Extranodal marginal zone B-cell lymphoma of the lung in Sjögren's syndrome patients: Reappraisal of clinical, radiological, and pathology findings

Papiris, Spyros; Kalomenidis, Ioannis; Malagari, Katerina; Kapotsis, G.E.; Harhalakis, Nikolaos; Manali, Effrosyni D.; Rontogianni, Dimitra; Roussos, Charis; Moutsopoulos, Haralampos Μ.

doi:10.1016/j.rmed.2006.04.005

Cited by 42 publications

(37 citation statements)

References 40 publications

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“…The youngest patient was aged 60 years, and the oldest was aged 71 years. Female patients predominated in this and in most other reported studies 7,9 with a male to female ratio of 1:6. All patients in this series were Caucasian.…”

Section: Discussionsupporting

confidence: 64%

“…13 MZLs of MALT-type of the lung have been associated with underlying Helicobacter pylori infection, human immunodeficiency virus infection, common variable immunodeficiency, chronic hepatitis C, and Borrelia burgdorferi infection, as well as autoimmune diseases. 9 In our series, out of 13 patients with pulmonary MALT lymphoma, 7 (54 %) met the criteria for SjÖgren's syndrome. Similar to previous published reports, 7,14 the mean age at lymphoma diagnosis in this study was 66 years.…”

Section: Discussionmentioning

confidence: 55%

“…The involvement of the lungs by MALT lymphoma in patients with pSS has been reported only briefly in the literature. 9,10 According to the latest World Health Organization (WHO) lymphoma classification scheme, MALT lymphomas are considered a subtype of B-cell marginal zone lymphoma (MZL). 11 The presence of MALT in the lung was first described by Bienenstock and colleagues in 1973 as peribronchiolar lymphoid aggregates bearing a close similarity to Peyer's patches.…”

Section: Discussionmentioning

confidence: 99%

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Pulmonary MALT Lymphoma in Patients with Sjögren’s Syndrome

et al. 2017

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Original ResearchTo describe clinical features and outcomes of seven patients with pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma in the setting of underlying primary Sjögren's syndrome from a single center, we reviewed medical records of consecutive patients with pulmonary MALT lymphoma evaluated at our facility from January 1, 1999 to December 31, 2015 for clinical features, laboratory, pathologic and radiographic findings, management, and outcomes. Out of 13 patients with pulmonary MALT lymphoma, 7 (54 %) met the criteria for Sjögren's syndrome. The mean age at lymphoma diagnosis was 66 years; male-female ratio was 1:6. One-third of patients were asymptomatic at the time lymphoma was discovered. When symptomatic, patients reported nonspecific pulmonary complaints such as cough and dyspnea. All patients had positive antinuclear antibody and anti-SSA/Ro antibody. Rheumatoid factor was positive in six cases. A monoclonal gammopathy was present in three patients; the remaining four had polyclonal hypergammaglobulinemia. The radiologic, morphologic, and immunohistochemical features of primary Sjögren's syndrome-associated pulmonary MALT lymphomas did not differ significantly from pulmonary MALT lymphoma cases in general. All treatment modalities used resulted in complete and sustained response. One patient died 11 years after initial diagnosis with no lymphoma but of another cause. The remaining six patients are still alive and disease-free to date. The present series confirms the favorable course of pulmonary MALT lymphoma in Sjögren's patients. The overall imaging and pathologic features are in accordance with pulmonary MALT lymphoma not associated with primary Sjögren's syndrome. Further studies should be carried out in order to better understand pulmonary MALT lymphomagenesis, treatment, and outcomes in Sjögren's patients.

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Section: Discussionsupporting

confidence: 64%

Section: Discussionmentioning

confidence: 55%

Section: Discussionmentioning

confidence: 99%

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Pulmonary MALT Lymphoma in Patients with Sjögren’s Syndrome

et al. 2017

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“…The inflammatory infiltrate described above may become severe enough to sovereign structure and function of the exocrine glands. B lymphocyte hyperactivity may also lead to several lymphoproliferative manifestations including lymphoma development in 2-5% of patients [202][203][204] . Sjögren's syndrome may occur alone, as pSs or as secondary in association with another CTD, mainly RA and SLE nonobeying temporal rules [198] .…”

Section: Primary Sjögren's Syndromementioning

confidence: 99%

“…Airway manifestations in pSs are not per se disease expressions but probably part of an evolving process through several lymphoproliferative disorders from peribronchiolar BALT hyperplasia (follicular bronchiolitis) towards diffuse alveolar interstitial LIP characterized by the alveolar tissue infiltration of a polyclonal population of B lymphocytes and finally to the emergence of a monoclonal B lymphocytic population such as in the development of a marginal zone B-cell non-Hodgkin's lymphoma or other more aggressive lymphomas [204] ( fig. 12 a-d).…”

Section: Lymphoproliferative Manifestationsmentioning

confidence: 99%

Investigation of Lung Involvement in Connective Tissue Disorders

Papiris¹,

Manali²,

Kolilekas³

et al. 2015

Respiration

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Lung involvement in connective tissue disorders (CTDs) may present as pleomorphic since any lung compartment may be involved such as airways, exocrine secretory and alveolar epithelia, interstitial lung structure, pulmonary vasculature and pleura as well as, in specific disorders, several tissues of the thoracoabdominal ventilator pump. Any combination of the above anatomic structures may be involved concomitantly although some specific combinations may include a determinant of rheumatic disorders. The diagnosis of a specific CTD requires the fulfilment of clearly defined clinical and laboratory criteria including in most cases positivity for autoantibodies, mostly specific serologic combinations. In this setting, serologic investigation targets mainly, although not exclusively, the detection of antinuclear antibodies. A specific serologic positivity or a combination of autoantibodies constitutes not only a diagnostic criterion for a specific CTD, but may also characterize the pattern of respiratory manifestation in a determinant rheumatic disorder. Therefore, the investigation of lung involvement in CTDs requires adequate skills in the ambit of a multidisciplinary approach and an extended spectrum of diagnostic tools and modalities able to detect both early clinical clues and serologic conversion as well as any pathophysiologic alteration that regards the complexity of respiratory functional status. Although many patients with CTDs suffer from a ‘vicious' combination of lung involvement, lung drug toxicity and infections related to the above two as well as to the ‘mater' disease, for space reasons this review will focus on the established lung manifestations that regard the 7 major CTDs.

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