Immunoglobulin G4-related disease (IgG4-RD) is now a widely recognised multi-organ system disease characterised by elevated serum and tissue concentrations of IgG4. Two forms of thyroid involvement in IgG4-RD have been described, including Reidels thyroiditis (IgG4-related thyroid disease) and the fibrous variant of Hashimotos thyroiditis.
Objective: To describe a case of the development of massive neck fibrosis and mediastinal fibrosis in a patient with the fibrous variant Hashimotos thyroiditis.
Methods and case presentation: In January 2012, a 59-year-old woman with a 1-year history of subclinical hypothyroidism due to Hashimotos autoimmune thyroiditis was admitted to our department, presenting with a swelling in the anterior region of the neck, dyspnea, dysphagia, fatigue. The tumor progressed quickly to a ecklarge, stony hard mass on the anterior neck region. Analysis: TSH, 0.42 mU/ml, FT4, 13.90 pmol/l; AbTPO, 133.92 UI/ml, erythrocyte sedimentation rate (ESR), 58 mm/h; Echo-guided FNAB confirmed the diagnosis of Hashimoto thyroiditis and neck fibrosis. A computed tomography (CT) revealed a fibrotic mass located at the anterior-inferior aspect of the neck and mediastinum with displacement of the tracheal lumen and stenosis of oesophagus. We started therapy with prednisolone 60 mg daily and L-thyroxine substitution therapy. The follow-up lasted for 12 months. After 1 month of corticosteroid therapy, the patient had no compression symptoms. The treatment with prednisolone led to a significant subjective improvement and objective changes (a significant decrease of neck and mediastinal fibrosis), confirmed by regular clinical examinations, ultrasonography and computed tomography of the neck and mediastinum. Now the patient is in good health, undergoing a therapy by prednisolone 5 mg daily.
Conclusion: We have reported an unusual case of development of the massive neck fibrosis and mediastinal fibrosis in a patient with Hashimotos thyroiditis. which showed good response to steroid treatment. It is important for the physician to recognize the presence of mediastinal fibrosis in the presence of fibrous variant of Hashimotos Thyroiditis and initiate steroid therapy for resolution of both diseases.
27 Apr - 01 May 2013
European Society of Endocrinology