Rheumatologic Tests Research Articles

Ischemic Retinal Vasculitis after Previous Toxoplasma Chorioretinitis

Introduction: Ischemic retinal vasculitis is an inflammatory disease affecting the retinal vessels. Visual acuity decreases due to macular ischemia, macular edema, neovascularization leading to vitreous hemorrhage, fibrovascular proliferation or tractional retinal detachment. Purpose: To present the case of a patient with ischemic retinal vasculitis. Material and Methods: The case involves a 34-year-old woman. The following tests were performed: complete blood count, biochemistry, coagulogram, rheumatological tests, serum calcium level, angiotensin-converting enzyme, CT of the lungs, MRI of the brain and spine, serological tests for: cytomegalovirus, herpes simplex virus (type 1 and 2), varicella-zoster virus, toxoplasmosis, tuberculosis, syphilis, as well as fluorescein angiography and optical coherence tomography. Results and Discussion: The patient was admitted to the hospital with visual acuity of the right eye, 0.8/0.9, and of the left eye, 1.0. The intraocular pressure in both eyes was normal: 14 mmHg in the right eye and 15 mmHg in the left eye. The following pathological finding was detected in the right eye fundus: an old chorioretinal cicatrix under the inferior temporal vascular arch with an ischemic exudate next to it, occlusion of the inferior temporal retinal arterial and venous branches, neovascularization and a preretinal hemorrhage. Fluorescein angiography showed delayed filling of the affected vessels, neovascularization, and major ischemia in the lower temporal part of the retina. The results of the serological tests for varicella zoster virus, herpes simplex virus type 1 and toxoplasmosis were outside the reference ranges. The patient was treated with Clindamycin and Acyclovir. Emergency laser therapy was performed in the ischemic retina area. Previous toxoplasma chorioretinitis was diagnosed, which had led to ischemic retinal vasculitis with sectoral involvement of the retinal vessels. The patient has been monitored over 3 - 4 month periods, showing high visual acuity and stable ocular condition. Conclusion: Patients with ischemic retinal vasculitis are a challenge when it comes to clarifying the etiological diagnosis. Treatment on time prevents severe and irreversible loss of vision. Occlusive vasculitis is a rare complication of ocular toxoplasmosis, but must be taken into consideration when young patients are involved.

Salivary metabolomic profile in adolescents with juvenile systemic lupus erythematosus.

The aim of this study was to characterize the salivary metabolomic profile in adolescents with juvenile systemic lupus erythematosus (jSLE). A total of 24 adolescents with jSLE (15.92 ± 2.06 years) and 12 systemically healthy controls (15.25 ± 2.7 years) were included in the study. Participants underwent rheumatologic testing and periodontal examination, with the recording of plaque index (PI), probing depth (PD), clinical attachment level (CAL), and bleeding on probing index (BPI). Unstimulated whole saliva was collected from both groups and stored at -80 ºC. The salivary proton nuclear magnetic resonance (1H-NMR) spectra were acquired in a spectrometer operating at 500 MHz. Partial least squared discriminant analysis (PLS-DA) and orthogonal PLS-DA (O-PLS-DA) were used for statistical analysis. Mean CAL and PI were significantly increased in the group with jSLE (p < 0.01). Patients with jSLE presented a significantly different salivary metabolic profile (accuracy = 0.54; R2 = 0.86; Q2 = -0.293), significantly higher salivary levels of N-acetyl sugars, and significantly reduced levels of phenylalanine, glycine, taurine, hydroxybutyrate, and valerate compared with healthy controls (p < 0.05). It is suggested that the salivary metabolomic profile analyzed by 1H NMR in patients with jSLE presents a different fingerprint that the systemically healthy subjects. Integrating the variation of metabolites with the identification of the metabolic pathways involved seems to provide a better understanding of the influence of systemic disease on salivary metabolites.

432 ACS-STEMI in a young man with triple positive antiphospholipid syndrome: clinical implications about antithrombotic therapy management

Abstract Antiphospholipid antibody syndrome (APS) is a systemic autoimmune disorder characterized by venous and arterial thromboembolic (TE) disease, and/or pregnancy morbidity, associated with persistent elevated antiphospholipid antibodies (lupus anticoagulant, LA, anticardiolipin, aCL, and anti-beta2-glycoprotein I, anti-β2GPI). A 51 year-old man, smoker, presented to our ER with chest pain. EKG showed Q waves and STE in the anterior leads, with increased troponin levels. A diagnosis of anterior STEMI was made and he was taken into our catheterization laboratory, where a high burden thrombotic plaque determining LM and ostial LAD subocclusion was treated with PPCI and a DES implantation; in addition, multiple failed PCI attempts were performed on a distal LAD occlusion, and an integrilin bolus was administered. The patient was admitted to our CICU and a DAPT with ASA and Ticagrelor was started. TTE showed LVEF 40%, apical akinesis, septal and anterior hypokinesis, and no significant valve disease. An aPTT abnormal value (100.5 s; reference range 25–38.5 s) was detected, not corrected by aPTT mixing study. Given suspected autoimmune prothrombotic state, normal Hb and PLT values and low bleeding risk, LMWH 100 IU/kg every 12 h was started, in addition to DAPT with switch from Ticagrelor to Clopidogrel. LA and aCL and anti-β2GPI IgG were positive, with negativity of others rheumatologic tests, confirming primary APS diagnosis. Repeat TTE showed EF 50%. The patient was discharged in ‘triple therapy’ (DAPT plus LMWH), and referred to the rheumatology clinic. LA, aCL, and anti-β2GPI tests 12 weeks later were positive, confirming a triple positive APS and satisfying revised Sapporo criteria. The rheumatologist switched therapy to Clopidogrel plus Warfarin. At 3 months follow-up, the patient, former smoker, was asymptomatic and TTE confirmed EF 50%, so he was recommended to continue with his medical therapy. APS is rarely associated with AMI (∼5.5%) and in only 2.8% cases AMI represents the onset of the disease. AMI pathogenesis in APS is considered to be acute thrombosis of coronary arteries, in contrast with atherosclerotic plaque rupture in typical AMI. Key discriminators to identify APS as potential underlying cause of AMI include young age, previous unprovoked thromboses, low platelets count (they are consumed in the thrombotic process), high aPTT value (LA may interfere with assembly of the prothrombinase complex on phospholipids), coronary artery thromboses in the setting of otherwise normal otherwise appearing coronary arteries. APS antibodies have also pro-inflammatory activity on vascular endothelial cells, leading to accelerated atherosclerosis. aGAPSS score (high risk ≥10) is useful for risk stratification of recurrent thrombosis and AMI in young patients with APS. The treatment of AMI in APS is therefore a clinical challenge. Strict management of additional CV risk factors is crucial. VKA (INR &amp;gt;3 with Warfarin, INR2–3 with Warfarin and ASA) should be provided for life, because of the very high risk of recurrent TE. DOACs are less effective and less safe than VKAs for TE prevention in APS. The role of coronary stents, considering higher rates of stent thrombosis after PCI in APS patients triple therapy concomitant risks, requires further studies. Due to lack of large, randomized, prospective studies, there is no clear experts consensus about optimal antithrombotic therapy in secondary prevention after arterial TE. APS patients with STEMI should undergo PCI, usually associated with thrombus aspiration, and in selected cases DES implantation in culprit lesion followed by triple antithrombotic therapy with short-term DAPT and long-term VKA.

1561. Recurrent Bilateral Culture Negative Abscesses Mimicking a Diabetic Foot Infection

BackgroundThe aim of this study is to present a novel case of recurrent bilateral culture negative pedal abscesses, mimicking a diabetic foot infection, as an extra-intestinal manifestation of previously undiagnosed ulcerative colitis. Culture negative abscesses may develop as a rare sequela of ulcerative colitis, with less than 40 cases documented within current literature. They often demonstrate a large neutrophilic predominance, negative cultures, and negative serology testing. The diagnosis was achieved utilizing a multi-specialty team approach with podiatry serving as the primary service coordinating the patient care.MethodsMultiple incision and drainage procedures were performed with copious purulence expressed from the plantar medial pedal compartment. The patient was initiated on broad spectrum antibiotics post-operatively without a clinical response. Advanced microbiological and rheumatologic testing was performed.ResultsSerial aerobic, anaerobic, acid fast, and fungal cultures as well as 16s rDNA testing remained negative for infection throughout the admission. Rheumatologic work-up consisted of broad autoimmune serologic testing (Table 1). Greatly elevated levels of calprotectin were identified, resulting in a colonoscopy for confirmation of the diagnosis of ulcerative colitis. The patient was then placed on infliximab and has remained free of recurrent abscesses for the 15 months of follow up.Table 1: Workup Results ConclusionThe differential diagnosis of a culture negative abscess is broad and a multi-specialty team approach must be considered. Criteria for the diagnosis has previously been proposed and includes: deep abscess with neutrophilic features, negative serologic testing, failure of broad-spectrum antibiotic therapy, and rapid improvement on steroids or other DMARDs with subsequent radiologic evidence of abscess resolution. To our knowledge no bilateral cases have been documented within the foot.Disclosures All Authors: No reported disclosures

A clinical approach to hypertrophic pachymeningitis.

Hypertrophic pachymeningitis (HP) is a non-usual manifestation of rheumatologic, infectious, and neoplastic diseases. Etiological diagnosis is a challenge, but when made promptly it creates a window of opportunity for treatment, with the possibility of a total reversal of symptoms. HP is an inflammatory process of the dura mater that can occur as a manifestation of sarcoidosis, granulomatosis with polyangiitis, and IgG4-related disease. The HP case evaluation is extensive and includes central nervous system imaging, cerebrospinal fluid analysis, serology, rheumatologic tests, and systemic survey for other manifestations sites. After systemic investigation, meningeal biopsy might be necessary. Etiology guides HP treatment, and autoimmune disorders are treated with corticosteroids alone or associated with an immunosuppressor. HP is a manifestation of several diseases, and a precise etiological diagnosis is crucial because of the difference among treatments. An extensive investigation of patients with HP helps early diagnosis and correct treatment.

DIFFUSE ALVEOLAR HEMORRHAGE AS A CONSEQUENCE OF MICROSCOPIC POLYANGIITIS DUE TO COVID-19

SESSION TITLE: Fellows' COVID-19 SESSION TYPE: Fellow Case Reports PRESENTED ON: October 18-21, 2020 INTRODUCTION: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a small vessel vasculitis of which microscopic polyangiitis (MPA) is a subset. They are known to cause life threatening renal failure and/or pulmonary hemorrhage. Viruses are a known trigger of vasculitis, including MPA. (1) We present a case of MPA caused by COVID-19 presenting as diffuse alveolar hemorrhage (DAH) CASE PRESENTATION: 55 year old female with a past medical history of hemorrhagic stroke, hypertension & asthma who presented with respiratory distress & severe hypoxemia. At the time of intubation, a moderate amount of blood was suctioned from her endotracheal tube. She was profoundly hypoxemic & required 100% FiO2 and PEEP 18 cmH2O. A SARS CoV2 PCR test was positive. Other admission laboratory values were significant for a creatinine of 4.9 mg/dL (baseline: 0.8mg/dL), hemoglobin of 8 g/dL (baseline: 11.8g/dL), ferritin 569 ng/mL, D-dimer 3.18 ug/mL, CRP 62.8 mg/L, ESR 118 mm/hr. Chest X-ray showed dense bilateral airspace opacities. A CT scan showed extensive bilateral consolidations with air bronchograms that were not typical for a COVID-19 pneumonia (Image 1) except in the apices where these dense consolidations had a peripheral pattern. She was admitted to the medical ICU where she continued to have bleeding into her endotracheal tubing. Bronchoscopy showed blood in the airways diffusely with no focality to the bleeding. A bronchoalveolar lavage was performed of two separate lobes, and was notable for progressively bloody aliquots consistent with DAH. Because of concern for a pulmonary-renal syndrome, rheumatologic testing was performed. She was empirically treated with high dose intravenous steroids and plasmapheresis. Unfortunately, profound hypoxia persisted and family elected to withdraw life-sustaining therapies. Two days after her death, serologies revealed a mildly positive ANA and a strongly positive anti-MPO ANCA, suggesting the diagnosis of MPA. DISCUSSION: While the primary signs and symptoms of COVID-19 are fever, cough, and dyspnea, other reported manifesations include kawasaki-like disease in children (2) and cutaneous manifestations such as livedo reticularis (3). We believe that in our case the patient’s infection with COVID-19 & its subsequent increased inflammatory state led to her developing MPA vasculitis with subsequent pulmonary-renal syndrome complicated by diffuse alveolar hemorrhage, rapidly progressive renal failure and ultimately death. Her clinical and serologic picture were consistent with a diagnosis of microscopic polyangiitis. It is unclear whether our patient had a smoldering vasculitis that was triggered by the hyper-inflammatory nature of COVID-19 or whether this virus caused a vasculitis de novo. CONCLUSIONS: With vasculitis potentially being one of COVID-19's presenting symptoms, prompt diagnosis and intervention is essential in improving outcomes. Reference #1: Yates M, Watts R. ANCA-associated vasculitis. Clin Med (Lond). 2017;17(1):60-64. doi:10.7861/clinmedicine.17-1-60 Reference #2: Viner RM, Whittaker E. Kawasaki-like disease: emerging complication during the COVID-19 pandemic [published online ahead of print, 2020 May 13]. Lancet. 2020;10.1016/S0140-6736(20)31129-6. doi:10.1016/S0140-6736(20)31129-6 Reference #3: Sachdeva M, Gianotti R, Shah M, et al. Cutaneous manifestations of COVID-19: Report of three cases and a review of literature [published online ahead of print, 2020 Apr 29]. J Dermatol Sci. 2020;S0923-1811(20)30149-3. doi:10.1016/j.jdermsci.2020.04.011 DISCLOSURES: no disclosure on file for Christopher Damiano; No relevant relationships by Elie Fares, source=Web Response no disclosure on file for Catherine Kuntz; No relevant relationships by Kriti Pathak, source=Web Response

Analysis of musculoskeletal side effects of oral Isotretinoin treatment: a cross-sectional study

Background/ objectivesAcne vulgaris is a chronic inflammatory disease affecting the pilosebaceous unit. Isotretinoin is an effective treatment option for severe acne. The aim of this study was to evaluate musculoskeletal side effects of systemic isotretinoin treatment.MethodsNinety-four patients with acne vulgaris and 100 sex- and age-matched controls were enrolled in this study. Only the patients who had musculoskeletal symptoms were evaluated in this study. All participants were firstly assessed by a dermatologist. The patients were asked whether they had any musculoskeletal symptoms after isotretinoin treatment, if so, the feature and duration of the symptoms were recorded. The dosage of the drug, treatment duration, incidence of arthralgia, myalgia, low back pain, sacroiliitis and tendinopathy and laboratory test results were noted. The severity of pain was assessed by visual analog scale (VAS). The severity of acne vulgaris was evaluated by Global Acne Grading Scale (GAGS). Sacroiliac radiography, magnetic resonance imaging (MRI) and rheumatologic blood tests were requested from the patients meeting Assessment of Spondyloarthritis International Society (ASAS) criteria.ResultsOf the 94 patients, 71 were female and 23 were male. 47.9% of the patients had arthralgia, 53.2% had myalgia, 70.2% (66) had low back pain, 11.7% had sacroiliitis and 4.3% had tendinopathy. 37.8% of 66 patients with low back pain had inflammatory pain and 62.2% had mechanical pain. Bone marrow edema consistent with sacroiliitis was detected by sacroiliac MRI in 11 patients with inflammatory back pain. The median total cumulative dose of isotretinoin was significantly higher in patients with low back pain than in patients without low back pain (p = 0.014). There was no significant correlation between cumulative dose of drug, treatment duration and VAS with ESR and CRP (p > 0.05). Also no correlation was found between GAGS scores and musculoskeletal symptoms (p > 0.05).ConclusionLow back pain is one of the very common complications of isotretinoin. It can be mostly mechanical or inflammatory. Isotretinoin-induced low back pain is dose-related, and inflammatory back pain without sacroiliitis is also frequent. The clinicians should be aware of the back pain may be a reflective of sacroiliitis during isotretinoin usage.

Commentary on "Use of Rheumatologic Testing in Patients Who Eventually Receive a Diagnosis of Rheumatoid Arthritis".

Commentary on "Use of Rheumatologic Testing in Patients Who Eventually Receive a Diagnosis of Rheumatoid Arthritis".

Use of Rheumatologic Testing in Patients Who Eventually Receive a Diagnosis of Rheumatoid Arthritis.

Anti-cyclic citrullinated peptide antibody (ACPA) has excellent specificity and prognostic value in patients with early rheumatoid arthritis (RA). The American College of Rheumatology included ACPA in their 2010 classification criteria for RA, but we hypothesize that primary care physicians (PCPs) underuse ACPA, even when clinical suspicion for RA is high. We aimed to describe their use of diagnostic testing in patients who were referred to a rheumatologist and eventually diagnosed as having RA. In this retrospective cohort study, a systematic abstraction tool was used to review the medical records of patients seen between January 1, 2010 and June 15, 2014 in two rheumatology clinics: one private practice and one community health center associated with an academic medical center. For purposes of hypothesis generation, we compared the characteristics of patients with and without testing using unpaired t tests or Fisher exact tests. We identified 173 patients with RA referred from 141 different PCPs: 82.7% were women with a mean ± standard deviation age of 55.5 ± 18.6 years. ACPA and rheumatoid factor were ordered in 28.9% (95% confidence interval 22.6-36.2) and 41.0% (95% confidence interval 33.9-48.6) of patients, respectively. Imaging was underused. Almost half (45.7%, or 37/81) of the patients with documented symptom duration had a delay of at least 1 year before referral; however, ACPA utilization was not associated with the delay to treatment initiation. Most PCPs failed to order diagnostic tests for RA before referring a patient with polyarthritis who eventually received a diagnosis of RA. We also observed delays in diagnosis, with half of the patients waiting >1 year from symptom onset to diagnosis. These findings suggest educational efforts for PCPs should focus on emphasizing earlier diagnostic workups, especially ACPA, in patients suspected to have RA.

Optimization of the Order Menu in the Electronic Health Record Facilitates Test Patterns Consistent With Recommendations in the Choosing Wisely Initiative.

Thyroid and rheumatologic autoimmune testing are areas where evidence-based guidance from specialty organizations and Choosing Wisely support utilizing screening tests for autoimmune and thyroid disorders prior to more specialized testing. Adjustment of the orderable options in the electronic health record (EHR) can influence ordering patterns without requiring manual review or additional effort by the clinician. The menu was adjusted to reflect recommendations from Choosing Wisely to favor screening tests that automatically reflex to specialized testing on primary care providers' preference lists. Effectiveness was evaluated by reviewing total orders for individual tests. Shifts in ordering from individual screening tests (antinuclear antibody and thyrotropin) to ones that reflexed to specialized testing were observed in parallel with significant reductions in the corresponding specialized testing. Optimization of the EHR laboratory ordering menu can be used to shift ordering patterns toward Choosing Wisely recommendations.

Polyarteritis nodosa in a patient on hemodialysis; a case report

We represent a 40-year-old woman with end-stage renal disease (ESRD) of unknown etiology referred to the emergency department with episodes of chills and fever during hemodialysis. Further assessments revealed vasculitic skin rashes, as well as abdominal tenderness and later on, bloody diarrhea. Abdominal pain worsened during the course of admission, and as a result, the patient underwent cholecystectomy and appendectomy. Necrotizing vasculitis was diagnosed based on biopsy specimens taken from gall bladder and skin rashes. The diagnosis of poly arteritis nodosa (PAN) was established based on pathologic report and rheumatologic tests. Methylprednisolone pulses were administrated for 3 days followed by oral prednisolone and cyclophosphamide. After treatment, the signs and symptoms subsided. The present case report highlights the importance of timely diagnosis of PAN to prevent potentially irreversible consequences.

Neurological evaluation in patients infected with Chikungunya virus: analysis of a cohort of Rio de Janeiro/Brazil

Background: In addition to fever and typical joint pain caused by Chikungunya virus (CHIKV), demonstrations as meningoencephalitis, mielorradiculopathy, mieloneuropathy and myelitis, Guillain-Barre syndrome have been described. However, most of the neurological changes have been reported in the acute phase, there are few records in the chronic phase of the disease. Given this, the objectives is to describe in this study the main changes to the neurological examination in patients infected by CHIKV in the chronic phase of the disease. Methods & Materials: Neurological assessment of patients with diagnosis of CHIKV served in a reference center in Rio de Janeiro/Brazil between February and July 2017. Results: Twelve patients were evaluated, 10 women, 2 men. All patients evaluated were chronic phase of the disease, with no signs of acute infection. Changes found in the clinical examination: hipopalestesia by at least two members in 5 (41.6%) patients, and hypoesthesia (tactile, thermal and painful) in 5 (41.6%). 3 patients (25%) presented concomitance of the two amendments. 2 (16.6%) did not show any changes to the neurological examination. Only 1 patient developed neurological symptoms during the acute phase of the disease (paresthesia in the lower limbs). No patient presented any neurological symptoms during the evaluation. 3 (25%) were in use of metrotexate (10 mg/day) due to the severity of the joint pain, but did not provide different neurological changes. Rheumatologic tests (FAN, rheumatoid factor) were negative in all. One patient has diabetes and other Sjogren's syndrome-neurological deficits remained with the same sensitivity pattern in these two cases. All patients had diagnostic confirmation for CHIKV (serological evaluation); no other infectious disease was identified. The pain described by all patients was of origin nociceptive, not neuropathic (according to the application questionnaire for neuropathic pain DN4). Conclusion: Patients in acute and chronic phase of infection by CHIKV may have neurological changes, even without a specific manifestation. The commitment of superficial sensitivity was the dominant one in this sample evaluation; no central symptom was observed. The severity of the joint pain, the most important thing in these cases, has not been directly linked to neurological changes.

Case Report: Polyarteritis nodosa or complicated Henoch-Schonlein purpura (IgAV), a rare case

Background: Polyarteritis nodosa (PAN) is a vasculitis that affects medium-sized arteries. PAN is a rare disease and requires a high clinical suspicion for diagnosis. PAN and HSP (newly named Immunoglobulin A-associated vasculitis) have narrowing differential diagnosis. Here, we reported a case of PAN. Case presentation: Our patient was a 65-year-old woman that came to hospital due to abdominal pain and skin lesion on the right upper and right lower extremities. All rheumatologic tests were negative. A biopsy of the skin lesion was reported as mild hyperkeratosis, slight spongiosis with intact basal layer. The dermis showed moderate to severe perivascular PMN infiltration with vessel wall degeneration and extravasation of RBCs. A colonoscopy reported diffuse mucosal erythema and erosions were seen in the rectum until 6cm of anal verge. An electromyogram test and nerve conduction velocity study of the upper extremities reported bilateral mild carpal tunnel syndrome, and in the right lower extremities mononeuritis multiplex could not be ruled out. Abdominopelvic CT scan reported diffuse wall thickening of terminal ileum associated with mesenteric fat and narrow enhancement of inferior Mesenteric artery with patchy filling defect. After evaluation, the patient received corticosteroid pulses plus cyclophosphamide. Conclusion: Diagnosis and treatment of PAN are important and PAN should be considered in a patient with skin lesions and neurological impairment.

Case Report: Polyarteritis nodosa or complicated Henoch-Schonlein purpura, a rare case.

Background: Polyarteritis nodosa (PAN) is a vasculitis that affects medium sized arteries. PAN is a rare disease and requires a high vilgilance for diagnosis. For instance, PAN and Henoch-Schonlein purpura (HSP) have narrowing differential diagnosis. Here, we report a case of PAN. Case presentation: Our patient was a 65 year old woman that came to hospital due to abdominal pain and skin lesion on the right upper and right lower extremities. All rheumatologic tests were negative. A biopsy of the skin lesion was reported as mild hyperkeratosis, slight spongiosis with intact basal layer. The dermis showed moderate to severe perivascular PMN infiltration with vessel wall degeneration and extravasation of RBCs. A colonoscopy reported diffuse mucosal erythema and erosions were seen in the rectum until 6cm of anal verge. An electromyogram test and nerve conduction velocity study of the upper extremities reported bilateral mild carpal tunnel syndrome, and in the right lower extremities mononeuritis multiplex could not be ruled out. Abdominopelvic CT scan reported diffuse wall thickening of terminal ileum associated with mesenteric fat and narrow enhancement of inferior Mesenteric artery with patchy filling defect. After evaluation, the patient received corticosteroid pulses plus cyclophosphamide. Conclusion: Diagnosis and treatment of PAN is important and PAN should be considered in a patient with skin lesions and neurological impairment.

Rheumatology training in India compared to the United Kingdom

Background: Rheumatology is an emerging specialty in India, and there is a huge demand for trained rheumatologist to satiate the overwhelming musculoskeletal disease burden in India. Rheumatology training in the UK is structured and comprehensive. This study was aimed to explore the strengths and weaknesses in the rheumatology training in India as compared to the UK. Methods: This was an observational semiqualitative study. An online questionnaire was circulated electronically to rheumatology trainees across the UK and India. The questionnaire was designed to explore the curriculum content, training and research opportunities, training assessment methodologies, and employability in two countries. Results: A total of 77 respondents participated in this study, 52% (40/77) were from the UK and 48% (37/77) from India. We noted heterogeneity in the structure and delivery of training with multiple incongruous training curricula being prevalent in India. Institutional academic training supervision was comparable in both countries. UK trainees received comparatively more structured supervision for procedural skills. Indian trainees were proficient with laboratory-based rheumatological and immunological tests. Professional research and academic programs were incorporated in UK training. Mandatory training for generic skills was lacking in India. Specialty exit examination was mandatory in both countries. Employment opportunities were better perceived in the Indian subcontinent. Conclusions: In this study comparing rheumatology training between the UK and India,a lack of structured and coherent national curriculum in India was noted. Harmonisation of rheumatology training in India is essential to be at par with the well-established postgraduate rheumatology training curriculum in developed countries.

Thiamine Deficiency Mimicking Guillain-Barré Syndrome

SESSION TITLE: Critical Care 4 SESSION TYPE: Affiliate Case Report Slide PRESENTED ON: Tuesday, October 31, 2017 at 07:30 AM - 08:30 AM INTRODUCTION: Thiamine (Vitamin B1) is an essential vitamin utilized in a number of cellular functions and metabolic pathways. Thiamine deficiency can be associated with peripheral neuropathy (termed dry beriberi), lactic acidosis and cardiovascular insufficiency (wet beriberi). Shoshin beriberi refers to a variant of this condition characterized by acute onset and rapidly progressive deterioration of cardiovascular function which, if not treated could result in cardiovascular collapse and death. CASE PRESENTATION: A 47 year old Cambodian male with no significant past medical history presented with numbness, tingling and progressive weakness of his lower extremities for a month. He drank alcohol occasionally. He was alert and oriented but had decreased lower extremity strength and decreased patellofemoral reflexes bilaterally. Lab work was significant for hyponatremia, hypokalemia, mild renal failure and an elevated lactic acid of 6.1 mg/dL. He was admitted and given fluids for presumed dehydration but had persistent weakness and lactic acidosis. On the third day of his admission, he developed hypotension requiring vaseopressor support. DISCUSSION: An extensive initial work-up including cultures, HIV, hepatitis panel, urine toxicology screen, RPR and rheumatologic testing was negative. An echocardiogram showed normal left ventricular function and evidence of pulmonary hypertension and mild right ventricular dysfunction. CT of the head, cervical spine, chest, abdomen and pelvis were all unremarkable. Electromyography showed demyelination and a diagnosis of Guillain-Barre Syndrome was considered, despite a lumbar puncture showing normal protein levels in the CSF. The patient was administered IVIG. A thiamine level sent on day 2 of his admission resulted at 7 nmol/L (normal 8 -30) 5 days after it was sent confirming a diagnosis of thiamine deficiency. Aggressive thiamine repletion was started, IVIG was stopped and the patient improved with rapid normalization of his lactic acidosis and blood pressure. He also slowly recovered his motor strength. CONCLUSIONS: Severe thiamine deficiency is rare in developed countries, and is likely vastly under-detected in the absence of standardized testing. As has been previously reported1, this case highlights to physicians caring for critically ill patients the potential for missed diagnoses and poor outcomes in patients with thiamine deficiency. Reference #1: 1. Koike, et al “Rapidly developing weakness mimicking Guillain-Barré syndrome in beriberi neuropathy: two case reports.” Nutrition, 2008 DISCLOSURE: The following authors have nothing to disclose: Anton Edwards, Nader Azab, Gerardo Carino No Product/Research Disclosure Information

Massive Ascites in the Absence of Liver Disease: A Rare Case of Pseudo-pseudo Meigsʼ Syndrome

Pseudo-pseudo Meigs' syndrome (PPMS) is an extremely rare phenomenon seen in patients with Systemic Lupus Erythematosus (SLE), described by the presence of large volume exudative ascites, pleural effusions, and an elevated CA-125. In the below case, we depict a young patient who presented with massive abdominal ascites, pleural effusions, significant lymphadenopathy, and hypoalbuminemia. Although rare and of uncertain etiology, PPMS is becoming more recognized in the literature, and earlier detection is crucial in avoiding an extensive and costly workup. Patient is a 23-year-old female, who presented to the emergency department for 8 months of progressively worsening abdominal distention and pain. Patient underwent extensive workup including CT abdomen/pelvis, which showed massive abdominal/pelvic ascites, and enlarged inguinal lymph nodes without signs of cirrhosis or splenomegaly. Subsequent paracentesis removed 10 liters of clear fluid with serum ascites albumin gradient of 0.2. No malignant cells were identified. Hepatology was consulted and concluded no evidence of liver abnormality. Additional lab work ruled out cardiac or renal causes, but did show elevated CA-125. Bone marrow biopsy showed no evidence of myelodysplasia. PET scan showed marked lymphadenopathy of the head, neck, thorax, and pelvis, but ultrasound guided excisional lymph node biopsies revealed benign lymphoid hyperplasia. Quantiferon gold testing for tuberculosis was negative. On the brink of exploratory laparotomy, the patient was tested for SLE. She had multiple positive rheumatologic tests consistent with lupus and was formally diagnosed with SLE by rheumatology. Her condition has been stable on prednisone and hydroxychloroquine. PPMS is an extremely rare manifestation of SLE that should be on the differential for patients who present with ascites of unknown etiology. Other SLE related gastrointestinal manifestations are clinically important as well and include intestinal pseudo-obstruction, mesenteric vasculitis, protein losing enteropathy, and acute pancreatitis. Unfortunately, SLE is often overlooked in these patients. If diagnosed in a timely manner, further extensive and costly workup, including surgery may be avoided. The general consensus among experts is to treat the underlying SLE. Prognosis based on the few cases that have been published is good, as patients usually respond to corticosteroids, and in more refractory cases, immunomodulators or immune-suppressors.Figure: Massive Abdominal Ascites.

Inflammatory arthritis: An under-recognized immune-relate adverse effect.

e14565 Background: Checkpoint inhibition (CPI) has significant and durable effectiveness against a broadening range of cancers and currently is FDA approved for more than 10 different malignancies. With increasing use of CPI, there is a need to understand the range and extent of immune-related adverse effects (irAE). Though certain irAEs, such as colitis and pneumonitis have been well studied, CPI- induced arthritis (CA) has not been widely recognized nor well characterized with data on the subject largely comprised of case reports and small case series. Methods: We retrospectively reviewed 125 patients (pts) with advanced melanoma treated with CPI to define the incidence and clinical features of CA. We identified 20 pts who developed CA during the course of CPI treatment. Cases were included only if active rheumatic signs or symptoms developed after receiving therapy. Demographic data (gender, age), type of CPI, number of CPI cycles, treatment of CA and other irAE manifestations were extracted from the medical chart. Results: 16% of patients treated with CPI developed CA. Among the 20 pts, the average age at CA onset was 70.3 (43, 84) with 75% of pts male. 80% had received anti-PD-1 monotherapy, 15% combination anti-PD-1 + anti-CTLA-4 and 5% monotherapy with CTLA-4 inhibition. The average number of infusions prior to symptom onset was 13.5 (1-48) after a median time point of 6.6mths on therapy. Basic rheumatologic testing (RF, CCP, ANA) was only positive in 10% of cases. 20% of pts were treated with non-steroidal anti-inflammatory drugs alone, whereas 40% required corticosteroids (systemic or injection). The remaining 40% necessitated treatment with disease modifying anti-rheumatic drugs. Conclusions: CA is a debilitating irAE with a higher incidence (16%) than most other irAEs and a negative impact on the quality of life of pts affected. Based on our retrospective review, this irAE tends to delayed in onset and often requires systemic immune suppressants for effective treatment. We posit that this is an under recognized and undertreated irAE. Further work is needed to better define the clinical factors that may affect or predict its development.

Cervicocephalic Arterial Dissection

Cervicocephalic arterial dissection is not a common disease (2.5 % of total brain infarction) and its pathogenesis is still not fully understood. However, it seems that it is multifactorial And rarely seen in the postpartum priod.A 40-year-old female patient who referred to emergency department because she experienced right lower limb Paresis 10 days following the delivery and her Paresis was progressed within 72 hours. She has no history of DM ,HTN, preeclampsia and underlying cerebrovascular diseaseOn evaluations, Brain MRI revealed multiple infarction in the left MCA territory . ECG, TTE ,coagulation and rheumatologic tests were normal. leftextracranial carotid artery dissection was found in the cervical MRA.The likelihood of postpartum dissection is rare , But it could be due to vasculardamage associated with the Valsalva maneuver during labor ; hemodynamic and hormonal changes due to pregnancyare also involved.

Atypical inflammation of the middle ear

The objective of the present study was to characterize the patients presenting with atypical inflammation of the middle ear and consider the currently available methods for their examination. A total of 20 patients at the age from 16 to 66 years were admitted to the Department of Ear Microsurgery during the period from 2008 and 2016 for the treatment of atypical inflammation of the middle ear. Eleven of them (18 ears) were found to have tuberculous lesions (TL) of the middle ear while the remaining 9 patients (11 ears) suffered giant cell vasculitis (GCV). All the patients underwent the general clinical and otorhinolaryngological examination, computed tomography of the temporal bones and the thoracic cavity organs, cytological, bacteriological, pathomorphological, and molecular-genetic studies including PCR diagnostics, rheumatological tests, as well as counseling by a phthisiotherapist and rheumatologist. The primary localization of TL in the middle ear was documented in 6 patients including its association with lung lesions in 5 cases. The clinical picture of the disease in 5 patients was that of smoldering exudative pathology and in 6 ones was accompanied by suppurative perforative otitis media. According to the laboratory analyses, bacteriological diagnostics proved efficient in 9% of the patients, pathomorphological and cytological diagnostics in 18% and 27.3% of the cases respectively while the effectiveness of PCR diagnostics was estimated at 55%. The diagnosis in individual patients was established within the period from 1 month to 1.5 years after they first sought medical advice in connection with complaints of the ear disease. Tuberculosis of the middle ear began to develop as exudative middle otitis that acquired the form of bilateral pathology in 4 patients. Three patients had a concomitant pulmonary disease. In 4 patuents, the diagnois of middle ear tuberculosis was established based on the presence of the specific antibodies and in 5 ones based on the results of the pathomorphological study. All the studies were carried out in duplicate. The period between the beginning of the disease and the establishment of the definitive diagnosis varied from 1 month to 1 year.