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

INTRODUCTION:

Diffuse alveolar hemorrhage (DAH) is a potentially life threatening condition, and requires prompt management. Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of DAH. Here we describe a case of IPH with rare associations in a young adult.

CASE PRESENTATION:

19 years old male from Northern India presented with recurrent haemoptysis (2 tsf/day, for 3 years) with generalized weakness, and progressive dyspnea (MRC grade II). He had previously been diagnosed as a case of iron deficiency anemia (Hb 2 g/dl), and received blood transfusion and hematinics. There was no history of joint pains, rash, photosensitivity, alopecia, oral ulcers, Raynauds phenomenon, dysuria, bleeding from other sites , recurrent chest infections, or tuberculosis. On examination he was pale with pulse rate 110/min; BMI was 18 kg/m2. Systemic examination was within normal limits . His X Ray Chest showed cardiomegaly. ECG showed sinus tachycardia with nonspecific interventricular conduction delays, with T wave inversion (I, aVL, V4-V6). Troponins and CKMB were not raised. His hemoglobin was 7.8 g/dl; peripheral smear was suggestive of iron deficiency. Liver, renal functions and ABG (PO2 89 mm Hg, FiO2 0.21) were normal. CECT Chest revealed bilateral ground glass opacities. Pulmonary function testing showed mild restriction (FVC 3.05 L, 68 % predicted), with increased diffusion capacity for carbon monoxide (DLCO). Bronchoalveolar lavage (BAL) and transbronchial lung biopsy (TBLB) revealed hemosiderin laden macrophages, suggestive of IPH. Bacterial culture, AFB, fungal cultures and other staining were negative. Work up for connective tissue including RF, ANA, ANCA were normal . Echocardiography revealed dilated cardiomyopathy (DCMP) with severe LV dysfunction (LVEF 25%) . On further evaluation he gave history of recurrent diarrhea in childhood, not foul smelling, without any blood or mucus, though was asymptomatic since last 5 years. Serum Anti Tissue Transglutaminase antibody titres were strongly positive (291.66 IU/ml, normal range <30 IU/ml). Duodenal biopsy showed subtotal villous atrophy (Marsh Grade IIIB), confirming the diagnosis of celiac disease (CD). A final diagnosis of IPH with CD (Lane Hamilton Syndrome) with DCMP was made. Patient was started on gluten free diet, beta blockers & diuretics. In a short follow up of two months, patient has shown symptomatic improvement. Hemoptysis has decreased, hemoglobin has improved to 10.2 g/dl. A repeat ECHO has shown LVEF of 35%.

DISCUSSION:

DAH is bleeding from pulmonary microvasculature. A prompt work up for vasculitides, connective tissue disorders and other causes with symptomatic treatment and immunosuppression is required.1 IPH is a rare cause of DAH, with an incidence of 0.2-2.0/million per year. The association between IPH and CD was first described by Lane and Hamilton in 1971.1 28 cases have been reported so far, with good response to gluten free diet, and few requiring immunosuppression. CD has been reported in 4-5.8% patients with DCMP, with improvement following gluten free diet. Selenium and carnitine deficiency may also be contributory. Screening for CD has been recommended in patients with DCMP.2 This is the first case report of Lane Hamilton syndrome with DCMP in adults. Two other cases have been reported in pediatric age group. Both cases required gluten free diet alongwith immunosuppression. 3

CONCLUSIONS:

Screening for CD is important in a case of IPH. DCMP is a rare third association. It is potentially reversible with gluten free diet.1) R. Lazor: Chapter 2. Alveolar haemorrhage syndromes. European Respiratory Society Monograph 54: 15-31, 20112) Lodha A, Haran M, Hollander G, et al: Celiac disease associated with dilated cardiomyopathy. South Med J 102:1052-4, 20093) Narula N, Rawal P, Kumar RM, Ram Thapa B. Association of celiac disease with cardiomyopathy and pulmonary hemosiderosis. J Trop Pediatr 56: 201-3, 2010DISCLOSURE: The following authors have nothing to disclose: Gopi Khilnani, Pawan Tiwari, Alpesh Goyal, Lavleen Singh, Arun Arora, Randeep GuleriaNo Product/Research Disclosure InformationAll India Institute of Medical Sciences, New-Delhi, India.

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

INTRODUCTION:

Behcet's disease is a rare vasculitic disease usually associated with ulcers involving the oral and genital mucosa with rare pulmonary involvement.

CASE PRESENTATION:

A 23-year-old man was admitted with complaints of a gradually enlarging left supraclavicular mass and worsening shortness of breath of two months duration. Review of systems at the time of presentation revealed the occasional presence of oral ulcers and acne like lesions . Physical examination was significant for increased facial plethora, dilated veins on the chest and firm left supraclavicular lymph nodes. A chest X-ray, followed by Ct scans of the chest (Fig 1 & 2) and the neck revealed a right pleural effusion and thromboses of the superior venacava, internal jugular and subclavian veins. Thoracentesis revealed a chylous effusion that was exudative in nature (Triglyceride concentration- 799mg/dl, protein of 3.6 gm/dl and LDH of 947 U/L). Pleural fluid analysis was negative in terms of malignant cells or infectious agents. Biopsy of the lymph nodes revealed only benign cellular elements. The patient also developed pustular rash on his trunk and oral ulcers, biopsy of which demonstrated superificial and perivascular inflammation consistent with behcet's disease. He was then started on systemic anticoagulation, cyclophosphamide and prednisone with marked improvement in systemic symptoms. However, in spite of triglyceride restriction, the patient had persistent pleural effusions ultimately needing thoracic duct ligation and partial decortication.

DISCUSSION:

Behcet's disease is a chronic multi-system inflammatory disease diagnosed based on the following criteria: oral ulcers and at least any two of the following: recurrent genital ulceration; uveitis, skin lesions including erythema nodosum, pseudofolliculitis or papulopustular lesions and positive pathergy test. Chylothorax, defined as triglyceride concentration in the pleural fluid >110mg/dl is a very rare presenting manifestation of Behcet's disease with only 16 reported cases so far in the medical literature. Thrombosis of the left subclavian and the innominate vein obstructs the drainage of the thoracic duct, with subsequent increase in the intraluminal pressure of the same and the back pressure forcing leakage of chyle from the pleural lymphatics. Management can be quite challenging. Aside from anticoagulation and immunosuppression for the underlying disease, treatment of the chylothorax may require thoracic duct ligation or even decortications, as in our case

CONCLUSIONS:

We reiterate that behcet's disease should be considered as a cause in a new presentation of chylothorax.1) Husain SJ, Sadiq F, Zubairi AB, Khan JA. Massive unilateral chylous pleural effusion: a rare initial presentation of Behcet's disease. Singapore Med J. 2006 Nov;47(11):978-80.2) Coplu L, Emri S, Selcuk ZT, et al. Life threatening chylous pleural and pericardial effusion in patient with Behcet's syndrome. Thorax 1992;47:64-5.DISCLOSURE: The following authors have nothing to disclose: Sreedivya Chava, Subhraleena Das, Sujith Cherian, Wasim Hamarneh, Pramod Pantangi, Robert LenoxNo Product/Research Disclosure InformationSUNY Upstate Medical University, Syracuse, NY.

SESSION TYPE: Pleural Cases IIPRESENTED ON: Wednesday, October 24, 2012 at 11:15 AM - 12:30 PM

INTRODUCTION:

The diagnosis and management of an inflammatory multisystem vasculitic disorder can be challenging in a critically ill patient.

CASE PRESENTATION:

A 23-year-old male presented with fevers, night sweats, a left neck mass and face and upper extremity swelling. He had no prior history of oral or genital ulcers. CT-thorax revealed bilateral pleural effusions, mediastinal and cervical LAP, with right brachiocephalic and subclavian vein occlusion and a clot in the SVC. Bone marrow biopsy and peripheral smears were nonrevealing. Pleural fluid analysis suggested chylothoraces. Despite extensive collaterals, mediastinoscopy was attempted but abandoned due to extreme mediastinal fibrotic changes. Right VATS for mediastinal biopsies revealed significant fibrosis. Postoperatively steroids were used to ameliorate airway edema and facilitate extubation. Left supraclavicular LN and EBUS guided mediastinal LN biopsies were also negative for malignancy. Repeat CT revealed a new left ventricular free wall cardiac mass adjacent to the atrioventricular groove. Cardiac MRI showed a well-defined 4.5cm spherical mass in the AV groove consistent with a thrombosed coronary artery pseudo-aneurysm. Shortly after weaning steroids, he became febrile with new oral aphthous ulcers as well as anterior chest wall ulcers. Broad spectrum antibiotics were initiated. Pleural fluid, oral ulcer swab and blood cultures were negative. Oral ulcer biopsy revealed superficial and perivascular inflammation. His clinical presentation was consistent with Behcet's disease(BD), with the presentation of ulcers, which were masked initially by the steroids. He was started on cyclophosphamide with steroids. He subsequently underwent ligation of the thoracic duct as well as thoracic duct and tributary platinum coil embolization. Currently, his effusions remain small, he no longer has signs of SVC syndrome, and the cardiac mass has reduced in size as he continues to be on immunosuppressive agents.

DISCUSSION:

Behcet's disease is a systemic inflammatory vasculitis of unknown etiology, characterized by relapsing episodes of orogenital ulcers, skin lesions, ocular lesions and rarely vascular, gastrointestinal and neurological involvement. Pulmonary involvement is uncommon (1-7.7% cases) and presents as thromboangiitis, pulmonary artery aneurysms, recurrent pneumonias and BOOP. Chylothorax as an initial manifestation of BD is extremely rare and is hypothesized to be secondary to large vein occlusion, such as SVC thrombosis causing lymphatic obstruction. The spectrum of cardiac diseases may include pericarditis, coronary artery stenosis or aneurysm, myocarditis, cardiomyopathy, valvular pathology and aneurysm of aorta and its branches. There are no laboratory findings specific for BD.

CONCLUSIONS:

The diverse clinical manifestations of BD can be challenging especially with an atypical presentation.1) Mendes D, et al. Behcet's disease--a contemporary review. J Autoimmun.2009;32(3-4):178-88.DISCLOSURE: The following authors have nothing to disclose: Pankaj Mehta, Heather Smith, Fatme Allam, Leslie Kohman, Darren Tabechian, Christian Peyre, David Sugarbaker, Marcelo DaSilva, Barbara RobinsonNo Product/Research Disclosure InformationSUNY Upstate Medical University, Liverpool, NY.

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

INTRODUCTION:

Levamisole was initially developed as an antihelminthic and an immunomodulatory drug, [1,2] but has since been discontinued due to serious side effects, such as vasculitic skin necrosis and agranulocytosis.[3] Levamisole is now used to cut cocaine - up to 70% of cocaine seized in the United States is contaminated with levamisole. Here, we report a non-fatal case of levamisole-associated vasculopathy.

CASE PRESENTATION:

A 48-year old black woman with type II diabetes mellitus, hypertension and recent cocaine use was assessed for a skin rash. It first appeared on her right ear, and then spread over a 3-day period to involve her left ear, her maxillae, her right breast, her abdomen and the lateral aspects of her upper arms and thighs. The rash began as an eythematous macule, which enlarged to form a purpuric center with an erythematous border. The rash on her abdomen encircled her umbilicus, with a radial measurement of 10-12 cm. All her skin rashes were associated with mild pruritis and tenderness. Associated symptoms included mild chest pain, shortness of breath, and occasional chills. Physical exam confirmed the skin lesions - no oral or vaginal ulcers were present. Laboratory tests revealed leucopenia, anemia, and a positive urine drug screen for cocaine. The initial differential diagnoses were an autoimmune vasculitis and a fixed drug eruption, due to lisinopril, hydrochlorathiazide, metformin, or gabapentin. Initially, all her medications were stopped, oral and topical steroid therapy was commenced, and a 5 mm punch biopsy of an early lesion on her lateral thigh was performed. Histopathology showed fibrin thrombi within the vessels of the superficial venous plexus, as well as a minimal perivascular lymphocytic infiltrate. These findings, in the context of recent cocaine use and the characteristic skin rash, are characteristic of levamisole-associated vasculopathy. Thus, we were able to counsel the patient to avoid cocaine and to improve adherence to her antihypertensive and diabetic medications.

DISCUSSION:

Our case emphasizes the importance of a thorough clinical interview and clinico-pathological correlation: recreational cocaine use may result in unusual clinical manifestations, such as a purpuric skin rash.

CONCLUSIONS:

When confronted by a skin rash in a patient who uses cocaine, a clinician should always consider levamisole-induced vasculopathy.1) Lionel ND, Mirando EH, Nanayakkara JC, Soysa PE. Levamisole in the treatment of ascariasis in children. Br Med J. 1969 Nov 8;4(5679):340-1.2) Perk K, Chirigos MA, Fuhrman F, Pettigrew H. Some aspects of host response to levamisole after chemotherapy in a murine leukemia. J Natl Cancer Inst. 1975 Jan;54(1):253-6.3) Rongioletti F, Ghio L, Ginevri F, Bleidl D, Rinaldi S, Edefonti A, Gambini C, Rizzoni G, Rebora A. Purpura of the ears: a distinctive vasculopathy with circulating autoantibodies complicating long-term treatment with levamisole in children. Br J Dermatol. 1999 May;140(5):948-51.DISCLOSURE: The following authors have nothing to disclose: Dominique Pepper, Andrea McCann, Lance Atchley, Jericho BellNo Product/Research Disclosure InformationUMMC Internal Medicine, Jackson, MS.

Recurrent aphthous ulcers are a common oral mucosal condition affecting about 20 percent of the general population and characterized by recurrent ulcers on the nonkeratinized mucosa. The majority of patients with recurrent aphthous ulcers tend to be healthy, although appropriate management of recurrent aphthous ulcers includes ruling out an oral manifestation of systemic disease. Management involves controlling active disease locally to reduce pain and maintain function and reducing the frequency and severity of recurrences, with a goal of a prolonged remission.

Gingival adenoid cystic carcinoma (ACC) is a rare malignancy. We describe the diagnosis and treatment of a 43 year-old woman who presented with a persistent oral ulcer for approximately 1 year, and subsequent pain in the left posterior maxillary region. Clinical examination revealed an ulcer in the left upper molar gingiva, with swelling in the region from the second premolar to the third molar. X-ray images demonstrated the involvement of the maxillary alveolar bone. The histopathological and immunohistochemical features were diagnostic of ACC. ACC is often presented as a gingival lesion; thus, it may easily be neglected by patients. The identification of this tumor using specific pathological analyses prevents misdiagnosis and enables clinicians to determine the appropriate treatment. In this case, no recurrence or distant metastasis was observed after 2 years of follow-up.

A 40-year-old manual labourer presented with easy fatiguability, recurrent vomiting and loss of weight of 3 months, duration. Upon examination, there was significant axillary and cervical lymphadenopathy. No pallor, icterus or clubbing was evident. There was generalised hyperpigmentation and multiple oral ulcers. The blood pressure 90/60 mm Hg in the right upper limb in the supine position. Investigations showed a low serum cortisol. Mantoux test was strongly positive (20 mm).A fine needle aspiration biopsy of the cervical lymph node revealed reactive changes. Bone marrow aspiration and biopsy were normal. Cervical lymph node biopsy showed caseating granulomas suggestive of tuberculous lymphadenitis. A CT scan of the abdomen showed bilaterally enlarged adrenal glands with hypodense areas suggestive of necrosis. He was diagnosed with extrapulmonary disseminated tuberculosis with tuberculous adrenalitis. He was started on directly observed therapy (DOTS) for disseminated tuberculosis and 40 mg of prednisolone. He is improving with treatment.

Objective:

To assess oral cancer awareness, its associated factors and related sources of information among a selected group of Malaysians.

Methods:

A cross-sectional survey was conducted on all Malaysian ethnic groups aged ≥15 years old at eight strategically chosen shopping malls within a two week time period. Data were analysed using chi-square tests and multiple logistic regression. Significance level was set at α<0.05.

Results:

Most (84.2%) respondents had heard of oral cancer. Smoking was the most (92.4%) recognized high risk habit. Similar levels of awareness were seen for unhealed ulcers (57.3%) and red/white patches (58.0%) as signs of oral cancer. Age, gender, ethnicity, marital status, education, occupation and income were significantly associated with oral cancer awareness (p<0.05).

Conclusions:

There was a general lack of awareness regarding the risk habits, early signs and symptoms, and the benefits of detecting this disease at an early stage. Mass media and health campaigns were the main sources of information about oral cancer. In our Malaysian population, gender and age were significantly associated with the awareness of early signs and symptoms and prevention of oral cancer, respectively.

Background:

Osteonecrosis is a benign condition characterised by necrotic exposed bone, and is associated with bisphosphonate use. Osteonecrosis of the external auditory canal is rare, with only a few reported cases. Method: Two case reports of temporal bone osteonecrosis are presented.

Results:

A 64-year-old man with a history of immunoglobulin G kappa multiple myeloma developed a right external auditory canal ulcer 6 years after commencement on clodronate. A 72-year-old woman taking alendronate for osteoporosis, initially diagnosed and treated for right-sided otitis externa, was found to have underlying exposed bone in the right external auditory canal, with a computed tomography scan confirming destruction of the temporal bone.

Conclusion:

With increasing use of both oral and intravenous bisphosphonates in the community for benign conditions such as osteoporosis and for malignant conditions such as breast cancer and multiple myeloma, the diagnosis of bisphosphonate-associated osteonecrosis should always be considered in patients with a temporal bone lesion, and a relevant drug history taken.

BACKGROUND:

SBR759, an iron (III)-based oral phosphate binder, was developed for the treatment of hyperphosphataemia in chronic kidney disease stage V patients receiving maintenance renal replacement therapy (RRT). Serum phosphate-lowering efficacy and dose response of SBR759 (3-, 6-, 9- and 12-g/day doses) were compared with placebo.

METHODS:

Japanese patients with hyperphosphataemia (P ≥ 5.5 mg/dL [≥1.78 mmol/L]) receiving maintenance RRT (N = 63) were randomised to receive either SBR759 (3-, 6-, 9-, 12-g/day dose) or placebo (12-g/day dose) for 4 weeks. The primary endpoint was change from baseline in 72-h post-dialysis serum phosphate levels at week 4 for different doses of SBR759 versus placebo. Secondary endpoints were change from baseline in serum phosphate levels and dose-dependent efficacy of SBR759 during the 4-week treatment period.

RESULTS:

SBR759 showed significant reduction in serum phosphate levels compared with placebo at week 4, demonstrating a significant linear dose response (P < 0.001). Incidence of adverse events was comparable between the SBR759 treatment groups (7/13 and 5/12 in the 3- and 12-g/day groups, respectively, and 8/13 in the 6- and 9-g/day groups) and was 6/12 in the placebo group. Discoloured faeces and diarrhoea were the most frequently reported adverse events. Two serious adverse events were reported-one in the SBR759 3-g/day group (1/13, skin ulcer) and one in the SBR759 12-g/day group (1/12, arthralgia).

CONCLUSIONS:

SBR759 showed significant phosphate-lowering efficacy and dose-dependent response compared with placebo in patients with chronic kidney disease receiving RRT.