Manifestation Of Systemic Disease Research Articles

Not Your Typical Case of Lupus Pneumonitis

SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Pulmonary manifestations of Systemic Lupus Erythematosus (SLE) are varied and can affect any component of the respiratory tree. They include acute lupus pneumonitis (ALP), organizing pneumonia, interstitial lung disease and diffuse alveolar hemorrhage. We describe a unique case of lupus pneumonitis presenting as dense bilateral consolidation. CASE PRESENTATION: A 23-year-old African American female presented with rash, fatigue, arthralgias and dry cough. She was febrile, hypotensive, tachycardic and tachypnic. Physical exam was significant for oral ulcers, coarse rhonchi in bilateral lung fields, scaly hyperpigmented plaques on her face and erythematous macules on the lower extremities. She decompensated rapidly requiring intubation and was admitted for further evaluation. Initial laboratory analysis were significant for pancytopenia, mild transaminitis, a protein gap and proteinuria. Chest imaging showed dense consolidation in the lower lobes (Image 1). The patient underwent a bronchoscopy, which noted clear airways without pus or blood; sequential alveolar aliquots were not consistent with alveolar hemorrhage. An extensive infectious workup was negative; however, autoimmune workup was positive (Table 1). A clinical diagnosis of SLE was made. The patient failed to improve on antibiotics; low dose steroids were added with no change. Acute lupus pneumonitis was considered given the negative infectious workup and the persistent lower lobe opacities. She was started on pulse dose steroids with rapid radiographic resolution and clinical improvement, facilitating extubation. DISCUSSION: Acute lupus pneumonitis (ALP) is a life-threatening syndrome occurring in 1—4% of patients with SLE.1 The diagnosis can be elusive as the clinical presentation is non-specific. While chest x-ray can show patchy acinar infiltrates, usually with a lower lung zone predominance, dense bilateral consolidation has not been previously well described.2 Additionally, our patient did not have evidence of diffuse alveolar hemorrhage on bronchoscopy nor findings consistent with organizing pneumonia, which can both present with patchy alveolar opacities. She only improved after initiation of pulse dose steroids for ALP. CONCLUSIONS: Clinicians should consider ALP in patients presenting with dense consolidations, negative infectious workup and clinical suspicion of SLE. The overall prognosis for ALP is poor with 50-90% mortality despite treatment; early diagnosis is of utmost importance to provide the best chance of recovery.3 Reference #1: Torre, Olga, and Sergio Harari. “Pleural and Pulmonary Involvement in Systemic Lupus Erythematosus.” Pulmonary Involvement in Systemic Disease (2011). Reference #2: Kim, Jeung Sook et al. “Thoracic Involvement of Systemic Lupus Erythematosus: Clinical, Pathologic, and Radiologic Findings.” Journal of Computer Assisted Tomography 24.1 (2000): 9-18. Reference #3: Murin, Susan et al. “Pulmonary Manifestations of Systemic Lupus Erythematosus.” Clinics in Chest Medicine 19.4 (1998): 641-65. DISCLOSURE: The following authors have nothing to disclose: Bhavika Kaul, Simon Yau, Venkata Bandi No Product/Research Disclosure Information

The Second Greatest Masquerader? Sarcoidosis Presenting as a Nodular Lung Mass Mimicking Malignancy

SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Sarcoidosis is a systemic granulomatous disease that involves the lungs in 90% of cases. Pulmonary Sarcoidosis can present with the classic pattern of hilar adenopathy and parenchymal ground glass opacities, but also with a wide range of more unusual patterns. We present the case of a young female patient with a lung mass on CT scan that was initially concerning for malignancy but was ultimately found to be Sarcoidosis. CASE PRESENTATION: A 44 year old African American woman with a past medical history of diabetes, obesity, and asthma presented with shortness of breath for one month. She had been treated with Prednisone for a presumed asthma exacerbation; her breathing improved initially but deteriorated after being tapered off Prednisone. She ultimately came to our facility as her respiratory status worsened and she developed night sweats and unintentional weight loss. Chest radiograph revealed a mass-like opacity in the left lower lobe. CT Chest revealed a left lower lobe mass and diffuse hilar lymphadenopathy, which was concerning for malignancy. Initial CT-guided biopsy of the lung mass revealed granulomatous disease consistent with Sarcoidosis and without evidence of malignancy, fungal infection, or infection with acid fast bacilli. However, given the atypical presentation and ongoing concern for malignancy, bronchoscopy and then mediastinoscopy were performed. These both confirmed the diagnosis of Sarcoidosis. Prednisone was initiated but management has been challenging due to frequent episodes of hyperglycemia requiring insulin therapy. Given her robust response, Prednisone has been continued despite poor glucose control. Repeat imaging after 3 months shows a significant decrease in the size of the left lower lobe lung mass. DISCUSSION: Sarcoidosis is known to mimic neoplastic, infectious, or other granulomatous conditions, but dominant nodular mass-like lesions resembling neoplasm are a rare manifestation. Such an atypical presentation requires thorough investigation to rule out other causes. Repeated biopsy on two non-contiguous sites is recommended. In our patient there was initially a high clinical suspicion for malignancy, so the team did not feel comfortable relying on a single biopsy result. After two additional biopsies of other sites the diagnosis of Sarcoidosis was confirmed. Because Sarcoidosis can masquerade as so many conditions, it may be overlooked or disregarded in the search for alternative diagnoses. It is therefore imperative for physicians to keep a high clinical suspicion for Sarcoidosis to prevent delay in diagnosis and treatment. Steroid-sparing agents such as Rituximab can also be considered if patients are intolerant to first-line steroid therapy. CONCLUSIONS: Sarcoidosis can present with a wide range of unusual patterns and a high clinical suspicion is important to prevent delay in management. Reference #1: Baughman RP, Culver DA, Judson MA. A Concise Review of Pulmonary Sarcoidosis, Am J Respir Crit Care Med.2011;183:573-81 DISCLOSURE: The following authors have nothing to disclose: Wing-Tai Kong, Changwan Ryu, Afshin Ahoubim, Christopher Erb No Product/Research Disclosure Information

Diffuse Alveolar Hemorrhage Due to Rheumatic Fever

SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is a potentially devastating complication of myriad pulmonary and cardiovascular diseases. CASE PRESENTATION: A 43 year-old woman presented with fever, dyspnea and polyarthralgias for several weeks. Physical exam was notable for temperature 102.7, IV/VI systolic murmur at the apex, and right-sided crackles. There was tender synovitis of the bilateral wrists and knees. Chest radiograph showed diffuse infiltrates, right greater than left. Electrocardiogram showed PR-interval prolongation (0.25ms). She was treated with antibiotics for pneumonia, but developed hypoxic respiratory failure requiring mechanical ventilation. Bronchoscopy revealed progressively bloody return. Echocardiography revealed severe mitral regurgitation with ruptured chordae tendinae of anterior and posterior leaflets. Sedimentation rate was 124 and antistreptolysin-O titer was >2300 IU/ml. Respiratory cultures, respiratory viral panel, and mycoplasma, legionella, and HIV tests were negative. Antinuclear antibodies, antineutrophil cytoplasmic antibodies, and antiglomerular basement membrane antibodies were negative. She was diagnosed with DAH due to rheumatic fever with mitral valve involvement. Penicillin therapy was begun and she was taken for urgent mitral valve replacement. Pathologic evaluation demonstrated severe acute and chronic inflammation of the anterior and posterior leaflets consistent with rheumatic valve disease. DISCUSSION: DAH is a potentially life-threatening condition resulting from a wide array of pathology. It can be categorized into three distinct DAH syndromes: vasculitis/capillaritis, “bland” pulmonary hemorrhage, DAH due to another process or condition.1The pathogenesis of DAH from valvulopathy falls into the “bland” category, as alveolar bleeding occurs through increased mechanical pressure, rather than inflammation of the pulmonary vasculature. Right upper lobe predominant bleeding is characteristic in mitral regurgitation because the regurgitant jet is toward the right pulmonary venous system. Transesophageal studies show that the flow velocity and pressure gradient are highest at the orifice of the right pulmonary venous system.2 CONCLUSIONS: While not as prevalent in more developed countries, acute rheumatic fever and rheumatic heart disease are still prominent global health concerns with potentially life-threatening complications. This case serves to remind clinicians to consider rheumatic valvulopathy as a possible etiology when alveolar hemorrhage is diagnosed, and to consider DAH as a cause of respiratory failure in patients with rheumatic and non-rheumatic mitral regurgitation. Reference #1: Ioachimescu OC, Stoller JK. Diffuse alveolar hemorrhage: diagnosing it and finding the cause. Cleve Clin J Med. 2008;75:258, 260, 264-5. Reference #2: Roach JM, Stajduhar KC, Torrington KG. Right upper lobe pulmonary edema caused by acute mitral regurgitation: diagnosis by transesophageal echocardiography. Chest. 1993;103:1286-1288. DISCLOSURE: The following authors have nothing to disclose: Steven Register, Stephanie Taylor, Brice Taylor No Product/Research Disclosure Information

Cavitation and Confusion: A Puzzling Presentation of Granulomatous Disease

SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: A 32-year-old HIV-negative African American man presented with a 7-month history of altered mental status (AMS) and functional decline for which he underwent multiple rounds of hospitalizations and diagnostic testing before the correct diagnosis was made. CASE PRESENTATION: He was first seen at an outlying hospital for AMS, which was attributed to drug use. Chest x-ray (CXR) revealed bilateral cavitary lung lesions, and he was treated for pneumonia and discharged. He returned one month later with persistent AMS. Brain MRI showed diffuse leptomeningeal enhancement. Lumbar puncture (LP) revealed lymphocytic pleocytosis, low glucose, and elevated protein. All infectious testing was negative. Transbronchial lung biopsy showed inflammation, but no organisms. He was discharged on itraconazole for presumed disseminated fungal infection. He returned with progressive mental status changes four months later. MRI and LP were unchanged, but CSF angiotensin-converting enzyme (ACE) was four times normal. Chest imaging again showed cavitary lesions, and CT guided lung biopsy revealed necrotizing granulomatous inflammation. Microbiological tests were again negative except one serum complement fixation (CF), which was positive for histoplasmosis. The patient was treated with amphotericin and discharged on itraconazole until he presented to us two months later. By this time, the patient had developed gait abnormalities and urinary incontinence. Imaging demonstrated progression of prior lesions. Both transbronchial lung and brain biopsies revealed necrotizing and non-necrotizing granulomas. All fungal tests including histoplasmosis fungal CF were negative. Serum ACE level was elevated and, considering prior elevated CSF ACE and granulomas in the lung and brain, sarcoidosis was diagnosed. The patient was treated with high dose steroids and his clinical status, along with lung and brain lesions, improved. DISCUSSION: The elusiveness of this diagnosis in large part stemmed from the common diagnostic pitfalls of anchoring bias and reliance on general disease paradigm rather than specific clinical context. Despite the severity and progression of the patient’s symptoms on treatment, an incorrect diagnosis persisted on the basis of a single positive complement fixation test without microbiological confirmation. Additionally, the elevated CSF ACE was ignored in this unusual presentation of sarcoidosis. Although sarcoid is classically thought of as non-necrotizing, recent literature suggests sarcoidosis can cause a spectrum of granulomatous inflammatory tissue responses. CONCLUSIONS: This case illustrates the importance of ongoing diagnostic re-evaluation with consideration of atypical presentations of rare diseases when faced with discordant laboratory data or unexpected clinical progression. Reference #1: Rosen Y. Four decades of necrotizing sarcoid granulomatosis: what do we know now? Arch Pathol Lab Med. 2015;139(2):252-62. DISCLOSURE: The following authors have nothing to disclose: Meredith Greer, Udayan Shah No Product/Research Disclosure Information

Acral manifestations of systemic diseases: Drug-induced and infectious diseases

Drug reactions and systemic diseases often involve the skin. In particular, most drug-induced reactions and many infectious diseases present with dermatologic manifestations localized acrally, that is on distal portions of limbs (hand, foot) and head (ears, nose). A detailed review of all acral dermatologic signs of drug reactions and systemic diseases is beyond the scope of this paper, although some of these disorders will be discussed specifically here.

Molecular modeling of Gly80 and Ser80 variants of human group IID phospholipase A2 and their receptor complexes: potential basis for weight loss in chronic obstructive pulmonary disease.

Weight loss is a well known systemic manifestation of chronic obstructive pulmonary disease (COPD). A Gly80Ser mutation on human group IID secretory phospholipase A2 (sPLA2) enhances expression of the cytokines that are responsible for weight loss. In this study, we seek to establish a structural correlation of wild type sPLA2 and the Gly80Ser mutation with function. sPLA2 with glycine and serine at the 80th positions and the M-type receptor were modelled. The enzymes were docked to the receptor and molecular dynamics was carried out to 70ns. Structural analysis revealed the enzymes to comprise three helices (H1-H3), two short helices (SH1 and SH2), and five loops including a calcium binding loop (L1-L5), and to be stabilized by seven disulfide bonds. The overall backbone folds of the two models are very similar, with main chain RMSD of less than 1Å. The active site within the substrate binding channel shows a catalytic triad of water-His67-Asp112, showing a hydrogen bonded network. Major structural differences between wild type and mutant enzymes were observed locally at the site of the mutation and in their global conformations. These differences include: (1) loop-L3 between H2 and H3, which bears residue Gly80 in the wild type, is in a closed conformation with respect to the channel opening, while in the mutant enzyme it adopts a relatively open conformation; (2) the mutant enzyme is less compact and has higher solvent accessible surface area; and (3) interfacial binding contact surface area is greater, and the quality of interactions with the receptor is better in the mutant enzyme as compared to the wild type. Therefore, the structural differences delineated in this study are potential biophysical factors that could determine the increased potency of the mutant enzyme with macrophage receptor for cytokine secreting function, resulting in exacerbation of cachexia in COPD.

Common Oral Manifestations of Select Systemic Diseases: Anemia, Diabetes Mellitus and HIV

In this article, we discuss several common abnormal signs and symptoms that may be present in the oral cavity initially with or without any systemic symptoms at a typical dental practice. The purpose of this discussion is to increase clinical awareness for establishing differential diagnoses so that patients may obtain appropriate referrals for appropriate medical treatment.

Radiology in the Study of Bone Physiology

In this article, we review the core principles of bone physiology alongside imaging examples that demonstrate such principles. The core principles of bone physiology are reviewed and further solidified with a corresponding abnormal pathophysiologic example. The key principles of bone physiology to be reviewed include the following: (1) formation and growth, (2) maintenance and repair, (3) metabolism and regulation, and (4) neoplastic disease. Lastly, a collection of secondary bone diseases is presented to demonstrate the skeletal manifestations of numerous systemic diseases. With this integrative method, we hope to emphasize the value of using radiology to teach physiology within a clinical context. This is especially relevant now, as many US medical schools undergo curricular reform with more emphasis on integrative interdisciplinary learning. Ultimately, we intend to provide a paradigm for incorporating radiology into the pre-clinical medical curriculum through a review of basic science physiology that underlies key radiographic findings of the skeletal system. Radiology is known for its role in helping make diagnoses and clinical decisions. However, radiology is also well suited to enhance medical education by offering the ability to visualize physiology in action. This is especially true in skeletal radiology, where radiographic osseous changes represent a wide range of physiological processes. Therefore, skeletal radiology can be a useful tool for illustrating concepts of physiology that underlie the normal and abnormal radiologic appearances of bone. Radiology is an important but underutilized tool for demonstrating concepts in bone physiology.

Management of erectile dysfunction in patients with preexisting cardiovascular disease

Erectile dysfunction (ED) is common among patients with cardiovascular diseases (CVD). Sexual problems usually precede the onset of CVD and should, therefore, be considered as a risk factor for cardiac events. Similarly, patients with preexisting CVD are at increased risk of experiencing ED. Therefore, ED and CVD might be considered as two different clinical manifestations of the same systemic disease. Moreover, these conditions share many common pathophysiological mechanisms. Patients with preexisting CVD and ED should be stratified according to their cardiovascular risk. Sexual activity is safe in patients at low risk of CVD and these individuals might be treated with pro-erectile medications. On the other hand, men at high risk of CVD should be reassessed and the cardiac condition should be stabilized before they might have sexual attempts. Recent evidence suggests that lifestyle changes, administration of phosphodiesterase type-5 inhibitors, and testosterone supplementation might improve sexual function and reduce the risk of experiencing CVD during follow-up.

Systemic disease manifestations associated with epilepsy in tuberous sclerosis complex

Epilepsy is one of the most disabling symptoms of tuberous sclerosis complex (TSC) and is a leading cause of morbidity and mortality in affected individuals. The relationship between systemic disease manifestations and the presence of epilepsy has not been thoroughly investigated. This study utilizes a multicenter TSC Natural History Database including 1,816 individuals to test the hypothesis that systemic disease manifestations of TSC are associated with epilepsy. Univariate analysis was used to identify patient characteristics (e.g., age, gender, race, and TSC mutation status) associated with the presence of epilepsy. Individual logistic regression models were built to examine the association between epilepsy and each candidate systemic or neurologic disease variable, controlling for the patient characteristics found to be significant on univariate analysis. Finally, a multivariable logistic regression model was constructed, using the variables found to be significant on the individual analyses as well as the patient characteristics that were significant on univariate analysis. Nearly 88% of our cohort had a history of epilepsy. After adjusting for age, gender, and TSC mutation status, multiple systemic disease manifestations including cardiac rhabdomyomas (odds ratio [OR] 2.3, 95% confidence interval [CI] 1.3-3.9, p = 0.002), retinal hamartomas (OR 2.1, CI 1.0-4.3, p = 0.04), renal cysts (OR 2.1, CI 1.3-3.4, p = 0.002), renal angiomyolipomas (OR 3.0, CI 1.8-5.1, p < 0.001), shagreen patches (OR 1.7, CI 1.0-2.7, p = 0.04), and facial angiofibromas (OR 1.7, CI 1.1-2.9, p = 0.03) were associated with a higher likelihood of epilepsy. In the multivariable logistic regression model, cardiac rhabdomyomas (OR 1.9, CI 1.0-3.5, p = 0.04) remained significantly associated with the presence of epilepsy. The identification of systemic disease manifestations such as cardiac rhabdomyomas that confer a higher risk of epilepsy development in TSC could contribute to disease prognostication and assist in the identification of individuals who may receive maximal benefit from potentially novel, targeted, preventative therapies.

Association between pruritus and systemic diseases

Pruritus is a common subjective skin symptom. Ten to fifty percent of adults present with different degrees of refractory pruritus, which greatly affects the physical and mental health of patients. Pruritus can be divided into two types, primary pruritus and pruritus secondary to various systemic diseases, such as renal insufficiency, cholestasis, hematologic disorders, malignancies, some infections, etc. Some scholars consider that pruritus is an early clinical manifestation of some systemic diseases, and may serve as an early clue to diagnosis of systemic diseases. Key words: Pruritus; Uremia; Liver & gallbladder diseases; Hematologic diseases; Endocrine system diseases; Infection

Diagnosis dan Penatalaksanaan Uveitis dalam Upaya Mencegah Kebutaan

Uveitis is an inflammation of the uvea which may result in blindness. Uveitis may be caused by limited inflamation of the uveal tract, manifestation of systemic diseases (autoimmune, infection, cancer), expansion of inflammation in the cornea and sclera, trauma or idiopathic. Anterior uveitis is an inflammation of the iris and cilliary body with symptoms of pain, red eye, photophobia, and decrease in visual acuity. Intermediate uveitis is the inflammation of the pars plana and frequently involves anterior vitreous and posterior uveitis. Clinical manifestation of intermediate uveitis is usually mild without red eye and pain, however vision may decrease due to macular edema and cell aggregation in vitreous. Posterior uveitis is an inflammation involving choroid layer, which is common in developing countries due to high prevalence of infectious diseases (toxoplasmosis, tuberculosis, HIV, syphilis). Patient may complain of blurry vision but not accompanied by pain, red eye, and photophobia. Complications of posterior uveitis are cataract, glaucoma, macular edema, keratopathy, turbidity of vitreous, retinal detachment, and optic nerve atrophy. The prognosis of posterior uveitis is worse than anterior uveitis. Panuveitis is an inflammation of the uvea and surrounding structures (retina, vitreous). Diagnosis is made based on anamnesis, ophthalmic examination, laboratory examination, and imaging. Treatment of uveitis is intended to reduce inflammation, minimize structural destruction, prevent blindness, reduce pain and photophobia. Corticosteroid and immunosuppresant are the drugs of choice to manage the inflammation, where NSAID is used to reduce pain and cyclopegic administration to prevent posterior synechiae. Antimicrobial is given if uveitis is caused by infection. Underlying diseases of uveitis must be treated comprehensively to prevent further progression, complications and blindness. Normal 0 false false false IN X-NONE X-NONE

Skin and Soft Tissue Infections.

The skin is colonized by a diverse collection of microorganisms which, for the most part, peacefully coexist with their hosts. Skin and soft tissue infections (SSTIs) encompass a variety of conditions; in immunocompromised hosts, SSTIs can be caused by diverse microorganisms-most commonly bacteria, but also fungi, viruses, mycobacteria, and protozoa. The diagnosis of SSTIs is difficult because they may commonly masquerade as other clinical syndromes or can be a manifestation of systemic disease. In immunocompromised hosts, SSTI poses a major diagnostic challenge, and clinical dermatological assessment should be initially performed; to better identify the pathogen and to lead to appropriate treatment, etiology should include cultures of lesions and blood, biopsy with histology, specific microbiological analysis with special stains, molecular techniques, and antigen-detection methodologies. Here, we reviewed the epidemiology, pathophysiology, clinical presentation, and diagnostic techniques, including molecular biological techniques, used for SSTIs, with a focus on the immunocompromised host, such as patients with cellular immunodeficiency, HIV, and diabetic foot infection.

Genetically defined autoinflammatory diseases

Autoinflammatory diseases are hyperinflammatory, immune dysregulatory conditions that typically present in early childhood with fever and rashes and disease-specific patterns of organ inflammation. This review provides a historic background of autoinflammatory disease research, an overview of the currently genetically defined autoinflammatory diseases, and insights into treatment strategies derived from understanding of the disease pathogenesis. The integrative assessment of autoinflammatory conditions led to the identification of innate pro-inflammatory cytokine 'amplification loops' as the cause of the systemic and organ-specific disease manifestations, which initially centered around increased IL-1 production and signaling. More recently, additional innate pro-inflammatory cytokine amplification loops resulting in increased Type I IFN, IL-17, IL-18, or IL-36 signaling or production have led to the successful use of targeted therapies in some of these conditions. Clinical findings such as fever patterns, type of skin lesions, genetic mutation testing, and the prevalent cytokine abnormalities can be used to group autoinflammatory diseases.

Advancements in the management of uveitis.

Uveitis may exist as a clinical manifestation of an underlying systemic disease or may represent an idiopathic entity, sometimes with a very characteristic pattern. Different forms of uveitis have been defined on the basis of three important variables: chronicity, anatomic location, and underlying etiology. The evolving understanding of the immune system has resulted in a more targeted approach to manage patients with different forms of uveitis, although clearly this approach is at a very early stage. Altered patterns of cellular processing and different cytokine expression, including TNF, IL-1, IL-2, IL-6, and IL17, have been defined in uveitis, and this has laid the pathway for targeted therapy. Furthermore, approved biologic therapies for some of the more common autoimmune illnesses have now been tested in uveitis. Adalimumab and infliximab have been the best studied anti-TNF agents and indeed have now been recommended by an expert panel as the first line of treatment for ocular manifestations of Behçet's disease and the second line of treatment for other forms of uveitis. Adalimumab has been recently approved for intermediate uveitis, posterior uveitis, and panuveitis. Other biologic agents have been tested, including daclizumab, a monoclonal antibody directed against IL-2, anti-IL1, and anti-IL-6 receptor agents and therapies that block antigen-presenting cell and T-cell interaction, such as abatacept. In small case series, other biologics such as interferon and rituximab have also been evaluated. Although these biologic therapies have provided a larger armamentarium to treat uveitis, challenges remain. Uveitis is not a disease, but a manifestation of many potential systemic diseases that may have specific individual therapeutic targets. Identification and characterization of these underlying diseases are not always possible and, more importantly, the most effective therapies for each entity have not been defined. In this study, an approach to manage patients with uveitis is presented and current therapy is reviewed.

Mo1813 Epidemiology, Risk Factors and Systemic Manifestations of Crohn's Disease in the United States: A Population Based Study

Mo1813 Epidemiology, Risk Factors and Systemic Manifestations of Crohn's Disease in the United States: A Population Based Study

Knowledge, Attitude and Practice on Dental Caries and Oral Hygiene among Medical Students at Janaki Medical College Teaching Hospital

Background: Oral health habits are measures people learn and practice regularly in order to maintain good oral health or prevent oral diseases. The mouth is an integral part of the body, and there are oral manifestations of many systemic diseases that must be managed in both healthy and medically compromised people. Oral health knowledge is considered to be an essential requirement for health-related practices. A good quality of life is possible if students maintain their oral health and become free of oral disease. Dental caries are increasing in developing countries day by day where preventive programs have not been implemented properly. The level of information on oral health knowledge and practices among medical students is unidentified and worthy of investigation in Nepal, and this study aimed at assessing the level of knowledge, attitude and practices on dental caries and oral hygiene among medical students studying at Janaki Medical College Teaching Hospital, Janakpurdham. Methods:A standard structured closed-questionnaire was designed and distributed to medical students studying in MBBS programme to assess the knowledge, attitude and practice about dental caries and oral hygiene. Results: Out of 330 medical students, 145 were male and 185 were female, of which male had the highest knowledge regarding dental caries which was found to be statistically significant. Most of the students had attitude of visiting once in year for dental check up. The highest number of students had once per day brushing habit in early morning with fluoride containing tooth paste. Conclusions: The present study concluded that the knowledge and practice status of oral hygiene and dental caries among medical students was found to be satisfactory. More oral health education programs must be conducted for the control of oral diseases.

The association between systemic sclerosis disease manifestations and esophageal high-resolution manometry parameters.

We aimed to evaluate the associations between systemic sclerosis (SSc)-related systemic manifestations and esophageal function using high-resolution manometry (HRM). Patients with SSc that had undergone HRM between 1/2004 and 9/2014 were identified and HRMs were analyzed according to the Chicago Classification. Clinical characteristics were identified via retrospective chart review and compared among motility diagnoses while adjusting for age, gender, race, and SSc-disease duration. Seventy-nine patients (85% female, ages 25-77) were included. Clinical characteristics were compared between patients with absent contractility (AC, n = 40), ineffective esophageal motility (IEM; n = 15), and normal motility (n = 19); the five remaining patients met criteria for other motility diagnoses. Groups differed in severity of skin involvement measured by the modified Rodnan skin score (0-51): AC (adjusted mean 12.6), IEM (4.4), normal (4.3), p = 0.043. Pulmonary function tests [percent predicted FVC and DLCO) were lower in AC (adjusted mean, FVC: 70.3, DLCO 51.1), than IEM (FVC: 92.0; DLCO: 76.9) and normal motility (FVC: 80.0; DLCO: 67.2), p values 0.057 (FVC) and 0.007 (DLCO). Groups did not differ by SSc-disease duration, autoantibodies, or reported symptoms of dysphagia or reflux. In patients with SSc, absent esophageal contractility on HRM was associated with increased skin disease severity and worse lung function. Obtaining HRM to identify SSc patients with more severe esophageal dysfunction could be considered to enable implementation of management strategies in patients potentially at risk for increased morbidity and mortality.

Secondary myopathy due to systemic diseases.

BackgroundSome systemic diseases also affect the skeletal muscle to various degrees and with different manifestations. This review aimed at summarizing and discussing recent advances concerning the management of muscle disease in systemic diseases.MethodLiterature review by search of MEDLINE, and Current Contents with appropriate search terms.ResultsSecondary muscle disease occurs in infectious disease, endocrine disorders, metabolic disorders, immunological disease, vascular diseases, hematological disorders, and malignancies. Muscle manifestations in these categories include pathogen‐caused myositis, muscle infarction, rhabdomyolysis, myasthenia, immune‐mediated myositis, necrotising myopathy, or vasculitis‐associated myopathy. Muscle affection may concern only a single muscle, a group of muscles, or the entire musculature. Severity of muscle affection may be transient or permanent, may be a minor part of or may dominate the clinical picture, or may be mild or severe, requiring invasive measures including artificial ventilation if the respiratory muscles are additionally involved. Diagnostic work‐up is similar to that of primary myopathies by application of non‐invasive and invasive techniques. Treatment of muscle involvement in systemic diseases is based on elimination of the underlying cause and supportive measures. The prognosis is usually fair if the causative disorder is effectively treatable but can be fatal in single cases if the entire musculature including the respiratory muscles is involved, in case of infection, or in case of severe rhabdomyolysis.ConclusionSecondary muscle manifestations of systemic diseases must be addressed and appropriately managed. Prognosis of secondary muscle disease in systemic diseases is usually fair if the underlying condition is accessible to treatment.

Diagnostic and Therapeutic Implications of Erectile Dysfunction in Patients with Cardiovascular Disease

Erectile dysfunction (ED) and cardiovascular disease (CVD) share many common pathophysiologic pathways and might be regarded as two different clinical manifestations of the same systemic disease. Consequently, ED and CVD are pathologic conditions that often coexist in the same patient. The urologist plays an important role in the management of ED in patients with a history of cardiovascular events. Therapeutic measures aimed at improving sexual function in CVD patients should be considered only after careful evaluation of the underlying cardiologic condition and assessment of ability to exercise. Sexual activity and treatment of ED might trigger cardiac events in selected patients with preexisting CVD; therefore, proerectile therapies should be administered only to low-risk patients for whom subsequent risk of cardiac events would not be increased. Conversely, men at high risk of CVD should receive cardiologic reassessment and stabilization before attempting sexual activity and receiving ED treatment. Risk reduction and lifestyle changes, administration of phosphodiesterase type 5 inhibitors, and testosterone replacement therapy, as indicated, might provide benefits not only in terms of improving sexual function but also for reducing the risk of future cardiac events. Patient summaryErectile dysfunction (ED) and cardiovascular disease (CVD) share many pathophysiologic mechanisms and often coexist in the same patient. We evaluated the role of the urologist in the management of ED in patients with preexisting CVD and the impact of measures aimed at improving sexual function on the subsequent risk of cardiac events.