A young lady with inflammation of unknown origin

Takayasu’s arteritis (TAK) is a systemic vasculitis mainly affecting the aorta and its first branches. The initial presentation can be very non-specific while its sequelae can be debilitating and fatal. Apart from clinical and biochemical tests, imaging studies remain pivotal for the diagnosis of this rare disease. Delay in treatment may result in vascular stenosis, leading to morbidity and mortality. We report a case of a young woman who presented with anemia with no obvious causes. Subsequently she developed ischemic symptoms and the diagnosis of TAK was established with magnetic resonance angiography (MRA). Our case illustrates the importance of recognition of the possibility of TAK in young women who presented with non-specific systemic upset and anemia of chronic inflammation. A high index of suspicion is needed and imaging studies should be considered early. The treatment of TAK will also be briefly reviewed.

Clinical characteristics and comorbidities of psoriatic arthritis (PsA) in Hong Kong

Aim The primary objective of this study was to describe the clinical characteristics of psoriatic arthritis (PsA). The secondary objective was to evaluate the prevalence of various PsA comorbidities and their associated factors, with particular emphasis on metabolic syndrome (MetS). Methods Consecutive patients fulfilling the Classification Criteria for Psoriatic Arthritis (CASPAR) from two local hospitals were recruited between June 2016 and January 2018. Demographic data and related clinical parameters were collected and analyzed. MetS was defined by the International Diabetes Federation criteria for Asians. Results For the study, 201 eligible PsA patients were recruited: 124 were men and 77 were women. The mean age of onset of PsO and PsA was 36.6 ± 14.2 and 44.5 ± 12.6 respectively. Of the patients, 64.2% had central obesity, 18.4% had diabetes, 32.8% had hypertension and 35.8% had MetS. Univariate analysis showed that the (1) age onset of PsA, (2) PsA duration, (3) PsO duration, and (4) tender joint-count were the potential associative factors of MetS. Subsequent regression model identified that both age onset and disease duration of PsA were significantly associated with MetS, with p-values of 0.02 and 0.018, respectively. Older age of onset (46.5 ± 12.2 vs 43.4 ± 12.7 years) or longer disease duration (9.8 ± 8.4 vs 7.0 ± 6.7 years) of PsA increased the likelihood of developing MetS. No association of MetS was found with ESR or CRP levels, PASI, dactylitis count, enthesitis index, tender and swollen joint count, age onset of PsO and severe skin status. Conclusion PsA is a heterogeneous disease with an extremely diverse range of clinical features. It is also notably associated with other comorbidities, especially metabolic syndrome, in which it is closely related to arthritis onset and duration. In view of their common prevalence, regular screening of these PsA-related comorbidities is highly recommended.

Clinical and Radiological Assessment in Axial Spondyloarthritis

The Assessment of SpondyloArthritis International Society (ASAS) has developed the concept of axial spondyloarthritis (SpA) in 2009. The symptoms and burden of disease of nonradiographic axial SpA and Ankylosing Spondylitis (AS) are similar and both can affect multiple organs and systems. Assessment and monitoring in SpA are, therefore, crucial. Different instruments have been developed for assessing and monitoring the wide variety of presentations in SpA. Generally, disease monitoring of patients can include patient reported outcome, clinical findings, laboratory tests, imaging, and disease activity composite scores.

A case of idiopathic inflammatory myopathy complicated by Epstein-Barr virus-associated lymphoma

We report a male patient who had refractory idiopathic inflammatory myopathy (IIM) presented with antisynthetase syndrome, being treated by potent immunosuppressants for years, developed Epstein-Barr virus (EBV)-associated lymphoma. Despite the stepping down of the immunosuppressives and active lymphoma therapy, the patient died. On top of the typical association of IIM and malignancy, rare EBV-associated tumors related to EBV infection secondary to the use of potent immunosuppressive therapies could occur. Further investigations are advisable if there are new symptoms and signs or in refractory IIM cases. This report serves as a diagnostic alert that the causation by EBV infection in unusual tumors found in patients with IIM should be considered, as both the treatment and prognosis may differ. A balance between the risks and benefits of immunosuppressive therapies should always be achieved.

Management of immune-related adverse events in patients treated with immune checkpoint inhibitors– Rheumatology point of view

The development of immunotherapy has revolutionized the cancer treatment in the recent years. Immune checkpoint inhibitors (ICPis) such as anti-cytotoxic T-lymphocyte-associated antigen 4 (anti-CTLA-4) and anti-programmed cell death protein (anti-PD1) and its ligand (anti-PDL1) have become part of the standard treatment of various malignancies. Immune-related adverse events (irAEs) were common in patients treated with ICPis. Rheumatologists should be aware of the upcoming challenges in the management of irAEs in a patient receiving treatment with ICPis.

An unusual case of heart attack

We reported a patient with antiphospholipid syndrome (APS) secondary to systemic lupus erythematosus (SLE) who suffered from myocardial infarction and graft occlusion after coronary artery bypass surgery. Our patient illustrates the impact of accelerated atherosclerosis in patients with SLE and APS and the importance of early institution of antithrombotic therapies. In this article, the treatment of APS is summarized. Although the main stay of treatment is anticoagulation, there is emerging evidence to support rituximab as an option for “refractory” cases of APS.

Successful treatment of refractory Behçet’s myositis with Tumor Necrosis Factor inhibitor

We report a case of refractory Behçet’s disease (BD) with small foot muscle myositis, which required Magnetic Resonance Imaging (MRI) for diagnosis. It demonstrates that BD may take years to develop to a full-blown picture. Our patient’s disease activity was promptly under control with the administration of Tumor Necrosis Factor inhibitor (TNFi), namely Adalimumab, after the failure of multiple immunosuppressants. The prognosis of myositis in BD is usually favorable. Image-guided muscle biopsy and HLA-B51 gene testing may be required in an atypical case. Corticosteroids remain the key therapy for myositis in BD, while TNFi should be considered in refractory cases.

An unusual case of heart attack

We reported a patient with antiphospholipid syndrome (APS) secondary to systemic lupus erythematosus (SLE) who suffered from myocardial infarction and graft occlusion after coronary artery bypass surgery. Our patient illustrates the impact of accelerated atherosclerosis in patients with SLE and APS and the importance of early institution of antithrombotic therapies. In this article, the treatment of APS is summarized. Although the main stay of treatment is anticoagulation, there is emerging evidence to support rituximab as an option for “refractory” cases of APS.