Four major medical societies involved with hydroxychloroquine (HCQ) therapy concur on the need for common principles and cooperation to minimize the risk of ocular toxicity. At a daily dosage of ≤5 mg/kg/day actual body weight, the risk of retinal toxicity from HCQ is <2% for usage up to 10 years. Widespread adoption of more sensitive testing techniques, such as optical coherence tomography and automated visual fields, by eye care providers will allow the detection of early toxicity and thus preserve the patient's visual function. Baseline testing is advised to rule out confounding disease when a patient is started on HCQ. Annual screening with sensitive tests should begin no more than 5 years after treatment initiation. Providers should be sensitive to the medical value of HCQ, and not stop the drug for uncertain indications. It is important to note that effective communication among prescribing physicians, patients, and eye care providers will optimize the utility and safety of HCQ.

Hydroxychloroquine (HCQ) and chloroquine (CQ) are well-established medications used in treating systemic lupus erythematosus and rheumatoid arthritis, as well as skin conditions such as cutaneous lupus erythematosus. In rare cases, arrhythmias and conduction system abnormalities, as well as cardiomyopathy, have been reported in association with HCQ/CQ use. Recently, however, the corrected QT interval (QTc)-prolonging potential of these medications, and risk of torsade de pointes (TdP) in particular, have been highlighted in the setting of their experimental use for COVID-19 infection. This report was undertaken to summarize the current understanding of HCQ/CQ cardiac toxicity, describe QTc prolongation and TdP risks, and discuss areas of priority for future research. A working group of experts across rheumatology, cardiology, and dermatology performed a nonsystematic literature review and offered a consensus-based expert opinion. Current data clearly indicate that HCQ and CQ are invaluable medications in the management of rheumatic and dermatologic diseases, but they are associated with QTc prolongation by directly affecting cardiac repolarization. Prescribing clinicians should be cognizant of this small effect, especially in patients taking additional medications that prolong the QTc interval. Long-term use of HCQ/CQ may lead to a cardiomyopathy associated with arrhythmias and heart failure. Risk and benefit assessment should be considered prior to initiation of any medication, and both initial and ongoing risk-benefit assessments are important with regard to prescription of HCQ/CQ. While cardiac toxicity related to HCQ/CQ treatment of rheumatic diseases is rarely reported, it can be fatal. Awareness of the potential adverse cardiac effects of HCQ and CQ can increase the safe use of these medications. There is a clear need for additional research to allow better understanding of the cardiovascular risk and safety profile of these therapies used in the management of rheumatic and cutaneous diseases.

This is a prospective, open-label, proof-of-concept study of tofacitinib, a Janus kinase inhibitor, as a steroid sparing therapy in corticosteroid-dependent pulmonary sarcoidosis. 5 patients with corticosteroid-dependent pulmonary sarcoidosis were treated with tofacitinib 5 mg twice daily. The primary endpoint was a ≥50% reduction in corticosteroids at week 16 with no worsening in pulmonary function or respiratory symptoms. 60% of patients (3/5) met the primary endpoint. One patient was lost to follow up prior to steroid taper, and another was withdrawn due to worsening of known neurosarcoidosis. The three patients who met the primary endpoint each tapered to ≤5 mg/day prednisone, respiratory symptoms improved, and spirometry remained stable. In this proof-of-concept study, the addition of a JAK-inhibitor allowed 60% of patients with pulmonary sarcoidosis to successfully taper corticosteroids. JAK-inhibitors are a promising therapy for pulmonary sarcoidosis, which require further investigation in randomized trials.

Objective.To evaluate the efficacy and safety of anakinra in inpatient management of acute gout and pseudogout.Methods.Hospitalized patients with acute gout (n = 77) or pseudogout (n = 11) or both (n = 3) were analyzed for response to anakinra and adverse effects.Results.Half of all patients had comorbidities limiting the treatment choice. Anakinra was well tolerated, and 92% of gout flares and 79% of pseudogout flares responded to treatment.Conclusion.Anakinra is an effective and safe treatment for acute gout and pseudogout in hospitalized patients, particularly in those with comorbidities.

A 70-year old woman with history of longstanding rheumatoid arthritis and recent diagnosis of complete heart block leading to a dualchamber pacemaker im plantation presented to the hospital with several weeks of worsening dyspnoea and lower extremity swelling. On physical exam, she had evidence of both intraand extravascular fluid accumulation as evidenced by elevation in jugular venous pressure and lower extremity oedema. Electrocardiogram demonstrated normal pacemaker function with atrial-sensed, ventricular-paced rhythm. Echocardiography showed moderate-severe increased wall thickness with moderately reduced left ventricular ejection fraction (Supplementary material online, Videos S1-S4). Diastolic parameters were consistent with a Grade III abnormal filling pattern, low mitral annular tissue velocity, and a global longitudinal strain (GLS) of À9.4% with associated relative apical sparing (ratio of apical/basal segments 1.2). Given concern for an infiltrative cardiomyopathy, she underwent 99m technetium pyrophosphate scintigraphy revealing semiquantitative visual Grade 3 and diffuse uptake (Perugini Grade 3) on single-photon emission computed tomography. Serum immunofixation, free light chains, and alpha galactosidase A were normal. Right ventricular biopsy was negative on Congo red staining. Electron microscopy revealed disorganized myofibrils, abundant vacuoles, multiple lamellar inclusion bodies, and curvilinear cytoplasmic bodies, findings that are pathognomonic for hydroxychloroquine-induced restrictive cardiomyopathy (Figure). Hydroxychloroquine was discontinued, and the patient started on guideline directed heart failure therapy. Several months later, repeat echocardiography revealed modest improvement in left ventricular ejection fraction.Hydroxychloroquine accumulates in lysosomes, inhibiting molecular breakdown with resultant accumulation of phospholipids and glycogen. Clinical presentation includes biventricular hypertrophy, restrictive physiology (late-stage), and conduction system abnormalities. Importantly, in an era when bone scintigraphy is being used with increasing frequency to diagnose transthyretin (ATTR) amyloid cardiomyopathy, we hope to raise awareness for the potential of a false-positive result in patients taking hydroxychloroquine.Hydroxychloroquine-induced restrictive cardiomyopathy. (Panel A) Parasternal long-axis and apical views showing left ventricular concentric hypertrophy. (Panel B) Pulsed-wave and tissue Doppler showing a pseudo-normal filling pattern and depressed mitral annular velocity with a normal E/e 0 ratio. (Panel C) Polar map of the left ventricle showing relative apical sparing and a GLS of À9.4%. (Panel D) Diffuse uptake of 99m technetium pyrophosphate on planar imaging and single-photon emission computed tomography. (Panel E) Electron microscopy of a myocardial section obtained from an endomyocardial biopsy showing cytoplasmic changes of the cardiomyocyte including disorganization of myofibrils, lamellar inclusion bodies (red arrows), abundant vacuoles, and curvil...

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Chronic thromboembolic pulmonary hypertension (CTEPH) is an uncommon disease that is the most serious complication associated with unresolved pulmonary embolism. This disease has several risk factors, but no familial pattern has been described. Few thrombophilic conditions have been reported to increase risk of CTEPH, and none of the hereditary thrombophilias causes this disease. The reason CTEPH develops in some patients after pulmonary embolism remains unknown. We describe a 54-year-old woman and her maternal aunt who both underwent pulmonary thromboendarterectomy for CTEPH. This represents the first description of familial CTEPH.

This is a prospective, open-label, proof-of-concept study of tofacitinib, a Janus kinase inhibitor, as a steroid sparing therapy in corticosteroid-dependent pulmonary sarcoidosis. 5 patients with corticosteroid-dependent pulmonary sarcoidosis were treated with tofacitinib 5 mg twice daily. The primary endpoint was a ≥50% reduction in corticosteroids at week 16 with no worsening in pulmonary function or respiratory symptoms. 60% of patients (3/5) met the primary endpoint. One patient was lost to follow up prior to steroid taper, and another was withdrawn due to worsening of known neurosarcoidosis. The three patients who met the primary endpoint each tapered to ≤5 mg/day prednisone, respiratory symptoms improved, and spirometry remained stable. In this proof-of-concept study, the addition of a JAK-inhibitor allowed 60% of patients with pulmonary sarcoidosis to successfully taper corticosteroids. JAK-inhibitors are a promising therapy for pulmonary sarcoidosis, which require further investigation in randomized trials.Trial Registration: clinicaltrials.gov NCT03793439; registered Jan 4, 2019