Recurrent spontaneous hemothorax: a rare manifestation of I
Immunoglobulin G4-related disease (IgG4-RD) encompasses a group of immune-mediated disorders that are gaining increasing recognition. There have been no reports of IgG4-RD that presents as spontaneous hemothorax. One such case has been reported in the journal BMC Pulmonary Medicine.

A 61-year-old woman experienced recurrent right-sided chest pain and transient syncope. A significant decrease in her hemoglobin level and thick bloody pleural fluid demonstrated spontaneous hemothorax.

The elevated serum IgG4 and histopathological analysis of the right pleura and intercostal lymph node specimens all supported the diagnosis of IgG4-RD in this patient.

Further diagnostic evaluation did not reveal other causes for spontaneous hemothorax. She received steroids and no recurrent bleeding event occurred during a follow-up period of more than 1 year.

Case highlights:-
- The authors reported a very rare case of IgG4-RD, adding recurrent spontaneous hemothorax as a novel manifestation of this uncommon disease.

- The most probable mechanism might be pleural involvement by IgG4-RD. Because hemothorax can be lethal, this peculiar presentation in IgG4-RD is worthy of attention.

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