Cardiovascular Manifestations in Rheumatoid Arthritis.

Rheumatoid arthritis (RA) is a systemic inflammatory disorder that characteristically affects the joints. RA has extra-articular manifestations that can impact multiple organ systems including the heart, lungs, eyes, skin, and brain. Cardiovascular involvement is a leading cause of mortality in RA. Cardiovascular manifestations of RA include accelerated atherosclerosis, heart failure, pericarditis, myocarditis, endocarditis, rheumatoid nodules, and amyloidosis. Inflammation is an important mediator of endothelial dysfunction and is a key driver of cardiovascular risk and complications in patients with RA. Prompt identification of cardiac pathologies in patients with RA is essential for appropriate management and treatment. Choosing the most appropriate treatment regimen is based on individual patient factors. In this article, we provide a comprehensive review of the epidemiology, pathophysiology, clinical manifestations, diagnosis, and medical management of cardiovascular manifestations of RA. We also discuss the relationship between anti-rheumatic medications, specifically non-steroidal anti-inflammatory drugs, corticosteroids, methotrexate, statins, tumor necrosis factor inhibitors, interleukin-6 inhibitors, Janus kinase inhibitors, and cardiovascular disease.

Dual-Energy Computed Tomography (DECT) Resolves the Diagnostic Dilemma in an Atypically Presenting Case of Gout.

Gout is a common inflammatory arthropathy that presents as acute monoarthritis, most commonly of the first metatarsophalangeal (MTP) joint. Chronic polyarticular involvement may lead to confusion with other inflammatory arthropathies, including rheumatoid arthritis (RA). A thorough history, physical examination, synovial fluid analysis, and imaging are keys to establishing a correct diagnosis. Although a synovial fluid analysis remains the gold standard, the affected joints may be difficult to access by arthrocentesis. In cases where a large monosodium urate (MSU) crystal deposition is in the soft tissues - the ligaments, bursae, and tendons, it becomes a clinical impossibility. In such cases, dual-energy computed tomography (DECT) can assist in differentiating gout from other inflammatory arthropathies, including RA. Additionally, DECT can perform quantitative analysis of tophaceous deposits and, therefore, assess response to treatment.

Treatment of Pyoderma Gangrenosum With Mycophenolate and Hyperbaric Oxygen Therapy: A Case Report and Literature Review.

Pyoderma gangrenosum is an uncommon inflammatory ulcerative skin disorder with an unclear etiology. In many cases, it is associated with several underlying systemic diseases, with inflammatory bowel disease being the most common one. Since it does not have any specific clinical or laboratory findings, it is a diagnosis of exclusion. A multidisciplinary approach is vital in treating pyoderma gangrenosum. Its recurrence remains common, and it also has an unpredictable prognosis. Here, we report a case report of pyoderma gangrenosum, which was successfully treated with mycophenolate and hyperbaric oxygen therapy.

Hydroxychloroquine and Cardiotoxicity.

Hydroxychloroquine (HCQ) has been used for rheumatological diseases such as systemic lupus erythematous and rheumatoid arthritis and demonstrated to improve clinical symptoms and reduce long-term sequelae. The drug is metabolized in the liver, is primarily excreted through the kidney, and works by modulating major histocompatibility complex (MHC) and various cytokines, suppressing the immune system in the process. Prolonged administration and high dosages of HCQ have been associate with cardiotoxic effects such as bradycardia, tachycardia, QT prolongation, atrioventricular block, and cardiomyopathy. Common cardiac biopsy findings of HCQ-induced toxicity are enlarged and vacuolated cells on light microscopy along with the presence of myelinoid and curvilinear bodies on transmission electron microscopy. HCQ cardiotoxicity is not very well recognized, and there are no current guidelines for routine cardiac function monitoring from either rheumatology or cardiology societies.

Cardiovascular Manifestations of Systemic Sclerosis: An Overview of Pathophysiology, Screening Modalities, and Treatment Options.

Systemic sclerosis, previously known as scleroderma, is a heterogeneous, systemic disease that is defined by its 3 pathological hallmarks: the production of autoantibodies, small vessel vasculopathy, and fibroblast dysfunction, leading to an increased deposition of extracellular matrix. We conducted a review of the available literature that covers the cardiovascular manifestations of SSc: electrical conduction abnormalities, pulmonary hypertension, pericardial disease, and atherosclerosis. Within each major category, we will discuss the definition, diagnostics, and available treatment options. Increased mortality from cardiovascular complications necessitates early screening and management. Annual screening with noninvasive modalities is encouraged. The current management of each complication generally follows the management algorithms of patients regardless of SSc status and is dependent on the severity of the patient's clinical presentation.

Cardiac Manifestations of Systemic Lupus Erythematous: An Overview of the Incidence, Risk Factors, Diagnostic Criteria, Pathophysiology and Treatment Options.

Systemic lupus erythematosus (SLE) is a complex connective tissue disease that can potentially affect every organ of the human body. In some cases, SLE may present with diverse cardiac manifestations including pericarditis, myocarditis, valvular disease, atherosclerosis, thrombosis, and arrhythmias. Heart disease in SLE is associated with increased morbidity and mortality. It is unclear whether traditional treatments for coronary artery disease significantly impact mortality in this population. Current therapeutic agents for SLE include glucocorticoids, hydroxychloroquine, mycophenolate mofetil, azathioprine, methotrexate, cyclophosphamide, and B cell-directed therapies. This article will provide a comprehensive review and update on this important disease state.

Cardiac Manifestations in Idiopathic Inflammatory Myopathies: An Overview.

Idiopathic inflammatory myopathies are a group of autoimmune diseases that are characterized by muscle inflammation resulting in elevated muscle enzyme release and distinctive biopsy findings. This group of conditions includes polymyositis, dermatomyositis, inclusion body myositis, and necrotizing autoimmune myopathy. Although they have many similarities, the inflammatory myopathies differ in their clinical, pathological, and treatment realms. Extramuscular manifestations may involve many organs that include the skin, joints, heart, lungs, and gastrointestinal tract. Cardiovascular involvement is one of the leading causes of mortality in polymyositis and dermatomyositis. Surveillance and prevention of cardiovascular risk factors are therefore essential. In this article, we review the epidemiology, pathophysiology, clinical manifestations, diagnosis, and management of cardiovascular complications of idiopathic inflammatory myopathies with the main focus on polymyositis and dermatomyositis.

Rheumatoid Arthritis: Common Questions About Diagnosis and Management.

Rheumatoid arthritis is the most commonly diagnosed systemic inflammatory arthritis, with a lifetime prevalence of up to 1% worldwide. Women, smokers, and those with a family history of the disease are most often affected. Rheumatoid arthritis should be considered if there is at least one joint with definite swelling that is not better explained by another disease. In a patient with inflammatory arthritis, the presence of a rheumatoid factor and/or anti-citrullinated protein antibody, elevated C-reactive protein level, or elevated erythrocyte sedimentation rate is consistent with a diagnosis of rheumatoid arthritis. Rheumatoid arthritis may impact organs other than the joints, including lungs, skin, and eyes. Rapid diagnosis of rheumatoid arthritis allows for earlier treatment with disease-modifying antirheumatic drugs, which is associated with better outcomes. The goal of therapy is to initiate early medical treatment to achieve disease remission or the lowest disease activity possible. Methotrexate is typically the first-line agent for rheumatoid arthritis. Additional disease-modifying antirheumatic drugs or biologic agents should be added if disease activity persists. Comorbid conditions, including hepatitis B or C or tuberculosis infections, must be considered when choosing medical treatments. Although rheumatoid arthritis is often a chronic disease, some patients can taper and discontinue medications and remain in long-term remission.

Diagnosis and management of rheumatoid arthritis.

Rheumatoid arthritis is the most commonly diagnosed systemic inflammatory arthritis. Women, smokers, and those with a family history of the disease are most often affected. Criteria for diagnosis include having at least one joint with definite swelling that is not explained by another disease. The likelihood of a rheumatoid arthritis diagnosis increases with the number of small joints involved. In a patient with inflammatory arthritis, the presence of a rheumatoid factor or anti-citrullinated protein antibody, or elevated C-reactive protein level or erythrocyte sedimentation rate suggests a diagnosis of rheumatoid arthritis. Initial laboratory evaluation should also include complete blood count with differential and assessment of renal and hepatic function. Patients taking biologic agents should be tested for hepatitis B, hepatitis C, and tuberculosis. Earlier diagnosis of rheumatoid arthritis allows for earlier treatment with disease-modifying antirheumatic agents. Combinations of medications are often used to control the disease. Methotrexate is typically the first-line drug for rheumatoid arthritis. Biologic agents, such as tumor necrosis factor inhibitors, are generally considered second-line agents or can be added for dual therapy. The goals of treatment include minimization of joint pain and swelling, prevention of radiographic damage and visible deformity, and continuation of work and personal activities. Joint replacement is indicated for patients with severe joint damage whose symptoms are poorly controlled by medical management.

Systemic sclerosis: bilateral improvement of Raynaud's phenomenon with unilateral digital sympathectomy.

OBJECTIVES: To demonstrate that unilateral digital sympathectomy, in patients with Raynaud's phenomenon (RP) and systemic sclerosis (SSc), may result in bilateral resolution of RP and digital ulcerations. METHODS: We report a case of SSc and RP that had bilateral benefits from unilateral digital sympathectomy. A computer-assisted Medline/PubMed search of the medical literature was performed for 1960 through June 2009 using the keywords sympathectomy, Raynaud's phenomenon, systemic sclerosis, CREST, and digital ulcers. These searches were also combined with text words unilateral, ipsilateral, bilateral, digital sympathectomy, selective sympathectomy, autonomic nervous system, hyperhidrosis, etiology, pathogenesis, hypothesis, and treatment. Only pertinent literature, primarily in the English language, was included. RESULTS: The majority of patients with SSc have RP and many suffer from digital ulcerations. Medical and behavioral management may have limited benefit and surgical intervention can be considered in recalcitrant cases, although efficacy data are sparse. We describe a man with limited SSc who underwent unilateral digital sympathectomy but manifested bilateral benefit. To our knowledge, this is the first published report of contralateral response with this procedure. The patient ultimately demonstrated these digital benefits when stressed with extreme cold and hypoxia while mountaineering. Despite the onset of high-altitude sickness and cerebral edema, his fingers remained unaffected while other mountaineers sustained severe frostbite or died of hypothermia. CONCLUSIONS: Selective unilateral sympathectomy in SSc, for RP with digital ulcerations, can result in bilateral benefits despite intense challenge with cold and hypoxia.

Fungal leukocytoclastic vasculitis as a presentation of systemic vasculitis in a patient with systemic lupus erythematosus.

Patients with systemic lupus erythematosus (SLE) commonly have vasculitis with cutaneous involvement. Disease activity associated with SLE is frequently responsible for the vasculitis, although infection is also important to consider. We describe a young woman with SLE who was found to have leukocytoclastic vasculitis due to Candida albicans.