Deconstructing IgG4-related disease involvement of midline structures: Comparison to common mimickers.

Objective: A series of destructive and tumefactive lesions of the midline structures have been recently added to the spectrum of IgG4-related disease (IgG4-RD). We examined the clinical, serological, endoscopic, radiological, and histological features that might be of utility in distinguishing IgG4-RD from other forms of inflammatory conditions with the potential to involve the sinonasal area and the oral cavity.

Methods: We studied 11 consecutive patients with erosive and/or tumefactive lesions of the midline structures referred to our tertiary care center.

IgG4-related disease in Italy: clinical features and outcomes of a large cohort of patients.

Objectives: To describe the clinical features, treatment response, and follow-up of a large cohort of Italian patients with immunoglobulin (Ig)G4-related disease (IgG4-RD) referred to a single tertiary care centre.

Method: Clinical, laboratory, histological, and imaging features were retrospectively reviewed. IgG4-RD was classified as 'definite' or 'possible' according to international consensus guidelines and comprehensive diagnostic criteria for IgG4-RD.

Methotrexate in refractory bilateral juvenile temporal arteritis: Report of a case.

Juvenile temporal arteritis is a rare inflammatory disease of the temporal arteries that affects young adults. The clinical course is benign and the surgical excision of the affected artery is usually curative. Here we report a case of bilateral juvenile temporal arteritis with significant peripheral eosinophilia and elevated IgE, refractory to surgical excision and even to a short course of corticosteroids.