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pp 1–4 | Cite as

Thoracic Hyper-IgG4-Related Disease Mimicking Malignant Pleural Mesothelioma

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

Keywords

IgG4-related disease Pleura Mesothelioma Pleural effusion 

Notes

Compliance with Ethical Standards

Conflict of interest

None.

Ethical Approval

The manuscript was written in compliance with ethical standards.

Informed Consent

A written informed consent has been obtained before publication.

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Filippo Lococo
    • 1
    Email author
  • Teresa Di Stefano
    • 1
  • Cristian Rapicetta
    • 1
  • Roberto Piro
    • 2
  • Maria Carolina Gelli
    • 3
  • Francesco Muratore
    • 4
  • Tommaso Ricchetti
    • 1
  • Sofia Taddei
    • 2
  • Maurizio Zizzo
    • 5
  • Alfredo Cesario
    • 6
  • Nicola Facciolongo
    • 2
  • Massimiliano Paci
    • 1
  1. 1.Unit of Thoracic SurgeryAzienda Unità Sanitaria Locale-IRCCS di Reggio EmiliaReggio EmiliaItaly
  2. 2.Pulmonology UnitAzienda Unità Sanitaria Locale-IRCCS di Reggio EmiliaReggio EmiliaItaly
  3. 3.Unit of PathologyAzienda Unità Sanitaria Locale-IRCCS di Reggio EmiliaReggio EmiliaItaly
  4. 4.Unit of ReumatologyAzienda Unità Sanitaria Locale-IRCCS di Reggio EmiliaReggio EmiliaItaly
  5. 5.Unit of Surgical OncologyAzienda Unità Sanitaria Locale-IRCCS di Reggio EmiliaReggio EmiliaItaly
  6. 6.Direzione Scientifica, Fondazione Policlinico Universitario A. Gemelli-IRCCSRomeItaly

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