Pneumocystis jirovecii Pneumonia in Patients with Rheumatic Diseases: Risk Assessment and Prophylaxis

Pneumocystis jirovecii pneumonia (PJP) is an uncommon opportunistic infection in patients with rheumatic diseases with high mortality. Unlike other non-HIV conditions, international guideline for PJP prophylaxis in rheumatic diseases is currently lacking. Recent evidence regarding the risk of PJP and effectiveness of prophylaxis has been accumulating. This Review provides an update on the information about risk factors associated with PJP in patients with rheumatic diseases based on rheumatic diagnoses, use of immunosuppressive agents and other disease-related factors. The second part of the article summarizes evidence regarding the effectiveness of PJP prophylaxis by considering both disease-related and therapy-related factors. Finally, the Review outlined the currently available disease-specific recommendations and local guidelines, and appreciate the factors that influence physicians’ decision.

MRI in Axial Spondyloarthritis: Position Statements from the Hong Kong Society of Rheumatology

The Hong Kong Society of Rheumatology (HKSR) has developed evidence-based position statements on the use of magnetic resonance imaging (MRI) in axial spondyloarthritis (axSpA). A special interest group (SIG) developed the statements based on the literature review of the following research questions: 1) In whom should SI joint MRI be done to diagnose axSpA? 2) Should spine MRI be performed for diagnosis of axSpA? 3) Should spine and SI joint MRI be used in the assessment and monitoring of disease activity? 4) Which MRI sequence(s) should be used in axSpA? These statements have at least 75% agreement from voting rheumatology fellows of HKSR, and are developed to serve as local practice guidelines for rheumatologists and musculoskeletal radiologists in the management of axSpA.

Upadacitinib as Novel Treatment for Rheumatoid Arthritis with T-Cell Granular Lymphocytic Leukemia: A Case Report and Narrative Review

T-cell large granular lymphocyte (T-LGL) leukemia is a rare and indolent clonal disorder of LGLs, associated with rheumatoid arthritis and neutropenia. The authors present a case of a 62-year-old male with rheumatoid arthritis (RA) who was diagnosed with T-LGL leukemia, with predominant neutropenia, and a poor response to conventional treatment. Subsequently, tofacitinib (a Janus Kinase 1 and 3 inhibitor, [JAK1/3 inhibitor]) resulted in improvement of the patient’s RA symptoms and temporary improvement of the neutropenia. Ultimately, upadacitinib (a specific JAK1 inhibitor) resulted in further improvement of the neutropenia and control of his RA. To the best of our knowledge, this is the first case report of coexisting RA and LGL leukemia that was treated with upadacitinib and showed clinical improvement.

Tocilizumab and Renal Artery Stent—Therapeutic Strategy for Takayasu Arteritis

Takayasu vasculitis (TAK) is a form of large vessel vasculitis clinically manifesting as pulseless disease or hypertension. It is more common in South East Asia and Japan, India, and Mexico [1]. It is increasingly being recognized due to increased awareness among medical fraternity and better imaging modalities. Undetected hypertension, pulselessness, and syncope are more common symptoms and presentation during pregnancy is unusual and can lead to bad obstetric outcomes. Recent evidences support the use of tocilizumab for inducing remission in Takayasu arteritis. We report this rare case of vasculitis presenting in pregnancy as malignant hypertension. A 20-year-old pregnant woman (45 days) presented with headache and nausea but no fever. She had a history of intermittent claudication of legs for the past 3 years but not evaluated. During examination, pulses were felt normally and blood pressure (BP) 180/110, no murmurs in cardiac auscultation, but she had abdominal bruit (renal vessels). Other systems were normal. Echocardiogram (ECHO) showed dilated ascending aorta. Doppler of renal vessels showed narrowing of renal arteries. Unfortunately, she had to undergo termination of pregnancy (high BP in spite of antihypertensives). Her computed tomography (CT) angiogram showed features of TAK with type 5 pattern–she had methylprednisolone infusion 500 mg daily for 3 days, followed by injection tocilizumab 400 mg monthly 3 doses. Once remission was achieved, she had recanalization by percutaneous transluminal angioplasty of right renal artery. She is currently maintained on aspirin and telmisartan. Awareness of causes of high BP, inputs by radiologist, cardiologist, and rheumatologist and understanding by the patient and family helped to achieve good outcome albeit the miscarriage.

Uveitis and Rheumatological Diseases

Uveitis is the most common ophthalmological disorder in the field of rheumatology, accounting for a significant proportion of visual morbidity, both locally and internationally. Causative factors can be divided into infectious and noninfectious etiologies. The diagnosis of uveitis is a major challenge due to heterogeneity in presentation. The disease course may be acute monophasic, recurrent, or chronic relapsing. Complications include posterior synechiae, secondary cataract, ocular hypertension or glaucoma, macular edema, retinal vascular occlusion, epiretinal membrane, and so on, and ultimately visual loss. Antimicrobial therapy is indicated for infection, whereas noninfectious uveitis warrants a combination of steroids, immunosuppressives, and anti-inflammatory agents. With the advancement of biologics, treatment strategies in chronic, noninfectious uveitis have had multiple breakthroughs, particularly in treatment-resistant cases. This article provides a review of the diagnostic approach to uveitis based on symptomatology and ophthalmological findings, and discussion of relevant treatment modalities and strategies.

Association of Parvovirus B19 with Rheumatic Diseases

Human parvovirus B19 infections are well reported to be associated with different autoimmune disorders. They can either mimic or trigger autoimmune diseases, such as systemic lupus erythematous (SLE), rheumatoid arthritis (RA), and vasculitis. A lack of awareness about this infection can result in delays in diagnosis and poor care. In this review, the basic biology and clinical aspects of the parvovirus, human immune response, and the pathogenesis in the rheumatic diseases are discussed.

Recommendations on Management of Latent Tuberculosis Infection in Patients Initiating Anti-tumor Necrosis Factor Biologics

Patients with immune-mediated inflammatory diseases on anti-tumor necrosis factor (TNF) agents are at increased risk of developing active tuberculosis (TB). Screening and treatment of latent tuberculosis infection (LTBI) with the use of a standard screening protocol is important in reducing the risk of TB reactivation during therapies with anti-TNF agents. The Latent Tuberculosis Infection Working Group under the Tuberculosis Control Coordinating Committee of the Department of Health and the Hospital Authority has reviewed and assessed local and international scientific evidence and formulated guideline statements on the management of LTBI in patients initiating anti-TNF biologics.

Pharmacogenomics and Rheumatological Practice

Pharmacogenomics, the study of the effect of genetics on the variation in drug response, is designed to maximize drug efficacy and minimize adverse effects. It is assuming increasing importance in clinical practice. Rheumatologists, who manage patients with complex diseases with unpredictable outcomes over long periods of time, should embrace tools that help customize treatment for their patients. In this article, the basis for pharmacogenomics is presented, followed by description of the tests relevant to drugs encountered in rheumatology practice, concluding with ideas about implementation and future developments.

Update of Management of ANCA-Associated Vasculitis

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is associated with significant morbidity and mortality. Management of AAV is divided into induction of remission and maintenance of remission. Management of AAV has evolved with the aim of improving treatment outcomes and minimizing treatment toxicities. In this article, we will review the latest evidence on the treatment of AAV.

A Rare Cause of Young Stroke: What Rheumatologists Should Know about Fabry Disease

Systemic vasculitis is a category of autoinflammatory diseases usually involving multiple organs. Involvement of the central nervous system (CNS) may mimic other disorders, hence resulting in overdiagnosis. This is a case report of a young man who presented with acrodynia, episodic pyrexia, and recurrent strokes. He was treated for systemic vasculitis with glucocorticoids and immunosuppressants, which failed to prevent the stroke. Finally, Fabry disease was diagnosed after [Formula: see text]-galactosidase A gene mutation analysis was done. Rheumatologists should be aware of Fabry disease, which may present with multi-system involvement that mimics vasculitis. To avoid inappropriate treatment, a comprehensive differential diagnosis should be considered before a diagnosis of primary systemic vasculitis is made. Recurrent strokes, especially in males younger than 40, should raise suspicions of Fabry disease.