IgG4-related disease with central airway involvement diagnosed by cryobiopsy

A 70-year-old woman, previously treated with triple-inhalation therapy for bronchial asthma, presented to an ophthalmologist with diplopia. She had no history of smoking. MRI revealed an enlarged infraorbital nerve and nodular lesions in the lacrimal gland. Serum IgG4 levels were markedly elevated at 2158 mg/dL. Whole-body CT demonstrated thickened tracheal and bronchial walls, accompanied by multiple localised nodules in both bronchi (figure 1a,b). In addition, swelling of bilateral submandibular glands and pancreatic enlargement were observed. 18F-fluorodeoxyglucose positron emission tomography CT showed increased fluorodeoxyglucose uptake in the enlarged lesions, including the bronchial walls (figure 1c). White-light bronchoscopy (1T260, Olympus) revealed diffuse oedematous narrowing of the trachea and segmental bronchi, with numerous localised elevated nodules (figure 1d–f). Transbronchial biopsy (TBB) was performed using 1.9 mm forceps (FB-231-D, Olympus), and cryobiopsy was concomitantly performed using a 1.7 mm cryoprobe (20 402–410, Erbe Elektromedizin GmbH) targeting elevated nodular lesions. TBB specimens were small and exhibited significant crush artefacts, hindering assessment of inflammatory cell infiltrates (figure 2a, right). In contrast, cryobiopsy specimens were larger, with minimal crush artefacts, and demonstrated subepithelial infiltration of plasma cells and lymphocytes, along with fibrotic …