IgG4-related disease in pulmonary arterial hypertension on long-term epoprostenol treatment
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چکیده
IgG4-related disease (IgG4-RD) is a recently described systemic fibro-inflammatory condition characterised by tumefactive lesions and dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells [1]. IgG4RD has been described in a variety of organ systems, including the lacrimal gland, salivary gland, lungs, pancreas and kidneys, and often responds favourably to corticosteroids. The pathophysiological mechanisms for IgG4-RD remain unclear, but type 2 T-helper (Th2) cells are thought to play a critical role [2, 3]. We recently encountered a patient who developed IgG4-RD during the course of idiopathic pulmonary arterial hypertension (PAH). After this experience, we carefully examined PAH patients for IgG4-RD and found four additional cases.
منابع مشابه
IgG4-related disease in pulmonary arterial hypertension on long-term epoprostenol treatment.
IgG4-related disease (IgG4-RD) is a recently described systemic fibro-inflammatory condition characterised by tumefactive lesions and dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells [1]. IgG4RD has been described in a variety of organ systems, including the lacrimal gland, salivary gland, lungs, pancreas and kidneys, and often responds favourably to corticosteroids. The p...
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