Bronchial rupture related to endobronchial stenting in relapsing polychondritis.

IgG4-related disease is a relatively newly discovered entity [4, 5]. It is characterised by an inflammatory and fibrosing infiltrate rich in IgG4-positive plasma cells and often elevated IgG4 serum concentrations. The most well-known affected organ is the pancreas, a disease formerly known as auto-immune pancreatitis. However, all internal organs may be affected, for instance the salivary glands (Sjögren-like syndrome), retroperitoneum (retroperitoneal fibrosis), kidney (interstitial nephritis), lymph nodes and the aorta (inflammatory aneurysm) [4]. The aetiology and pathogenesis are not known. Early IgG4-related disease is very steroid responsive with quick and longstanding remissions, and decreases in serum IgG4 levels. In cases with extensive fibrotic lesions, remission after therapy is less likely.