Thoracic Hyper-IgG4-Related Disease Mimicking Malignant Pleural Mesothelioma

Filippo Lococo, Teresa Di Stefano, Cristian Rapicetta, Roberto Piro, Maria Carolina Gelli, Francesco Muratore, Tommaso Ricchetti, Sofia Taddei, Maurizio Zizzo, Alfredo Cesario, Nicola Facciolongo, Massimiliano Paci

Research output: Contribution to journalArticle

Abstract

We report a rare case of a IgG4-related disease presenting with recurrent pleural effusion, pleural thickness and multiple mediastinal lymphadenopathies and no involvement of other extrathoracic organs. A 65-year-old man with a previous asbestos exposure presented with cough and pain discomfort. A large right pleural effusion was detected and evacuated (siero-haematic liquid). With the suspicious of a pleural mesothelioma, a CT-scan before and a 18F -FDG PET/CT-scan later were performed revealing multiple pleural thickenings and multiple mediastinal lymphadenopathies with radiotracer uptake. EBUS-TBNA EBUS-TBNA did not result in a formal pathological diagnosis; thus, multiple pleural biopsy were performed via right thoracoscopy. At pathology the pleura was markedly thickened by a chronic fibroinflammatory process with scattered lymphoid follicles and a large number of mature plasma cells. Immunohistochemistry shows a mixed B (CD20+) and T (CD3+) population of lymphocytes, without light chain restriction and an increased number of IgG4-positive plasma cells. A presumptive diagnosis of IgG4-related disease was formulated. Total body CT-scan excluded other organ involvement. Blood test showed elevated serum IgG4 concentrations (253 mg/dL) and mild elevation of acute-phase reactants (C-reactive protein 10.7 mg/L). Autoimmune profile was negative. A diagnosis of definite IgG4-related disease was made, and treatment with prednisone 50 mg/day was started.

Original languageEnglish
Pages (from-to)387-390
Number of pages4
JournalLung
Volume197
Issue number3
DOIs
Publication statusPublished - Jun 1 2019

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Keywords

  • IgG4-related disease
  • Mesothelioma
  • Pleura
  • Pleural effusion

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

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