD was performed from a single Australian centre. Patients met the European Society of Immunodeficiency criteria for CVID and a diagnosis of GLILD was confirmed by a multidisciplinary team. Patients were managed with immunoglobulin (Ig) replacement and immunosuppressive agents if required: the decision for immunosuppression was made on the basis of symptoms and declining pulmonary function. All patients clinically improved. One patient had immunosuppressive treatment ceased. GLILD responds to varying immunosuppressive regimes when IgG monotherapy fails. Immunosuppressive therapy can be discontinued following improvement, but patients require close observation. This series helps inform future GLILD treatment guidelines.

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“…While immunoglobulin replacement may be effective for some patients [35], progression of disease is frequently seen, as in our patient. Moreover, effectiveness of corticosteroids is variable [2••, 15•, 36], often necessitating treatment with additional therapeutic agents.…”

Section: Discussion and Literature Reviewmentioning

confidence: 79%

Granulomatous-Lymphocytic Interstitial Lung Disease in 22q11.2 Deletion Syndrome: a Case Report and Literature Review

Sood

Funkhouser

Handly

et al. 2018

Curr Allergy Asthma Rep

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Purpose of Review Granulomatous-lymphocytic interstitial lung disease (GLILD) has classically been associated with common variable immune deficiency (CVID), but is increasingly being reported in other immunodeficiencies. We describe the second reported case of GLILD in a patient with 22q11.2 deletion syndrome (22q11.2DS) and review the recent literature surrounding GLILD. Recent Findings GLILD is characterized by granulomata and lymphoproliferation. Consensus statements and retrospective and case-control studies have better elucidated the clinicopathological and radiographic manifestations of GLILD, allowing for its differentiation from similar conditions like sarcoidosis. Gaps of knowledge remain, however, particularly regarding optimal management strategies. Combination therapies targeting T and B cell populations have recently shown favorable results. Summary GLILD is associated with poorer outcomes in CVID. Its recognition as a rare complication of 22q11.2DS and other immunodeficiencies therefore has important therapeutic and prognostic implications. Additional research is needed to better understand the natural history and pathogenesis of GLILD and to develop evidence-based practice guidelines.

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Intravenous Immunoglobulin Monotherapy for Granulomatous Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency

Cited by 12 publications

References 15 publications

Rituximab and antimetabolite treatment of granulomatous and lymphocytic interstitial lung disease in common variable immunodeficiency

Rituximab and antimetabolite treatment of granulomatous and lymphocytic interstitial lung disease in common variable immunodeficiency

Granulomatous lymphocytic interstitial lung disease: limiting immunosuppressive therapy—a single‐centre experience

Granulomatous-Lymphocytic Interstitial Lung Disease in 22q11.2 Deletion Syndrome: a Case Report and Literature Review

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