Common variable immunodeficiency-associated granulomatous and interstitial lung disease

Good's syndrome is a rare primary immunodeficiency associated with adult thymoma. Complications are mainly autoimmune manifestations and recurrent infections with encapsulated bacteria. Only one possible case of combined granulomatous-lymphocytic interstitial lung disease (GL-ILD) and Good's syndrome have been described earlier, but the patient died at the time of diagnosis. This is the first case of GL-ILD in Good's syndrome with a successful outcome. We present a case of a 43-year-old man with GL-ILD, who suffered from recurrent infections of Haemophilus influenzae and Pneumocystis jirovecii, with 8-year follow-up. After a thymectomy, he was diagnosed with Good's syndrome and GL-ILD. He was treated with prophylactic pivampicillin, quinolones and cephalosporins for his recurrent P. jirovecii and H. influenzae infections, an approach that proved unsuccessful due to resistance, with relapse after cessation. He was stabilised with oral diaminodiphenyl-sulfone for P. jirovecii and colistimethate-sodium inhalations for H. influenzae, which is a new approach to prophylactic treatment.

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“…Similarly, it has been recognised that early diagnosis of GL-ILD in CVID is essential for improved morbidity and survival 8 9…”

Section: Discussionmentioning

confidence: 99%

Granulomatous-lymphocytic interstitial lung disease and recurrent sinopulmonary infections in a patient with Good’s syndrome

2015

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“…It is the most common serious primary immunodeficiency with a prevalence of 1: 25000 – 50000 [1]. In a small minority of patients, single gene mutations resulting in B and T lymphocyte dysfunction have been identified [2].…”

Section: Introductionmentioning

confidence: 99%

“…The differential diagnosis of DLPD in the context of CVID is diverse and includes infection, hypersensitivity pneumonitis, cryptogenic organizing pneumonia, lymphoma and a constellation of radiological-pathological findings known as granulomatous and lymphocytic interstitial lung disease (GLILD) [1,8,9]. Although GLILD is the most common cause of DLPD in CVID, the histopathological features of this disorder are incompletely characterized.…”

Section: Introductionmentioning

confidence: 99%

Granulomatous and lymphocytic interstitial lung disease: a spectrum of pulmonary histopathologic lesions in common variable immunodeficiency—histologic and immunohistochemical analyses of 16 cases

Rao¹,

Mackinnon²,

Routes³

2015

Human Pathology

113

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Common Variable Immunodeficiency (CVID) is a primary immunodeficiency of unknown etiology characterized by low serum IgG, a decreased ability to make specific antibodies, and variable T cell defects. Approximately 20% of patients with CVID develop clinical evidence of a diffuse parenchymal lung disease with a constellation of histopathological findings termed granulomatous and lymphocytic interstitial lung disease (GLILD). In this study, we characterized the histological and immunohistochemical features in a series of 16 cases diagnosed by open lung biopsy. Peribronchiolar and interstitial lymphocytic infiltration, granulomatous inflammation and organizing pneumonia were consistent features; interstitial fibrosis with architectural remodeling was also found in a subgroup of patients. By immunohistochemistry, a predominance of CD4+ T lymphocytes with variable numbers of CD8+ T cells and B cells were present, with a striking absence of FOXP3 positive T regulatory cells. This heretofore unrecognized immunohistochemical finding, needs further investigation for a potential role in the pathogenesis of the condition. The presence of interstitial fibrosis with or without architectural remodeling in a subset of patients also needs additional study, for effect on prognosis.

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“…13 Similarly, human herpesvirus-8 was associated with GLILD in one study, 28 though this has not yet been confirmed. Non-infectious pathogenic mechanisms for the development of CVID lung disease have also been proposed, including aberrant B cell lymphoproliferation 29 and T cell-driven autoimmunity. 14 Through retrospective chart review, we found bronchiectasis to be associated with history of pneumonia and reduced CD4+ T cells in CVID.…”

Section: Introductionmentioning

confidence: 99%

Pulmonary radiologic findings in common variable immunodeficiency: clinical and immunological correlations

Maglione

Overbey

Radigan

et al. 2014

Annals of Allergy, Asthma & Immunology

104

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Common variable immunodeficiency-associated granulomatous and interstitial lung disease

Cited by 60 publications

References 28 publications

Granulomatous-lymphocytic interstitial lung disease and recurrent sinopulmonary infections in a patient with Good’s syndrome

Granulomatous-lymphocytic interstitial lung disease and recurrent sinopulmonary infections in a patient with Good’s syndrome

Granulomatous and lymphocytic interstitial lung disease: a spectrum of pulmonary histopathologic lesions in common variable immunodeficiency—histologic and immunohistochemical analyses of 16 cases

Pulmonary radiologic findings in common variable immunodeficiency: clinical and immunological correlations

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