Relapsing polychondritis: What's new in 2017?
[Relapsing polychondritis: What's new in 2017?] Rev Med Interne. 2017 Sep 07;: Authors: Dion J, Leroux G, Mouthon L, Piette JC, Costedoat-Chalumeau N Abstract Relapsing polychondritis (RP) is a rare condition characterized by recurrent inflammation of cartilaginous tissue and systemic manifestations. Data on pathophysiology are scarce and suggest an autoimmune mechanism. Recently, the possibility of dividing patients with RP into three distinct clinical phenotypes has been suggested: the hematological form representing less than 10% of patients, essentially older men with associated myelodysplasia and poor prognosis, the respiratory form representing about 25% of patients with predominant tracheobronchial involvement, and the mild and most frequent form, representing 65% of patients, with a good prognosis. Recent data on survival shows an improvement of overall prognosis compared to historical series. Reported poor prognosis factors are male gender, associated haemopathies and cardiac involvement. Few recent series suggest an interest for positron emission tomography for the diagnosis and the follow-up of treatment. Due to the lack of randomized therapeutic trial, treatment remains empirical and is mainly based on oral corticosteroids sometimes associated with immunosuppressive agents. The use of biologic agents has recently been reported in small retrospective series with different outcome. Finally, some selected patients with mild and occasional periphe...
Abstract A 78-year-old man had fever, persistent wheezing, and serum C-reactive protein elevation. Malignant lymphoma was suspected because of mediastinal lymph nodes swelling on CT and soluble interleukin 2 receptor elevation. Symmetric 18F-FDG uptake in the tracheobronchial tree and bilateral auricles was observed on PET/CT. He was finally diagnosed as having relapsing polychondritis by auricular cartilage biopsy. 18F-FDG PET/CT may have crucial role in evaluating the extent of inflammation and deciding the biopsy site of relapsing polychondritis.
This report provides new data regarding the diagnostic difficulties of this rare type of autoimmune disease, and further investigations are needed as cases accumulate.
Relapsing polychondritis is a rare disease of unknown etiology characterized by recurrent inflammation involving multiple cartilaginous structures throughout the body. A handful of reports have suggested a possible connection of IgG4-related disease with the pathogenesis of relapsing polychondritis. IgG4-related disease is also an uncommon immune-mediated systemic inflammation disorder that can involve the pancreas, kidneys, salivary glands, aorta, orbits, and skin, among others. The hallmark of this disease is a lymphoplasmacytic infiltrate enriched in IgG4-positive plasma cells and fibrosis in involved tissues.
Relapsing polychondritis is an autoimmune disorder characterized by inflammation of cartilaginous structures, predominantly of the ears, nose, and laryngo-tracheobronchial tree. Dermatological manifestations may be the initial presentation in about 12% of cases, with urticarial lesions occurring in 5-7% of the patients.
We describe a case of a woman with RP, Rheumatoid arthritis, Celiac disease, selective IgA deficiency, recurrent infections and asthma.
We report the case of a woman with a corticosteroid-resistant RP-associated aortitis, who was successfully treated with tocilizumab (TCZ). The FDG-PET/CT was a useful tool for diagnosing aortitis and assessing the effect of biotherapy. We conducted a systematic literature review confirming this is the first case of rapid and sustained remission in a patient with corticosteroid-resistant RP-associated aortitis after TCZ treatment administered as a first-line immunotherapy. However, further studies are needed to confirm the beneficial effect of TCZ used in this life-threatening condition.