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Objectives To assess the prevalence of avascular necrosis (AVN) in a large cohort of patients with idiopathic inflammatory myopathies (IIM) and define the major associated risk factors. Methods We retrospectively reviewed the electronic medical records of all patients with a definitive diagnosis of IIM enrolled in our registry between 2003 and 2017, and followed until 2020. Pertinent demographic, clinical, serologic and imaging data were collected. A matched group of patients without AVN was then selected for comparison. Results A total of 1680 patients were diagnosed with IIM. Fifty-one patients developed AVN, with an overall prevalence of 3%. Musculoskeletal MRI was available for 1085 patients and AVN was present in 46 patients (43 lower extremities and 3 upper extremities MRI studies), with a relative prevalence of 4.2%. Most patients with AVN were Caucasian females (57%) with a mean (s.d.) age at diagnosis of 44.5 (12.4) years. Sixty-one percent had DM and 29% had PM. The median time from onset of IIM to diagnosis of AVN was 46 months. The hip joint was most commonly involved in 76% of cases, followed by the knee joint in 15% and shoulder joint in 9%. Some 81% of patients were asymptomatic. Established risk factors for AVN were not found to be associated with the development of AVN in IIM patients. Conclusion Although mostly asymptomatic and incidental, the overall prevalence of AVN in IIM was 3% and the prevalence by MRI was 4.2%. None of the established risk factors was found to be associated with AVN development.

Gout is a metabolic disorder of purine metabolism that results in crystallization of uric acid in the form of monosodium urate crystals, affects 8.3 million Americans and is the most common cause of inflammatory arthritis in adults. Urate lowering therapy is the mainstay of treatment for chronic gout. Initial treatments of choice in gout include allopurinol, a purine analog which inhibits xanthine oxidase and decreases the production of uric acid as well as probenecid which increases the urinary excretion of uric acid. However, 3% of patients will fail these treatments. In 2010, pegloticase, a recombinant urate oxidase conjugated to polyethylene glycol, was approved for these patients. Pegloticase has been shown to rapidly normalize plasma uric acid values, resolve tophi and improve quality of life in these patients. Hereby we present a case of a 50-year-old African male admitted to the hospital with symptomatic anemia 1 week after pegloticase infusion. He was found to have glucose-6-phosphate dehydrogenase deficiency, predisposing him to hemolytic anemia. Hereby we discuss his clinical course, and suggest glucose-6-phosphate dehydrogenase deficiency screening prior to pegloticase infusion.

Increased right ventricular mass and VMI, the ratio of RV mass to LV mass, are predictors of mortality in incident, treatment-naive PAH. These findings challenge the notion that RV hypertrophy is adaptive in pulmonary hypertension.http://ow.ly/qzvt30ntJze

Atrial arrhythmia (AA) occurrence in pulmonary arterial hypertension (PAH) may determine clinical deterioration and affect prognosis. In this study we assessed AA incidence in idiopathic (IPAH) and systemic sclerosis related PAH (SSc-PAH) and evaluated risk factors, management, and impact on mortality. We collected baseline data from consecutive IPAH or SSc-PAH patients prospectively enrolled in the Johns Hopkins Pulmonary Hypertension Registry between January 2000 and July 2016. During follow-up AA onset, treatment, and outcome were recorded. Among 317 patients (201 SSc-PAH, 116 IPAH), 42 developed AA (19 atrial fibrillation, 10 flutter-fibrillation, 9 atrial flutter, and 4 atrial ectopic tachycardia) with a 13.2% cumulative incidence. Most events were associated with hospitalization (90.5%). Electrical or pharmacological cardioversion was attempted in most cases. Patients with AA had higher right atrial pressure, pulmonary wedge pressure ( P

Background: Supraventricular arrhythmias (SVA) occurrence in pulmonary arterial hypertension (PAH) patients may cause clinical deterioration and affect overall prognosis. Aim: To assess SVA incidence in idiopathic (IPAH) and systemic sclerosis related PAH (SScPAH) and evaluate risk factors, management, and impact on mortality. Methods: We collected baseline demographics, hemodynamics, six minute walking distance, and NTproBNP from consecutive IPAH or SScPAH patients prospectively enrolled in the Johns Hopkins Pulmonary Hypertension Registry from January 2000 to July 2016. During follow-up visits SVA occurrence, therapies and long-term outcome were recorded. Results: Among 317 patients (201 SScPAH, 116 IPAH, mean follow up of 67±51 months), 42 had SVA (34 atrial fibrillation, 18 atrial flutter and 4 atrial ectopic tachycardia). Incidence was slightly higher in SScPAH (13.9 vs 12.1%, p=0.22). In 64.3% of cases at least a second episode occurred. Most SVA were associated with hospitalization (90.5%). Electrical or pharmacological cardioversion was attempted in most cases. Patients with SVA had higher right atrial pressure, pulmonary wedge pressure, NTproBNP (p Conclusion: SVA occurrence in PAH is a matter of concern, acutely leading to clinical deterioration and ultimately impacting long-term prognosis. Therapeutic strategies aimed at preventing SVA recurrence may be a relevant goal.

Pulmonary hypertension due to heart failure with preserved ejection fraction (PH-HFpEF) has been poorly studied in patients with systemic sclerosis (SSc). We sought to compare clinical characteristics and survival of SSc patients with PH-HFpEF (SSc-PH-HFpEF) versus pulmonary arterial hypertension (SSc-PAH). We hypothesized that patients with SSc-PH-HFpEF have a similar poor overall prognosis compared with patients with SSc-PAH when matched for total right ventricular load. The analysis included 117 patients with SSc-PH (93 with SSc-PAH versus 24 with SSc-PH-HFpEF) enrolled prospectively in the Johns Hopkins PH Registry. We examined baseline demographics and hemodynamics at diagnostic right heart catheterization (RHC), two-dimensional echocardiographic characteristics, six-minute walking distance (6MWD), treatment modalities, and laboratory values (serum NT-proBNP, creatinine, uric acid, and sodium), and assessed survival. Demographics and clinical features were similar between the two groups. Baseline RHC showed significantly higher pulmonary and right heart pressures in the SSc-PH-HFpEF compared with the SSc-PAH group. Trans-pulmonary gradient (TPG), however, was equally elevated without significant difference between the groups. SSc-PH-HFpEF patients had left atrial enlargement on echocardiography compared with SSc-PAH patients. No significant differences were found between groups for 6MWD, NT-proBNP, and other laboratory values. Although overall median survival time was 4.6 years with no difference in mortality rate between the two groups (SSc-PH-HFpEF versus SSc-PAH: 75% versus 59%; P = 0.26), patients with SSc-PH-HFpEF had a twofold increased risk of death compared with SSc-PAH patients after adjusting for hemodynamics. Concomitant intrinsic pulmonary vascular disease and HFpEF likely contribute to very poor survival in patients with SSc-PH-HFpEF.