Systemic Sclerosis With a Normotensive Scleroderma Renal Crisis: A Diagnostic Dilemma.

Systemic sclerosis (SSc), also called scleroderma, is an auto-immune rheumatic disease that is characterized by fibrosis of the skin and internal organs and vasculopathy. Three of the severe manifestations of the disease include a scleroderma renal crisis (SRC), pulmonary arterial hypertension, and digital ulceration. Vascular manifestations like Raynaud's phenomenon are an almost universal symptom in patients with SSc and are often the earliest manifestation of the disease. An SRC occurs in approximately 10% of all patients with scleroderma. It is characterized by malignant hypertension and progressive renal failure. However, about 10% of SRC cases present with normal blood pressure or a normotensive renal crisis. A 65-year-old man with a history of peripheral vascular disease and newly diagnosed heart failure presented to the emergency department on account of progressive discoloration of the left big toe and intermittent confusion. Initially, he was noted to be hemodynamically stable, with bluish discoloration of his left lower extremity and left big toe, which was tender to palpation with palpable distal pulses. His left toe progressively became dusky and gangrenous, necessitating ray amputation by vascular surgery. His hospital course was further complicated by worsening acute kidney injury, requiring initiation of hemodialysis, and progressive hypoxia with the transition from room air to high-flow oxygen. As part of his workup for acute kidney injury (AKI), his antinuclear antibody (ANA) was found to be positive, with high titers, as well as elevated SCl-70 IgG. Despite the initiation of hemodialysis, and post-surgical revision, he continued to deteriorate. His family opted for comfort care measures, and he died a few days later. Although SSc is a rare disease, it is associated with significant morbidity and has one of the highest mortality rates among connective tissue diseases. SSc can present with heterogeneous manifestations, mimicking several isolated organ-specific conditions. This makes the diagnosis challenging, especially early in the course of the disease. A high index of suspicion, especially in the setting of rapidly progressing multi-organ involvement without a clear cause, should prompt further evaluation of systemic sclerosis.

Delayed Diagnosis of an Invisible Seizure: Cefepime-Induced Non-convulsive Status Epilepticus.

Cefepime-induced non-convulsive status epilepticus (NCSE) is a recognized adverse event of cefepime. Risk factors for this adverse event include older age, underlying renal dysfunction, previous brain injury, diabetes, and severe infection. We present a case of a 79-year-old woman with no prior seizure history, who was admitted for Pseudomonas aeruginosa surgical wound infection for which she was on cefepime. She developed acute encephalopathy with associated, occasional, right-sided myoclonic facial twitches 11 days into her admission. Electroencephalogram (EEG) confirmed NCSE as evident by epileptiform activity described as generalized periodic discharges with predominantly triphasic morphology. Cefepime was substituted with piperacillin-tazobactam> 24 hours after symptom onset. NCSE completely resolved two days after the discontinuation of cefepime. This case highlights the fact that NCSE can occur even when precautions such as renal dosing of cefepime are observed. Clinicians need to have a high index of suspicion for the condition when taking care of at-risk patients on cefepime, as delayed diagnosis correlates with potentially fatal outcomes.

Fatal Aerococcus urinae Aortic Valve Endocarditis with Severe Regurgitation.

BACKGROUND Aerococcus species are a rare cause of endocarditis. Micro-organism identification and antibiotic choice can pose significant management challenges to clinicians who care for patients with this infection. Aerococcus is a gram-positive micro-organism which is commonly misidentified because it shares many similarities with streptococcus and enterococcus species. Aerococcus urinae is usually found to cause urinary tract infections and occurs more frequently in patients with structural urinary tract abnormalities associated with urethral and ureteral obstruction such as kidney stones, phimosis, and prostate hyperplasia. However, it is reported to rarely cause endocarditis. CASE REPORT A 48-year-old man with a history of cocaine abuse and right hip replacement presented to our emergency department with acute encephalopathy. Through a complicated hospital course, he was found to be septic and the bacteria were initially misidentified as an alpha-hemolytic strep before being correctly identified as Aerococcus urinae. He was found to have multiple cerebral, likely septic, embolic infarcts and aortic valve endocarditis. Identification of the micro-organism on blood cultures was challenging, as were decisions about antibiotic choice. He died despite efforts of a multidisciplinary care team. CONCLUSIONS Our case highlights a unique case of Aerococcus endocarditis and shows the difficulty in initially identifying the bacteria. To our knowledge this is the first case reported in the setting of substance abuse. It also highlights the lack of appropriate guideline-directed therapy with regards to antibiotic choices in this group of patients, emphasizing the importance of further research in this regard.

A Case of Acute Necrotizing Myopathy in a Patient with Systemic Lupus Erythematosus.

BACKGROUND Systemic lupus erythematosus (SLE) is an autoimmune disorder affecting multiple organ systems with a wide spectrum of clinical presentation and associated with positive serologies. Musculoskeletal involvement in patients with SLE is relatively uncommon, occurring in approximately 4% to 16% of cases. Some patients can develop necrotizing myopathy without myositis. MRI in patients with SLE-associated necrotizing myopathy usually shows interstitial edema, while muscle biopsy often shows type 2 muscle fiber atrophy. We herein report an unusual case of acute necrotizing myopathy in a patient recently diagnosed with SLE. This case report focuses on the pertinent features related to the diagnosis of this patient while highlighting the management of acute necrotizing myopathy. CASE REPORT A 30-year-old African American woman presented to the Emergency Department with skin rashes, myalgia, polyarthralgia, and muscle weakness resulting in the inability to walk, 2 weeks after being diagnosed with SLE. Laboratory analysis showed elevated creatine kinase and myoglobin. She was found to have both MRI and biopsy findings suggestive of necrotizing myopathy. She was treated with mycophenolate mofetil and steroids, with an improvement of muscle strength and decrease in creatine kinase over a 2-week period. CONCLUSIONS Immune-mediated necrotizing myopathies are a rare group of debilitating myopathies that can be associated with SLE. The diagnosis of necrotizing myopathy in patients with SLE requires a high index of suspicion and careful work-up to establish a diagnosis. Muscle biopsy often shows type 2 muscle fiber atrophy. Immunosuppressive therapy is the mainstay of treatment, and early initiation of immunotherapies is associated with an improvement in patient outcomes.

The dilemma: scleroderma renal crisis vs lupus nephritis in a patient with mixed connective tissue disorder.

INTRODUCTION: Mixed connective tissue disorder (MCTD) is a rare connective tissue disorder characterized by features of systemic lupus erythematosus, dermatomyositis, systemic sclerosis, and rheumatoid arthritis. MCTD is associated with an elevated antibody titer to U1 small nuclear ribonucleoprotein. CASE DESCRIPTION: A 49-year-old man presented to the emergency department for evaluation of worsening shortness of breath with associated for bilateral hand pain and swelling associated with morning stiffness which was initially thought to be related to systemic lupus erythematous (SLE). He was also found to have a positive autoantibody, and he was later diagnosed with MCTD complicated by scleroderma renal crisis. CONCLUSION: MCTD is a rare connective tissue disorder with overlapping features of SLE, dermatomyositis, systemic sclerosis, and rheumatoid arthritis. The diagnosis of MCTD requires a high index of suspicion and careful workup. Immunosuppressive therapy is the mainstay of treatment that improves patient outcomes.

Missing in action.

Ectopically located parathyroid adenoma is one of the major causes of persistent and recurrent hyperparathyroidism and hypercalcemia. Approximately 0.3-8% of parathyroid adenoma is found in an ectopic location. Ectopic parathyroid adenomas are uncommon causes of persistent hypercalcemia and can be present at uncommon locations, including the hypoglossal nerve, the posterior triangle of the neck, axilla, and pericardium 3. A high index of suspicion is warranted when we see persistently elevated levels of parathyroid hormones (PTHs) and calcium levels post parathyroidectomy. Here, we present a patient who persistently had elevated calcium and PTH levels after parathyroidectomy.

Sertraline-Induced Acute Eosinophilic Pneumonia.

Acute eosinophilic pneumonia (AEP) is a rare but severe respiratory syndrome characterized by fever, hypoxemic respiratory failure, diffuse pulmonary infiltrates, and pulmonary eosinophilia. The most common cause of AEP is idiopathic, but it can be associated with antidepressant medications like sertraline. A 76-year-old female presented to our ED with acute hypoxemic respiratory failure. She had no history of smoking or prior lung disease. She did not improve after treatment with broad spectrum antibiotics so a trial of corticosteroids was initiated. Her work-up was negative for infectious or collagen vascular causes of the respiratory failure. She was diagnosed with AEP associated with sertraline. Her condition improved with corticosteroid therapy after discontinuation of sertraline. This case report highlights AEP as a possible adverse reaction of sertraline. Prompt discontinuation of the offending drug is necessary for early recovery.

A Framework for Inclusive Graduate Medical Education Recruitment Strategies: Meeting the ACGME Standard for a Diverse and Inclusive Workforce.

To help address health care disparities and promote higher-quality, culturally sensitive care in the United States, the Accreditation Council for Graduate Medical Education and other governing bodies propose cultivating a more diverse physician workforce. In addition, improved training and patient outcomes have been demonstrated for diverse care teams. However, prioritizing graduate medical education (GME) diversity and inclusion efforts can be challenging and unidimensional diversity initiatives typically result in failure.Little literature exists regarding actionable steps to promote diversity in GME. Building on existing literature and the authors' experiences at different institutions, the authors propose a 5-point inclusive recruitment framework for diversifying GME training programs. This article details each of the 5 steps of the framework, which begins with strong institutional support by setting diversity as a priority. Forming a cycle, the other 4 steps are seeking out candidates, implementing inclusive recruitment practices, investing in trainee success, and building the pipeline. Practical strategies for each step and recommendations for measurable outcomes for continued support for this work are provided. The proposed framework may better equip colleagues and leaders in academic medicine to prioritize and effectively promote diversity and inclusion in GME at their respective institutions.

Clinical features in patients with pulmonary embolism at a community hospital: analysis of 4 years of data.

The aim of this study is to assess the various clinical features, risk factors, and electrocardiographic (EKG) findings associated with acute pulmonary embolism (PE). Knowledge gained from the study may enable health care providers in diagnosis of PE, thus allowing them to carry out appropriate diagnostic testing and treatment after recognition of this potentially lethal disease. PE is common but frequently under-diagnosed clinical problem, associated with potentially fatal outcomes. Clinical presentation is highly variable, non-specific and most patients have an underlying identifiable risk factor. The presentation of PE can easily be confused with other cardio-pulmonary or systemic disorders. Prompt diagnosis of this potentially deadly disease is of utmost importance. Knowledge of salient features associated with PE may enable health care providers in diagnosis of PE, thus allowing them to carry out appropriate diagnostic testing and treatment after its recognition. We performed a single-center, cross-sectional descriptive study including all inpatient and emergency department encounters ≥18 years of age diagnosed with PE at our institution, a 300-bed inner city community hospital, during the dates January 2007 to December 2010. All patients were diagnosed with multi-detector 64-slice spiral computed tomography angiography. Using a standardized form, we performed simultaneous retrospective chart review to collect the necessary data required for the study. PE was confirmed in 334 patients during the 4 years study period. Mean age of subjects was 65.8 years (±16.4, range 22-98). Females represented 54 % of study subjects. Dyspnea, chest pain, and cough were present in 72, 38, and 19 % of the patients, respectively. Dyspnea was the only presenting symptom in 29 %. Tachypnea, hypoxia, tachycardia, and signs of DVT were present in 39, 35, 33, and 29 %, respectively. Cancer was most common risk factor present in 27 %, followed by prior history of venous thromboembolism (DVT or PE), immobilization, and surgery in 19, 15, and 15, respectively. EKG interpretation revealed normal sinus rhythm in 53 %, sinus tachycardia in 31 %, S1Q3T3 pattern in 6 %, and atrial fibrillation (AF) in 6 %.We also noted that 8 % of elderly patients had new onset AF at the time of diagnosis of PE. Diagnosis of PE remains a challenging task due to its variable presentation. Many of the classical features associated with this potentially fatal disease are often missing. This data re-emphasizes a wide spectrum of clinical presentation and non-specificity of symptoms of PE. Clinical suspicion of PE is a critical step and of paramount importance for further objective investigations, which would assist in the diagnosis and appropriate timely management of PE.

Acute interstitial pneumonia (hamman-rich syndrome) as a cause of idiopathic acute respiratory distress syndrome.

Hamman-Rich syndrome, also known as acute interstitial pneumonia, is a rare and fulminant form of idiopathic interstitial lung disease. It should be considered as a cause of idiopathic acute respiratory distress syndrome. Confirmatory diagnosis requires demonstration of diffuse alveolar damage on lung histopathology. The main treatment is supportive care. It is not clear if glucocorticoid therapy is effective in acute interstitial pneumonia. We report the case of a 77-year-old woman without pre-existing lung disease who initially presented with mild upper respiratory tract infection and then progressed to rapid onset of hypoxic respiratory failure similar to acute respiratory distress syndrome with unknown etiology. Despite glucocorticoid therapy, she did not achieve remission and expired after 35 days of hospitalization. The diagnosis of acute interstitial pneumonia was supported by the histopathologic findings on her lung biopsy.