Abstract Introduction Pulmonary hypertension (PH) is associated with several autoimmune disorders including systemic sclerosis, rheumatoid arthritis, lupus, Sjogren’s Disease, and mixed connective tissue disease but rarely are these conditions associated with a specific identifiable environmental trigger. Here we present a case of PH due to an autoimmune/inflammatory reaction triggered by illicit gluteal injections. Description A 48-year-old woman with chronic kidney disease and recurrent nephrolithiasis presented to the pulmonary vascular disease clinic for evaluation of several years of progressive exertional dyspnea, presyncope, and an echocardiogram suggestive of PH. She revealed a history of receiving unregulated cosmetic gluteal injections containing a nondescript substance 20 years prior. She exhibited Raynaud’s phenomenon and abnormal nailfold capillaroscopy. RHC showed features consistent with precapillary PH (Table 1). Ventilation-perfusion scan was not consistent with chronic pulmonary thromboembolic disease. Serologic workup was notable for positive ANA (1:1280 speckled), RF (32.2 IU/mL), and SS-A ( 8.0 AI) with negative centromere and SCL-70 antibodies. ACE levels (273 U/L), 1,25-dihydroxy vitamin D (175.0 pg/mL), and calcium were elevated while PTH was normal. She started inhaled treprostinil, ambrisentan, and tadalafil with improvement in her hypoxemia and exertional dyspnea. She later underwent a kidney biopsy which showed glomeruli and peritubular capillaries with distension of the capillary lumina with empty vacuoles and a weakly positive oil red stain. Overall findings seemed suspicious for migration of foreign injected material into the renal vascular spaces. Biopsy of the skin of her gluteal region showed dense fibrosis and similarly empty vacuoles. No granulomatous inflammation was noted. Given her exposure to silicone and other adjuvants with clinical features of scleroderma, we felt that this was consistent with a novel presentation of Autoimmune/Inflammatory Syndrome Induced by Adjuvants (ASIA syndrome) with pre-capillary PH. Discussion ASIA syndrome is an immune-mediated disorder that occurs in genetically susceptible patients due to exposure to environmental immunogenic stimuli. We describe a novel case of silicone injections leading to a multisystem inflammatory disorder and to the development of ASIA syndrome. Whether embolization of silicone and other adjuvants to the pulmonary vasculature was contributory in this case remains unknown as lung biopsy was not feasible Silicone embolization and deposit in lung tissue, including alveolar macrophages in BAL, have been previously described. ASIA syndrome characteristically improves with the removal of adjuvants. Treatment of associated precapillary PH should be undertaken if the pulmonary arteriopathy seems out of proportion to lung disease, and response to vasodilators should be carefully evaluated. This abstract is funded by: NIDA grant # R25 DA020537
Olsen et al. (Fri,) studied this question.