TY - JOUR T1 - IgG4-related disease in pulmonary arterial hypertension on long-term epoprostenol treatment JF - European Respiratory Journal JO - Eur Respir J SP - 1516 LP - 1519 DO - 10.1183/09031936.00144013 VL - 43 IS - 5 AU - Yuichiro Shirai AU - Yuichi Tamura AU - Hidekata Yasuoka AU - Toru Satoh AU - Masataka Kuwana Y1 - 2014/05/01 UR - http://erj.ersjournals.com/content/43/5/1516.abstract N2 - To the Editor: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]. IgG4-RD 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.The index case patient was a 19-year-old female who presented with progressive exertional dyspnoea corresponding to World Health Organization functional class (WHO-FC) II in March 2000. She was referred to Keio University Hospital (Tokyo, Japan), where complete pulmonary hypertension (PH) evaluations were conducted. Right heart catheterisation revealed an elevated mean pulmonary arterial pressure (mPAP) of 67 mmHg and a normal pulmonary arterial wedge pressure of 7 mmHg. There was no condition or underlying disease that accounted for her PH, leading to the diagnosis of idiopathic PAH. She was initially treated with warfarin and nifedipine, and an oral prostacyclin analogue, beraprost, was added in January 2002. 1 year later, her dyspnoea had deteriorated (to WHO-FC III), with marked elevation of the mPAP (to 95 mmHg). Beraprost was replaced by epoprostenol, and the dosage was gradually increased to 57 ng·kg−1·min−1, resulting in improved symptoms and haemodynamic parameters (mPAP 47 mmHg).In January 2007, the patient developed chronic sinusitis. Simultaneously, she noticed a gradual enlargement of bilateral lacrimal, parotid and submandibular glands, and of minor salivary glands of the lips, in conjunction with intermittent upper abdominal pain … ER -