RESUMEN
This is the first in a new series of case reports that will present real scenarios from our community hospital. The cases are chosen to highlight clinical dilemmas and offer review and perspective on what is currently known about the topic. We present the case of a 55-year-old Caucasian male who presented to the emergency department of our community hospital for worsening dyspnea. Evaluation in the Emergency department revealed a diagnosis of extensive pulmonary emboli in the pulmonary vasculature. The patient was admitted to the ICU. A clinical decision was made to initiate treatment with low-dose tissue plasminogen activator (tpa) followed by intravenous heparin infusion. Several hours later the patient developed significant epistaxis. A decision was made to stop the heparin infusion. Later that day, the patient had abrupt clinical deterioration with subsequent cardiac arrest and did not recover. We discuss the classification and treatment of acute pulmonary embolism, the management of epistaxis in an anticoagulated patient, and the clinical conundrum of balancing active bleeding in patient requiring anticoagulation.
RESUMEN
The idiopathic inflammatory myopathies (IIM) are rare sporadic disorders with an overall annual incidence of approximately 1 in 100,000 and with a higher incidence in women. IIM is an autoimmune process leading to muscle inflammation due to a 'dysfunctional adaptive immune response evidenced by cell-mediated myocytoxicity, a high prevalence of autoantibodies and overexpression of Major Histocompatibility (MHC) I and II molecules on the muscle sarcolemma'. These autoimmune processes can be appreciated as inflammatory infiltrates in muscle biopsies. Common clinical findings in patients diagnosed with IIM include proximal muscle weakness, elevated creatinine kinase levels, circulating autoantibodies, radiological findings of muscular inflammation, and sometimes edema; in some patients, systemic symptoms such as dysphagia can also be present. Currently, there is no specific IIM classification scheme that incorporates all IIM subtypes; however, the four major IIM subtypes include dermatomyositis, polymyositis, inclusion body myositis, and immune-mediated necrotizing myopathy (IMNM). Two clinical cases are presented in this case report to illustrate a smoldering IIM, antisynthetase syndrome, and a more progressive IIM, anti-signal recognition particle IMNM; highlight the utility of the myositis-specific autoantibody panel for early diagnosis, targeted therapy, and prognosis; and offer primary care providers clues to IIM diagnosis.
RESUMEN
Primary angiitis of the central nervous system (PACNS) is a rare disease with various clinical presentations. It is the preferred name for vasculitis that is confined to the central nervous system (CNS) and is often considered a diagnosis of exclusion in vascular or inflammatory CNS diseases. This case describes a 46-year-old right-handed female with a past medical history of hypertension (HTN), hyperlipidemia (HLD), diabetes mellitus type two (DM2), obesity, and hemorrhagic stroke who was transferred from an outside facility after a one-month hospitalization to be evaluated for CNS vasculitis. Emergency medical personnel who brought the patient to the receiving hospital endorsed dysarthria and fluctuating level of cognition. Before she was transferred, the patient underwent a series of computed tomography (CT) and magnetic resonance imaging and angiographies (MRI/MRA) as well as four-vessel angiography. The studies revealed multiple bilateral striatal and cortical infarcts, scattered narrowing and occlusion of major cerebral vasculature, as well as other signs initially more suggestive of intracranial atherosclerosis than CNS vasculitis. Before she was transferred, imaging demonstrated a new cortical stroke. Hypercoagulable studies were positive for protein C deficiency although ensuing echocardiograms with normal ejection fractions were negative for a source of cardioembolism. Having undergone extensive rheumatological, radiological, and neurosurgical evaluation in the receiving facility, recommendations were made for the patient to undergo leptomeningeal biopsy to confirm the diagnosis of vasculopathy and to specifically rule out intracranial atherosclerosis and reversible cerebral vasoconstriction syndrome (RCVS).