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Peer-reviewedCC BYBMC pulmonary medicine

IgG4-related lung disease and seropositive undifferentiated arthritis in a patient initially presenting with monoarthritis: a case report.

  1. 1Department of Radiology, Beilun Branch of the First Affiliated Hospital, School of Medicine, Zhejiang University, Ningbo, 315800, China.
  2. 2Department of Radiology, The First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, 310003, China. [email protected].

Abstract

Background

IgG4-related lung disease (IgG4-RLD) is a fibroinflammatory pulmonary manifestation of IgG4-related disease (IgG4-RD). Its comorbidity with seropositive undifferentiated arthritis (SUA) is rare and may be overlooked due to overlapping clinical and immunological features.Case presentation

A 71-year-old man with acute left knee pain secondary to a meniscal tear with synovitis, as confirmed by MRI, was scheduled for arthroscopy. A preoperative chest radiograph incidentally revealed diffuse right lung lesions.Diagnostic workup and therapeutic strategy

Laboratory studies showed elevated rheumatoid factor (RF) and anti-cyclic citrullinated peptide (anti-CCP) antibodies. The first multidisciplinary team (MDT) discussion established a diagnosis of SUA and initiated disease-modifying antirheumatic drug (DMARD) therapy, which resolved the arthralgia but did not improve the lung lesions. A subsequent MDT review, incorporating chest CT findings (thickened bronchovascular bundles and consolidation) and lung biopsy results, confirmed the diagnosis of IgG4-RLD. Treatment was then adjusted to high-dose glucocorticoids combined with DMARDs. Both pulmonary and articular manifestations showed significant remission.Conclusion

This rare case of IgG4-RLD complicating SUA highlights the importance of considering IgG4-RLD in patients with seropositive inflammatory arthritis presenting with atypical pulmonary imaging findings, particularly when lung lesions fail to respond to conventional DMARD therapy. MDT collaboration is essential for accurate diagnosis and optimal management of these concomitant autoimmune conditions.

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BibTeX
@article{peng2026igg4related,
  title = {{IgG4-related lung disease and seropositive undifferentiated arthritis in a patient initially presenting with monoarthritis: a case report.}},
  author = {Wenwen Peng and Tiefeng Li and Lun Zhang and Hanguang Dai and Qianqian Zhong and Yilei Zhao},
  journal = {BMC pulmonary medicine},
  year = {2026},
  month = aug,
  doi = {10.1186/s12890-026-04565-9},
  url = {https://europepmc.org/article/MED/42972335},
  note = {CC BY},
}
RIS
TY  - JOUR
TI  - IgG4-related lung disease and seropositive undifferentiated arthritis in a patient initially presenting with monoarthritis: a case report.
AU  - Wenwen Peng
AU  - Tiefeng Li
AU  - Lun Zhang
AU  - Hanguang Dai
AU  - Qianqian Zhong
AU  - Yilei Zhao
PY  - 2026
DA  - 2026/08/06
JO  - BMC pulmonary medicine
DO  - 10.1186/s12890-026-04565-9
UR  - https://europepmc.org/article/MED/42972335
AB  - Background  IgG4-related lung disease (IgG4-RLD) is a fibroinflammatory pulmonary manifestation of IgG4-related disease (IgG4-RD). Its comorbidity with seropositive undifferentiated arthritis (SUA) is rare and may be overlooked due to overlapping clinical and immunological features.Case presentation  A 71-year-old man with acute left knee pain secondary to a meniscal tear with synovitis, as confirmed by MRI, was scheduled for arthroscopy. A preoperative chest radiograph incidentally revealed diffuse right lung lesions.Diagnostic workup and therapeutic strategy  Laboratory studies showed elevated rheumatoid factor (RF) and anti-cyclic citrullinated peptide (anti-CCP) antibodies. The first multidisciplinary team (MDT) discussion established a diagnosis of SUA and initiated disease-modifying antirheumatic drug (DMARD) therapy, which resolved the arthralgia but did not improve the lung lesions. A subsequent MDT review, incorporating chest CT findings (thickened bronchovascular bundles and consolidation) and lung biopsy results, confirmed the diagnosis of IgG4-RLD. Treatment was then adjusted to high-dose glucocorticoids combined with DMARDs. Both pulmonary and articular manifestations showed significant remission.Conclusion  This rare case of IgG4-RLD complicating SUA highlights the importance of considering IgG4-RLD in patients with seropositive inflammatory arthritis presenting with atypical pulmonary imaging findings, particularly when lung lesions fail to respond to conventional DMARD therapy. MDT collaboration is essential for accurate diagnosis and optimal management of these concomitant autoimmune conditions.
SN  - 1471-2466
ER  -