An unusual presentation of polyarteritis nodosa: A case report.

Polyarteritis nodosa (PAN) is a rare form of vasculitis which involves medium and small sized arteries. It can involve any system of the body, but lungs are typically spared. PAN typically presents with non-specific symptoms, such as fever, weight loss and myalgias, and symptoms related to the system involved. Negative serum ANCA helps in differentiating it from other similarly presenting vasculitis and definitive diagnosis is made on histopathology. Limb ischaemia is a rare presentation as well as a rare complication of PAN. We present the case of a 28-year-old female who presented with severe lower limb ischaemia and intermittent leg claudication as initial symptoms which led to initial diagnosis of critical limb ischaemia. Later in the course of the illness she developed gastrointestinal symptoms and weight loss. She was diagnosed as having PAN based on laboratory results and histopathological evidence. Intravenous steroids halted the progress of the disease, but the patient required transmetatarsal amputation of the foot to remove dead tissue. Vasculitis like PAN, although much rarer than atherosclerotic peripheral vascular disease (PVD), should be considered as a differential diagnosis in cases of limb ischaemia in which there is lack of risk factors for PVD.

An unusual presentation of polyarteritis nodosa: A case report / Presentación inusual de poliarteritis nudosa: informe de un caso

Polyarteritis nodosa (PAN) is a rare form of vasculitis which involves medium and small sized arteries. It can involve any system of the body, but lungs are typically spared. PAN typically presents with non-specific symptoms, such as fever, weight loss and myalgias, and symptoms related to the system involved. Negative serum ANCA helps in differentiating it from other similarly presenting vasculitis and definitive diagnosis is made on histopathology. Limb ischaemia is a rare presentation as well as a rare complication of PAN. We present the case of a 28-year-old female who presented with severe lower limb ischaemia and intermittent leg claudication as initial symptoms which led to initial diagnosis of critical limb ischaemia. Later in the course of the illness she developed gastrointestinal symptoms and weight loss. She was diagnosed as having PAN based on laboratory results and histopathological evidence. Intravenous steroids halted the progress of the disease, but the patient required transmetatarsal amputation of the foot to remove dead tissue. Vasculitis like PAN, although much rarer than atherosclerotic peripheral vascular disease (PVD), should be considered as a differential diagnosis in cases of limb ischaemia in which there is lack of risk factors for PVD.(AU)

La poliarteritis nudosa (PAN) es una forma rara de vasculitis que afecta a las arterias de mediano y pequeño calibre. Puede afectar a cualquier sistema del cuerpo, exceptuando los pulmones. La PAN cursa normalmente con síntomas no específicos tales como fiebre, pérdida de peso y mialgias, y los síntomas relacionados con el sistema afectado. Los ANCA séricos negativos ayudan a diferenciarla de otras vasculitis con presentación similar, realizándose el diagnóstico definitivo mediante histopatología. La isquemia de las extremidades es una presentación inusual y una complicación rara de la PAN. Presentamos el caso de una mujer de 28 años con isquemia en las piernas y claudicación intermitente de la pierna como síntomas iniciales, que condujo a un diagnóstico inicial de isquemia crítica de las extremidades. Posteriormente, en el curso de la enfermedad, desarrolló síntomas gastrointestinales y pérdida de peso. Se diagnosticó PAN, sobre la base de las pruebas de laboratorio y la evidencia histopatológica. La administración intravenosa de esteroides interrumpió el progreso de la enfermedad, pero la paciente precisó amputación transmetatarsiana del pie para eliminar el tejido muerto. Las vasculitis de tipo PAN, aunque son mucho más infrecuentes que la enfermedad vascular periférica (EVP), deberán considerarse como diagnóstico diferencial en los casos de isquemia de las extremidades, en las que no existieran factores de riesgo de EVP.(AU)

An unusual presentation of polyarteritis nodosa: A case report.

Polyarteritis nodosa (PAN) is a rare form of vasculitis which involves medium and small sized arteries. It can involve any system of the body, but lungs are typically spared. PAN typically presents with non-specific symptoms, such as fever, weight loss and myalgias, and symptoms related to the system involved. Negative serum ANCA helps in differentiating it from other similarly presenting vasculitis and definitive diagnosis is made on histopathology. Limb ischaemia is a rare presentation as well as a rare complication of PAN. We present the case of a 28-year-old female who presented with severe lower limb ischaemia and intermittent leg claudication as initial symptoms which led to initial diagnosis of critical limb ischaemia. Later in the course of the illness she developed gastrointestinal symptoms and weight loss. She was diagnosed as having PAN based on laboratory results and histopathological evidence. Intravenous steroids halted the progress of the disease, but the patient required transmetatarsal amputation of the foot to remove dead tissue. Vasculitis like PAN, although much rarer than atherosclerotic peripheral vascular disease (PVD), should be considered as a differential diagnosis in cases of limb ischaemia in which there is lack of risk factors for PVD.

Pulmonary Langerhans Cell Histiocytosis in the Elderly Smoker.

Langerhans cell histiocytosis (LCH), formally referred to histiocytosis X, is a histiocytic disorder with unknown etiology. The pathogenesis is believed to originate from myeloid dendritic cells and is now considered an inflammatory myeloid neoplasm within the revised 2016 Histiocyte Society classification. Pulmonary Langerhans cell histiocytosis (PLCH) is a rare and isolated form of LCH with a strong affiliation with smoking in adults of 20-40 years of age. Characteristic CT chest and histologic findings are instrumental in the early recognition and management of a disease. We herein report a case of a Caucasian smoker female with a significant history of interstitial lung disease (ILD) presented with recurrent and progressive worsening dyspnea. History of ILD and recurring respiratory symptoms raised suspicion of PLCH. CT chest and pathological findings confirmed the diagnosis, and discontinuation of smoking resulted in favorable clinical outcomes.

Exogenous Lipoid Pneumonia Complicated by Mineral Oil Aspiration in a Patient With Chronic Constipation: A Case Report and Review.

Exogenous lipoid pneumonia is a rare and frequently misdiagnosed lung disease. It occurs as an inflammatory reaction secondary to either aspiration or inhalation of lipids. Our patient had a history significant for recurrent pneumonia and the use of mineral oil for chronic constipation. A chest computed tomography showed multifocal consolidative opacities with areas of low attenuation, highly suspicious of exogenous lipid pneumonia. The diagnosis was confirmed with combined bronchoalveolar lavage and transbronchial lung biopsy that showed lipid-laden macrophages consistent with exogenous lipoid pneumonia. After thorough medication review, apart from mineral oil, no other contributing factors were found. A diagnosis of exogenous lipoid pneumonia associated with the use of mineral oil made and successfully managed by stopping the offending agent and supportive antibiotics.

Double Bolus Alteplase Therapy during Cardiopulmonary Resuscitation for Cardiac Arrest due to Massive Pulmonary Embolism Guided by Focused Bedside Echocardiography.

Massive pulmonary embolism (PE) frequently leads to cardiac arrest (CA) which carries an extremely high mortality rate. Although available, randomized trials have not shown survival benefits from thrombolytic use. Thrombolytics however have been used successfully during resuscitation in clinical practice in multiple case reports and in retrospective studies. Recent resuscitation guidelines recommend using alteplase for PE related CA; however they do not offer a standardized treatment regimen. The most consistently applied approach is an intravenous bolus of 50 mg tissue plasminogen activator (t-PA) early during cardiopulmonary resuscitation (CPR). There is no consensus on the subsequent dosing. We present a case in which two 50 mg boluses of t-PA were administered 20 minutes apart during CPR due to persistent hemodynamic compromise guided by bedside echocardiogram. The patient had an excellent outcome with normalization of cardiac function and no neurologic sequela. This case demonstrates the benefit of utilizing bedside echocardiography to guide administration of a second bolus of alteplase when there is persistent hemodynamic compromise despite achieving return of spontaneous circulation after the initial bolus, and there is evidence of persistent right ventricle dysfunction. Future trials are warranted to help establish guidelines for thrombolytic use in cardiac arrest to maximize safety and efficacy.

Creutzfeldt-Jakob Disease Presenting as Expressive Aphasia and Nonconvulsive Status Epilepticus.

Creutzfeldt-Jakob disease (CJD), the most common form of human prion diseases, is a fatal condition with a mortality rate reaching 85% within one year of clinical presentation. CJD is characterized by rapidly progressive neurological deterioration in combination with typical electroencephalography (EEG) and magnetic resonance imaging (MRI) findings and positive cerebrospinal spinal fluid (CSF) analysis for 14-3-3 proteins. Unfortunately, CJD can have atypical clinical and radiological presentation in approximately 10% of cases, thus making the diagnosis often challenging. We report a rare clinical presentation of sporadic CJD (sCJD) with combination of both expressive aphasia and nonconvulsive status epilepticus. This patient presented with slurred speech, confusion, myoclonus, headaches, and vertigo and succumbed to his disease within ten weeks of initial onset of his symptoms. He had a normal initial diagnostic workup, but subsequent workup initiated due to persistent clinical deterioration revealed CJD with typical MRI, EEG, and CSF findings. Other causes of rapidly progressive dementia and encephalopathy were ruled out. Though a rare condition, we recommend consideration of CJD on patients with expressive aphasia, progressive unexplained neurocognitive decline, and refractory epileptiform activity seen on EEG. Frequent reimaging (MRI, video EEGs) and CSF examination might help diagnose this fatal condition earlier.

Zinc Chloride Smoke Inhalation Induced Severe Acute Respiratory Distress Syndrome: First Survival in the United States with Extended Duration (Five Weeks) Therapy with High Dose Corticosteroids in Combination with Lung Protective Ventilation.

Zinc chloride smoke bomb exposure is frequently seen in military drills, combat exercises, metal industry works, and disaster simulations. Smoke exposure presents with variety of pulmonary damage based on the intensity of the exposure. Smoke induced severe acute respiratory distress syndrome (ARDS) is often fatal and there are no standard treatment guidelines. We report the first survival of smoke induced severe ARDS in the United States (US) with prolonged use of high dose steroids (five weeks) and lung protective ventilation alone. Previously reported surviving patients in China and Taiwan required extracorporeal membrane oxygenation (ECMO) and other invasive modalities. We suggest that an extended course of high dose corticosteroids should be considered for the treatment of smoke inhalation related ARDS and should be introduced as early as possible to minimize the morbidity and mortality. We further suggest that patients with smoke inhalation should be observed in the hospital for at least 48 to 72 hours before discharge, as ARDS can have a delayed onset. Being vigilant for infectious complications is important due to prolonged steroid treatment regimen. Patients must also be monitored for critical illness polyneuromyopathy. Additionally, upper airway injury should be suspected and early evaluation by otorhinolaryngology may be beneficial.

Obesity paradox, obesity orthodox, and the metabolic syndrome: An approach to unity.

Obesity and the accompanying metabolic syndrome are strongly associated with heightened morbidity and mortality in older adults. In our review of more than 20 epidemiologic studies of major infectious diseases, including leaders such as tuberculosis, community-acquired pneumonia, and sepsis, obesity was associated with better outcomes. A cause-and-effect relationship between over-nutrition and survival with infection is suggested by results of two preliminary studies of infections in mice, where high fat feeding for 8-10 weeks provided much better outcomes. The better outcomes of infections with obesity are reminiscent of many recent studies of "sterile" non-infectious medical and surgical conditions where outcomes for obese patients are better than for their thinner counterparts --- and given the tag "obesity paradox". Turning to the history of medicine and biological evolution, we hypothesize that the metabolic syndrome has very ancient origins and is part of a lifelong metabolic program. While part of that program (the metabolic syndrome) promotes morbidity and mortality with aging, it helps infants and children as well as adults in their fight against infections and recovery from injuries, key roles in the hundreds of centuries before the public health advances of the 20th century. We conclude with speculation on how understanding the biological elements that protect obese patients with infections or injuries might be applied advantageously to thin patients with the same medical challenges.