SESSION TYPE: Miscellaneous Case Report Posters IIPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION:

Sarcoidosis is an idiopathic multi-systemic disease in which the underlying pathology is characterized by the formation of non-caseating granulomas within various organs. Clinical presentation can vary markedly and symptoms typically are associated with the pattern of organ involvement. Such symptoms range from dry cough and dyspnea to vague constitutional symptoms including fatigue, malaise, and low-grade fevers. We describe a rare case of sarcoidosis in which our patient's presenting symptom was persistent high grade fevers for seven weeks in the absence of any localizing symptoms.

CASE PRESENTATION:

A 40-year old caucasian male presented to our hospital with a seven week history of high grade fevers and 35-pound weight loss. His temperature was noted to be as high as 105° F on a few occasions. The fevers persisted in spite of multiple courses of empiric antibiotics in the outpatient setting. Other than extreme weakness, poor appetite, and relentless fever, our patient had no other localizing symptoms . His examination was impressive only for a temperature of 103° F. Initial labs were impressive for a white count of 1,600 cells/mL, hemoglobin of 9.2 g/dL, and platelet count of 121,000/µL; along with an elevated Angiotensin Converting Enzyme (ACE) level of 122 units/L. Chest CT was impressive for precarinal lymphadenopathy, diffuse nodular infiltrates with interlobular septal thickening, and hepatosplenomegaly. He subsequently underwent lung, liver, and bone marrow biopsies. Pathology showed non-caseating granulomas from all three sites. Acid fast and periodic acid-Schiff staining on all biopsy specimens failed to reveal any mycobacteria, yeast, or other fungal elements. Further work-up with fungal serologies and antigens, along with T-spot testing were negative. The diagnosis of sarcoidosis was made and he was started on high dose methylprednisolone. His fevers subsequently subsided and his treatment was eventually transitioned to oral prednisone at the time of discharge.

DISCUSSION:

Sarcoidosis can vary in presentation, duration, and severity of symptoms. Though low grade fevers can be a relatively common symptom, our patient presented with fevers as high as 105° F for over seven weeks. To our knowledge there have been very few cases describing isolated high grade fevers as a presenting feature of sarcoidosis.

CONCLUSIONS:

In conclusion, sarcoidosis should always be considered in the differential for patients with high grade fevers of unknown etiology. It must be emphasized that patients with non-caseating granulomas must be ruled out for presence of mycobacterial infection and disseminated fungal disease before the diagnosis of sarcoidosis is made.1) Nunes H, Soler P, Valeyre D. Pulmonary Sarcoidosis. Allergy 2005; 60: 565-582DISCLOSURE: The following authors have nothing to disclose: Anthony Cucci, Khalil Diab, Aliya NoorNo Product/Research Disclosure InformationIndiana University, Indianapolis, IN.

SESSION TYPE: Miscellaneous Cases IIPRESENTED ON: Tuesday, October 23, 2012 at 11:15 AM - 12:30 PM

INTRODUCTION:

PMLNs represent an unusual clinical and pathological condition. These benign nodules are usually asymptomatic and represent incidental findings in surgical specimens or autopsies. Lesions contain monomorphic round cells forming epitheloid nests with immunohistochemical features of meningothelial cells. Nature of this process is thought to be reactive, however pathophysiology has not yet been elucidated.

CASE PRESENTATION:

51 year old female, former smoker, with a history of asthma and chronic allergic sinusitis was referred to pulmonary clinic for evaluation of progressive dyspnea and wheezing. Pulmonary function tests revealed restrictive ventilatory defect, decreased diffusing capacity, and a positive metacholine challenge. Patient also noted an unintentional 12 pound weight loss over six weeks associated with generalized fatigue. Chest computer tomography revealed a right sided thyroid nodule; and multiple bilateral, less than 5 mm, cavitary lung nodules without mediastinal adenopathy. Age appropriate cancer screening including computed tomography of abdomen, mammography, PAP smear and colonoscopy were negative. Detailed autoimmune workup was negative. Thyroid nodule biopsy was negative for malignancy, thyroid function tests were normal. Bronchoscopy with transbronchial biopsies did not reveal the histological diagnosis. Patient was referred to thoracic surgery for a VATS lung biopsy. Results demonstrated PMLNs, which was confirmed by positive staining for progesterone receptors (PR) and epithelial membrane antigen (EMA). Patient's initial symptoms have improved on aggressive asthma and allergy therapy.

DISCUSSION:

Previously reported incidence of PMLNs is 0.07% to 4.9% in autopsy studies. However, recent report suggests the incidence of PMLNs found incidentally in lung biopsy and lobectomy specimens resected for underlying primary lung tumors may be as high as 14% and 48%, respectively. Although PMLNs are morphologically similar to meningiomas, recent molecular pathologic studies showed different lineage-specific genetic pathways. In our case, the indication for invasive diagnostic procedure was dictated by evaluation for malignant, infectious and inflammatory disease; all which can present as multiple small cavitary lung nodules.

CONCLUSIONS:

PMLNs should be considered a differential diagnostic possibility in case of diffuse, bilateral, small cavitary nodules noted on radiographic studies.1) Mukhopadhyay S, El-Zammar O et al. Pulmonary Meningothelial-like nodules. New Insinghts Into a Common but Poorly Understood Entity. Am J Surg Pathol. 2009. 33:487-94.DISCLOSURE: The following authors have nothing to disclose: Frantisek Sandor, Michal Kamionek, Amy Molis, Scott KopecNo Product/Research Disclosure Information, Worcester, MA.

SESSION TYPE: Miscellaneous Case Report Posters IIPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION:

We report the first case of lung cancer with concomitant capnocytophaga infection of the lung mass and mediastinal lymph nodes diagnosed by EBUS-TBNA with associated leukemoid paraneoplastic syndrome. We also highlight potential role of EBUS in diagnosing lung and mediastinal infections.

CASE PRESENTATION:

A 62 year old white male with 47 PPY smoking history, h/o bladder cancer s/p chemoradiation presented with complaints of cough, shortness of breath, mild intermittent hemoptysis and generalized fatigue since 2 months. He had experienced 7 lbs weight loss and night sweats. CT Chest showed a left lower lobe 6x7 cm lung mass with bilateral hilar and mediastinal lymphadenopathy Suspecting possible infection, the patient was started on intravenous ampicillin-sulbactam. Endobronchial ultrasound guided real time sampling (EBUS-TBNA) of the lymph node stations R11, L11 and 4 and 7 as well as lung mass was performed. Biopsies were sent for cultures. He declined chemotherapy and was ultimately referred to hospice.

RESULTS:

WBC 34K, rest of blood counts and chemistry parameters were unremarkable. Sputum and blood cultures remained negative. Cultures from lymph node and lung mass grew copious amount of capnocytophaga species in aerobic media. Cell block examination of biopsy from all lymph node stations and lung mass revealed poorly differentiated lung cancer. Platelet count increased from 330 to 637 over 4 months. WBC remained high in spite of antibiotic treatment although he never developed progressive signs of infection.

DISCUSSION:

To our knowledge, this is the first case of capnocytophaga infection involving mediastinal lymph nodes and lung mass Capnocytophaga is a gram negative rod which usually presents as sepsis and bacteremia in immunocompromised hosts, usually transmitted from dogs and cats. The leukocytosis was due to paraneoplastic syndrome, an entity often complicating adenocarcinoma of lung, thought to be due to increased production of granulocyte-monocyte colony stimulating factor by the malignant cells. This case also highlights the potential role of EBUS in diagnosing lung and mediastinal infections, particularly when other routine methods are unrevealing

CONCLUSIONS:

This case is unique as its first reported case of capnocytophaga infection in lung mass and mediastinal lymph nodes, associated with leukemoid paraneoplastic syndrome and highights potential role of EBUS in diagnosing infections of lung and mediastinum.1) Cooley, L. Capnocytophaga canimorsus: A Serious Infection Masquerading as a Serious Infection. Victorian Infectious Disease Bulletin, Sept 20012) Scully RE, Mark EJ, McNeely WF et al. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 20-1997. A 74-year-old man with progressive cough, dyspnea, and pleural thickening. N Engl J Med 1997;336:1895.903.3) Capnocytophaga canimorsus Infections in human: review of the literature and cases report. Eur. J. Epidemiol. 1996DISCLOSURE: The following authors have nothing to disclose: Amar Panchal, Yousef Shweihat, Ahmad NusairNo Product/Research Disclosure InformationMarshall University, Huntington, WV.

SESSION TYPE: Miscellaneous Case Report Posters IIPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION:

Pancoast's syndrome is characterized by a mass of the superior sulcus of the lung and involvement of the brachial plexus and cervical sympathetic nerves. Apical bronchial carcinoma is the most common cause of Pancoast's syndrome. Of the many other causes reported, infection is a rare one. A case of Pancoast's syndrome, secondary to apical lung abcess is reported.

CASE PRESENTATION:

45-year-old Caucasian female with past medical history including type 2 diabetes, hypertension, dyslipidemia, who presented to ED with increasing malaise, fatigue, weakness, and right-sided chest pain, neck pain and shortness of breath. Her chest x-ray on admission revealed no abnormalities. She was treated with pain medication. Her symptoms persisted. She had a CTA of the Chest which revealed a soft tissue mass located at the right lung apex medially and abuts the trachea, esophagus, and adjacent vertebral body. CT soft tissue of the neck revealed right apical lung mass, which is partially contiguous with the right scalene musculature. Patient complained of right hand numbness and weakness, for which MRI of the neck was done and confirmed earlier findings. Bronchoscopy showed normal endobronchial tree, and an ultrasound guided transbronchial needle aspiration of the right upper lobe mass was negative for malignant cells. Aspirate from the mass grew Streptococcus Viridans. Patient treated wit antibiotics and underwent anterior neck exploration of the right apical lung mass to relieve her brachioplexopathy

DISCUSSION:

Pancoast's syndrome includes Horner's syndrome, atrophy of he hand muscles and shoulder, axilla or arm pain. This syndrome is caused by an apical thoracic lesion, most commonly a bronchogenic carcinoma. Causes of Pancoast's syndrome not related to malignancy are recognized. Among the benign conditions causing Pancoast's syndrome, infections are extremely rare. A wide variety of organisms were identified but no single organism could be labeled as the most prevalent. Overall, bacteria (54.8%) seem to be the most common organisms with S aureus being the most frequent of these. Infection is a treatable etiology of Pancoast's syndrome.

CONCLUSIONS:

Infection is a treatable etiology of Pancoast's syndrome.1) Pancoast's Syndrome Secondary to Infectious Etiologies: A Not So Uncommon Occurrence Heath D. White, DO, MS, Bobbie Ann A. White, MA, Carl Boethel, MD and Alejandro C. Arroliga, MD. Send to: Pancoast's syndrome secondary to infectious etiologies: a not so uncommon occurrence. White HD, White BA, Boethel C, Arroliga AC. Am J Med Sci. 2011 Apr;341(4):333-6.2) Mehrotra A, Raj A, Tripathi K. Apical pulmonary tuberculosis presenting with Pancoast syndrome like features. J Assoc Physicians India 2001;49:380-13) Comet R, Monteagudo M, Herranz S, et al. Pancoast's syndrome secondary to lung infection with cutaneous fistulisation caused by Staphylococcus aureus. J Clin Pathol 2006;59:997- 8.DISCLOSURE: The following authors have nothing to disclose: Anas AlsadiNo Product/Research Disclosure Information, Temple, TX.

SESSION TYPE: Miscellaneous Global Case Report PostersPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION:

We present a case of 33 year old male, working in an oil rig as a welder, who had acute onset breathlessness at work place. Without a detailed occupational history and knowledge of disease such patients would have been misdiagnosed.

CASE PRESENTATION:

33 year male , oil rig welder, presented on Monday evening to our emergency department with acute onset breathlessness after four hours of welding. He also complained of fever, throat pain, malaise, fatigue, odd taste in mouth, two episodes of vomiting, nausea, and severe cough. He is a non smoker and has no history of previous illness. On examination he could not complete a full sentence. His blood pressure was normal with pulse rate of 100 per minute, respiratory rate of 28 per minute and oxygen saturation was 90% on room air. Blood gas analysis showed partial pressure of oxygen of 60mmHg. Other clinical examination, and routine lab workup was unremarkable. Detailed occupational history revealed that he was working on galvanised steel and hence exposed to zinc oxide fumes. A diagnosis of metal fume fever was made and was managed conservatively with oxygen, inhaled bronchodilators, inhaled and oral steroids. He was well within 12 hours, was observed and discharged after two days.

DISCUSSION:

Metal fume fever (MFF) is an occupational disease caused by inhalation of metal fumes. It is also called 'galvaniser's poisoning', or 'Monday morning fever'. This is because acute tolerance develops during daily exposure and the weekend away from work contributes to the syndrome's reoccurrence when welding is recommenced on a Monday. Galvanized metal is steel that is coated with zinc oxide to protect the steel from oxidation, corrosion, and its eventual weakening. Workers engaged in heating or welding of galvanized materials are at risk of exposure to zinc oxide fumes. The high temperatures cause the metals to liberate fumes that include the oxides of the metal and other combustion products. Their inhalation can induce MFF. The Occupational Safety and Health Administration (OSHA) has set a legal limit of five milligrams of zinc oxide fume per cubic meter of air (5mg/m3) averaged over an 8-hour work shift. The National Institute for Occupational Safety and Health (NIOSH) recommends 5 mg/m3 averaged over a work shift up to 10-hours per day, 40-hours per week, with a short-term exposure limit of 10mg/m3 averaged over a 15- minute period (1). The pathophysiology is unclear. There is evidence of an exposure dependent neutrophil alveolitis in association with tumour necrosis factor a, interleukin 6, and interleukin 8 cytokine releases from pulmonary cells. The short-lived tolerance occurs because of induction of metallothionein protein synthesis. These proteins bind to heavy metals preventing toxic metal accumulation (2) Symptoms usually appear 3 to 10 hours after exposure and include fever, chills, headache, myalgia, fatigue, dyspnea, cough, excessive thirst, metallic taste, and excessive salivation. Neuthrophilic leukocytosis may be found on laboratory tests. The chest roentgenogram is usually normal. Recovery is spontaneous, within 24 to 48 hours. Treatment is mainly supportive, consisting of analgesics, antipyretics, and rest. There are no known late complications (3). Corticosteroids have not been shown to be of any benefit (1). The common differential is influenza, occupational asthma, acute lung injury or chemical pneumonitis. The self limiting course, normal radiological, pathological findings and occupational history differentiates it from other conditions.

CONCLUSIONS:

MFF is an acute self limiting occupational syndrome. Its importance is that it often occurs in remote areas where emergency aid is negligible and yet it is easy to diagnose and manage. Increasing public and physician awareness, adequate protection, reduced time of exposure to metal fumes, will reduce the occurrence of this disease.1) J La State Med Soc VOL 161 November/December 20092) Emerg Med J 2002;19: 268-2693) Waldron HA. Non-neoplastic disorders due to metallic, chemical and physical agents. In: Raymond Parkes W. Occupational lung disorders. Oxford: Butterworth Heinemann, 1994:593-643DISCLOSURE: The following authors have nothing to disclose: Sanjeev Mehta, Ruchira Shende, Bhavin SampatNo Product/Research Disclosure InformationLilavati Hospital and Research Centre, Mumbai, India.

The differences in non-motor symptoms (NMS) along with the duration of Parkinson's disease (PD) in Chinese populations remain largely unknown. A total of 616 PD patients consisting of 189 patients with disease duration of less than 2years, 239 patients with duration between 2 and 5years, 122 patients with duration between 5 and 10years, and 66 patients with duration more than 10years from Southwest China were studied. Each patient was assessed using the non-motor symptoms scale (NMSS). The mean affected number of NMS was 8.8±5.8, and the mean total NMSS score was 36.3±33.2. The mean affected number of NMS and the mean total NMSS score increased with the disease progression. The following symptoms became more common and severe with the disease duration: falls due to fainting, fatigue, difficulty falling asleep, restless legs, perceptual problem/hallucination domain (i.e., "hallucinations," "delusions," and "double vision"), gastrointestinal domain (i.e., "dribbling saliva," "swallowing," and "constipation"), urinary domain (i.e., "urgency," "frequency," and "nocturia"), and miscellaneous domain (i.e., "excessive sweating"). However, some symptoms such as mood/apathy pleasure, sexual dysfunction, and others remained stable. NMS are prevalent in all disease duration of the Chinese PD patients. Although the mean affected number of NMS and NMMS score increased with the disease duration, NMS progression is symptom specific.

The clinical manifestations of drug-induced parkinsonism (DIP) and Parkinson disease (PD) are nearly indistinguishable, making it difficult to differentiate DIP from PD, especially in the early stages. We compared non-motor symptoms between patients with DIP and those with drug-naïve PD in the early stages using the Non Motor Symptoms Scale (NMSS).We prospectively enrolled 28 patients with DIP, 35 patients with drug-naïve PD, and 32 controls with no history of neurological diseases or related medical problems. We investigated demographic characteristics, medical and drug history, parkinsonian motor symptoms, and non-motor symptoms. We used the NMSS to evaluate non-motor symptoms in all patients.The total NMSS scores were higher in patients with PD than those with DIP, as were the scores for certain domains, including the cardiovascular, sleep/fatigue, urinary, sexual, and miscellaneous domains. When controlling for age and gender, the correlation analysis revealed that scores for urinary symptoms (urgency, frequency and nocturia), sleep disturbances (daytime sleep, restless legs), concentration, taste or smell were significantly associated with PD.Our data suggest that non-motor symptoms, particularly urinary symptoms, excessive daytime sleepiness, restless leg syndrome, attention deficit and hyposmia may be helpful to differentiate between DIP and PD in the early stages.

Imatinib mesylate is a receptor kinase inhibitor approved by the Food and Drug Administration for the treatment of malignant metastatic and/or unresectable gastrointestinal stromal tumors and chronic myelogenous leukemia. Although imatinib is generally well tolerated, certain adverse drug reactions are common. These include gastrointestinal side-effects such as diarrhea, nausea and vomiting, as well as hematological side-effects and other miscellaneous side-effects such as fatigue, edema, dermatitis and dyspnea. We present a previously unreported adverse effect of imatinib, gastric antral vascular ectasia, in a 74-year-old woman with gastrointestinal stromal tumor in remission treated with adjuvant imatinib. Endoscopy performed prior to starting imatinib showed normal gastric mucosa, but 8 months after starting imatinib showed diffuse gastric inflammation. Repeat endoscopy 1 month after discontinuing imatinib showed significant improvement in gastric inflammation.

Paediatric pulmonary hypertension, is an important cause of morbidity and mortality, and is insufficiently characterised in children. The Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension (TOPP) registry is a global, prospective study designed to provide information about demographics, treatment, and outcomes in paediatric pulmonary hypertension.Consecutive patients aged 18 years or younger at diagnosis with pulmonary hypertension and increased pulmonary vascular resistance were enrolled in TOPP at 31 centres in 19 countries from Jan 31, 2008, to Feb 15, 2010. Patient and disease characteristics, including age at diagnosis and at enrolment, sex, ethnicity, presenting symptoms, pulmonary hypertension classification, comorbid disorders, medical and family history, haemodynamic indices, and functional class were recorded. Follow-up was decided by the patients' physicians according to the individual's health-care needs.362 of 456 consecutive patients had confirmed pulmonary hypertension (defined as mean pulmonary artery pressure ≥25 mm Hg, pulmonary capillary wedge pressure ≤12 mm Hg, and pulmonary vascular resistance index ≥3 WU/m(-2)). 317 (88%) patients had pulmonary arterial hypertension (PAH), which was idiopathic [IPAH] or familial [FPAH] in 182 (57%), and associated with other disorders in 135 (43%), of which 115 (85%) cases were associated with congenital heart disease. 42 patients (12%) had pulmonary hypertension associated with respiratory disease or hypoxaemia, with bronchopulmonary dysplasia most frequent. Finally, only three patients had either chronic thromboembolic pulmonary hypertension or miscellaneous causes of pulmonary hypertension. Chromosomal anomalies, mainly trisomy 21, were reported in 47 (13%) of patients with confirmed disease. Median age at diagnosis was 7 years (IQR 3-12); 59% (268 of 456) were female. Although dyspnoea and fatigue were the most frequent symptoms, syncope occurred in 31% (57 of 182) of patients with IPAH or FPAH and in 18% (eight of 45) of those with repaired congenital heart disease; no children with unrepaired congenital systemic-to-pulmonary shunts had syncope. Despite severe pulmonary hypertension, functional class was I or II in 230 of 362 (64%) patients, which is consistent with preserved right-heart function.TOPP identifies important clinical features specific to the care of paediatric pulmonary hypertension, which draw attention to the need for paediatric data rather than extrapolation from adult studies.Actelion Pharmaceuticals.

Differences in the expression of non-motor symptoms (NMS) by Parkinson's disease (PD) patients may have important implications for their management and prognosis. Gender is a basic epidemiological variable that could influence such expression. The present study evaluated the prevalence and severity of NMS by gender in an international sample of 951 PD patients, 62.63% males, using the non-motor symptoms scale (NMSS). Assessments for motor impairment and complications, global severity, and health state were also applied. All disease stages were included. No significant gender differences were found for demographic and clinical characteristics. For the entire sample, the most prevalent symptoms were Nocturia (64.88%) and Fatigue (62.78%) and the most prevalent affected domains were Sleep/Fatigue (84.02%) and Miscellaneous (82.44%). Fatigue, feelings of nervousness, feelings of sadness, constipation, restless legs, and pain were more common and severe in women. On the contrary, daytime sleepiness, dribbling saliva, interest in sex, and problems having sex were more prevalent and severe in men. Regarding the NMSS domains, Mood/Apathy and Miscellaneous problems (pain, loss of taste or smell, weight change, and excessive sweating) were predominantly affected in women and Sexual dysfunction in men. No other significant differences by gender were observed. To conclude, in this study significant differences between men and women in prevalence and severity of fatigue, mood, sexual and digestive problems, pain, restless legs, and daytime sleepiness were found. Gender-related patterns of NMS involvement may be relevant for clinical trials in PD.