RESUMO
BACKGROUND: IgG4-related disease (IgG4-RD) is a chronic fibroinflammatory condition with multiple-organ involvement. Rupture of coronary artery aneurysms (CAAs) in IgG4-RD is rare. CASE SUMMARY: A 65-year-old man with IgG4-RD has suffered from recurrent episodes of arterial aneurysms since 2003. He presented with chest pain and hypotension caused by localized cardiac tamponade at right ventricle free wall due to the rupture of coronary artery aneurysm (CAA) of left anterior descending artery (LAD). An urgent LAD aneurysm repaired with bovine pericardium and obliterated aneurysmal sac with cryo-acrylate glue was done together with coronary artery bypass grafting (CABG) using saphenous vein graft (SVG) to LAD and SVG to posterior descending artery. Three-month after surgery, the follow-up coronary computed tomography angiography (CCTA) revealed a growing in size of LAD and the second obtuse marginal (OM) branch aneurysm. Heart team discussion agreed to schedule the patient for double coil embolization to LAD and second OM aneurysm under intravascular ultrasound guidance. Both aneurysms were successfully obliterated with vascular coils. Two-week follow-up coronary angiogram showed complete occlusion of LAD aneurysm and near occlusion of the second OM branch aneurysm. DISCUSSION: Coronary artery aneurysm rupture is a life-threatening condition that required prompt detection and treatments. In IgG4-RD patients, acute cardiac tamponade suggesting the rupture of CAA. Coil embolization is an alternative treatment in patients who suffered from recurrent CAA after surgical repair. Serial CCTA is important for early detection of aneurysm in IgG4-RD patients who had vascular involvement.
RESUMO
OBJECTIVES: We investigated the incidence, predictors, and survival of pulmonary hypertension (PH) determined by Doppler echocardiography in Thai patients with early SSc (systemic sclerosis), in which the majority were diffuse cutaneous SSc (DcSSc) with anti-topoisomerase I-positive. METHODS: We used an inception cohort study of patients with early SSc seen at the Rheumatology Clinic, Maharaj Nakorn Chiang Mai Hospital. All patients were assessed for clinical data and underwent Doppler echocardiography at the study entry and then annually. RESULTS: A total of 133 patients (81 female, 106 DcSSc, 103 anti-topoisomerase I-positive) with a mean disease duration of 11.9 months were recruited. During the mean observational period of 4.2 years, 14 patients developed PH concurrent with ILD and 6 with left heart disease. The incidence rate for the development of PH was 3.95 per 100 person years. The multivariate Cox regression analysis indicated higher NYHA class (HR 6.90, 95% CI 2.28-20.94, p = 0.001), telangiectasia (HR 4.18, 95% CI 1.25-13.92, p = 0.020), and enlarged LA diameter (HR 1.16, 95% CI 1.05-1.28, p = 0.005) as predictors of PH. Raynaud's phenomenon (HR 0.22, 95% CI 0.06-0.84, p = 0.026) and high oxygen saturation (HR 0.80, 95% CI 0.65-0.99, p = 0.047) were protective factors. The survival rate after PH diagnosis at 1, 3, and 5 years were 88.9%, 82.3%, and 48.0%, respectively. CONCLUSIONS: In this study cohort, the majority had early DcSSc, the incidence of PH was modest, and all cases developed concomitantly with ILD or left heart disease, resulting in poor survival. The presence of higher NYHA class, telangiectasia, and enlarged LA diameter was predictors of secondary PH. Further study regarding the treatment strategies for PH associated with ILD and left heart disease in SSc is needed. Key Points ⢠In this cohort of early SSc in which the main subtype was DcSSc, the incidence of PH was modest and all PH was secondary PH associated with ILD and left heart disease, resulting in poor survival. ⢠The presence of higher NYHA functional class, telangiectasia, and enlarged LA diameter was baseline predictors of developing secondary PH. ⢠Effective treatment strategies for secondary PH due to ILD and cardiac involvement in SSc patients are urgently needed.