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.

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.

Scleroderma (systemic sclerosis) is an autoimmune rheumatic disorder that is characterized by fibrosis, vascular dysfunction, and autoantibody production that involves most visceral organs. It is characterized by a high morbidity and mortality rate, mainly due to disease-related complications. Epidemiological data describing mortality and survival in this population have been based on both population and observational studies. Multiple clinical and non-clinical factors have been found to predict higher likelihood of death among thepatients. Here, we do an extensive review of the available literature, utilizing the PubMed database, to describe scleroderma and non-scleroderma related determinants of mortality in this population. We found that even though the mortality among the general population has declined, scleroderma continues to carry a very high morbidity and mortality rate, however we have made some slow progress in improving the mortality among scleroderma patients over the last few decades.

Gout is a well-known inflammatory arthritis and affects four percent of the United States population. It results from the deposition of uric acid crystals in joints, tendons, bursae, and other surrounding tissues. Prevalence of gout has increased in the recent decade. Gout is usually seen in conjunction with other chronic comorbid conditions like cardiac disease, metabolic syndrome, and renal disease. The diagnosis of this inflammatory arthritis is confirmed by visualization of monosodium urate (MSU) crystals in the synovial fluid. Though synovial fluid aspiration is the standard of care, it is often deferred because of inaccessibility of small joints, patient assessment during intercritical period, or procedural inexperience in a primary care office. Dual energy computed tomography (DECT) is a relatively new imaging modality which shows great promise in the diagnosis of gout. It is a good noninvasive alternative to synovial fluid aspiration. DECT is increasingly useful in diagnosing cases of gout where synovial fluid fails to demonstrate monosodium urate crystals. In this article, we will review the mechanism, types, advantages, and disadvantages of DECT.

Adult onset still’s disease (AOSD) is a rare inflammatory disorder characterized by high fevers, arthralgia, salmon rash and leukocytosis. It is a diagnosis of exclusion and popularly diagnosed by the Yamaguchi criteria. Cardiac involvement in AOSD causing myocarditis is seldom seen. Echocardiography is an important measure of cardiac manifestation, but a more characteristic confirmation is obtained by the cardiac MRI. It is an increasingly recognized noninvasive alternative to the gold standard endomyocardial biopsy. In this case, mid myocardial enhancement in the absence of left ventricular dysfunction was consistent with a diagnosis of myocarditis in AOSD. The delay in diagnosis was caused by AOSD masquerading as acute coronary syndrome. This case highlights the importance of early recognition of myocarditis in an atypical presentation of AOSD which results in early immunomodulatory therapy and an improved clinical outcome.

Extracorporeal membrane oxygenation (ECMO) provides complete or partial support of the heart and lungs. Ever since its inception in the 1960s, it has been used across all age groups in the management of refractory respiratory failure and cardiogenic shock. While it has gained widespread acceptance in the neonatal and pediatric physician community, ECMO remains a controversial therapy for Acute Respiratory Distress Syndrome (ARDS) in adults. Its popularity was revived during the swine flu (H1N1) pandemic and advancements in technology have contributed to its increasing usage. ARDS continues to be a potentially devastating condition with significant mortality rates. Despite gaining more insights into this entity over the years, mechanical ventilation remains the only life-saving, yet potentially harmful intervention available for ARDS. ECMO shows promise in this regard by offering less dependence on mechanical ventilation, thereby potentially reducing ventilator-induced injury. However, the lack of rigorous clinical data has prevented ECMO from becoming the standard of care in the management of ARDS. Therefore, the results of two large ongoing randomized trials, which will hopefully throw more light on the role of ECMO in the management of this disease entity, are keenly awaited. In this article we will provide a basic overview of the development of ECMO, the types of ECMO, the pathogenesis of ARDS, different ventilation strategies for ARDS, the role of ECMO in ARDS and the role of ECMO as a bridge to lung transplantation.

Purpose: Myeloid sarcoma (MS), chloroma or granulocytic sarcoma (GS) represents an extra medullary tumor of myeloblasts and/or immature myeloid cells. It is an uncommon manifestation of acute myeloid leukemia (AML) and less often, of chronic myeloid leukemia (CML), polycythemia Vera and primary myelofibrosis. Very rarely, these tumors can present de novo and when they do, often represents as a forerunner to the development of AML. Methods: 49-year-old non-smoker male with no prior medical history presented with post prandial epigastric pain since two weeks which was progressively worsening in severity. There was no associated nausea, vomiting, diarrhea, constipation. He denied weight loss, hematemsis or malena. Physical examination was unremarkable except for mild epigastric tenderness. Laboratory examination including a complete blood count, Erythrocyte sedimentation rate, liver and renal profile were normal. A computerized tomogram (CT) of the abdomen demonstrated concentric thickened distal part of duodenum with prominent mesenteric lymph nodes suggestive of a primary mesenteric or small bowel (SB) tumor versus lymphoma. Esophagogastroduodenoscopy showed erythematous and edematous areas in the third and fourth part of duodenum. Histological examination demonstrated diffuse infiltrates by uniformly blastoid appearing cells. A differential diagnosis of lymphoma and GS were considered. Immunohistochemical staining was consistent with myeloid sarcoma. Cytogenetic analysis was normal. A bone marrow biopsy was negative for AML, with no other metastasis noted on a staging work-up. The patient underwent intensive chemotherapy for AML with high-dose cytosine arabinoside and doxorubicin, which the patient has been tolerating well and following up with oncology. Results: See Methods for case. Conclusion: MS can develop in any anatomic site with a particular predilection for skin, bone, lymph nodes, soft tissue and genitourinary tract. Involvement of gastrointestinal tract (GI) is rare. It generally occurs in association with AML, as an initial presentation or a relapse. An isolated primary GS of small intestine in a non-leukemic patient is uncommon. De novo MS with GI involvement in aleukemic patient, can clinically, radiologically and histopathologically mimic other solid neoplasms, making it a diagnostic challenge. When the diagnosis of MS is made before the presentation of leukemia, the average time period to progression is 10 months. Clinicians need to be aware of this rare neoplastic condition and should have a high index of suspicion for progression to AML. Hence early recognition and prompt institution of therapy is crucial, and may delay its progression to a systemic disease.