Rheumatologic Causes Research Articles

Classification and Regression Tree Analysis in Adult Hemophagocytic Syndrome: Identifying Acute Heart Failure Predictors in ICU Patients.

Hemophagocytic syndrome (HPS) is a rare but life-threatening condition often complicated by heart failure (HF). This study aimed to identify predictors of acute heart failure in HPS patients using classification and regression tree (CART) analysis. A retrospective analysis was performed on 146HPS patients without a diagnosis of HF. Variables such as age, cardiothoracic ratio (CTR), etiology, N-terminal pro-brain natriuretic peptide (NT-proBNP), C-reactive protein (CRP), ferritin, triglyceride, and lactate dehydrogenase (LDH) levels at diagnosis were included. CART analysis was employed to develop models predicting heart failure status. The model's performance was evaluated using sensitivity, specificity, and overall accuracy. This study identified several key predictors of acute heart failure in HPS patients. CTR emerged as the most significant predictor, with patients exhibiting a higher ratio being at a greater risk of developing heart failure. NT-proBNP levels and CRP levels were also significant predictors, indicating cardiac stress and systemic inflammation. Age and etiology played crucial roles, with older patients and those with rheumatological causes showing a higher susceptibility to heart failure. The CART models demonstrated good accuracy, with CTR being the most important predictor. This study highlights critical factors, such as CTR, NT-proBNP, CRP levels, age, and etiology in predicting acute heart failure in HPS patients. Early identification of these predictors can facilitate timely interventions, potentially improving outcomes and reducing mortality rates. These findings provide valuable insights for clinical practice and pave the way for further research on acute heart failure management in HPS.

Larynx infiltration is not always a neoplasm – ultrarare rheumatological cause of hoarseness

Larynx infiltration is not always a neoplasm – ultrarare rheumatological cause of hoarseness

Life-threatening constrictive pericarditis accompanied by synthetic cannabinoid use: A case report and literature review

Rationale: Synthetic cannabinoids are increasingly used as recreational drugs and have been associated with adverse cardiovascular effects. However, reports of synthetic cannabinoids accompanied by constrictive pericarditis are limited. Patient’s concern: A 28-year-old male with a history of synthetic cannabinoid (Bonzai) abuse presented with chest discomfort, dyspnea, and lower extremity edema. Investigations revealed reduced left ventricular ejection fraction, elevated inflammatory markers, low electrocardiogram voltages, and atrial fibrillation. Diagnosis: Chest spiral computerized tomography scan and chest X-ray demonstrated pericardial calcification. Cardiac magnetic resonance imaging and right heart catheterization were done to confirm the possibility of constrictive pericarditis. Based on the patient’s addiction history and exclusion of rheumatologic and infectious causes, it was supposed that constrictive pericarditis and cardiomyopathy may be accompanied by synthetic cannabinoid use. Interventions: The patient received standard medical therapy, including loop diuretics for cardiomyopathy and constrictive pericarditis. Catheter ablation was recommended for his rhythm control, and he was planned for close monitoring of clinical and echocardiographic response and evaluation of the need for surgical pericardiectomy in the future. Outcomes: After 6 months follow-up, echocardiographic exam revealed no significant improvement in ventricular function. However, due to the high surgical risk, the patient’s poor compliance, and the continuation of drug abuse, he was not a good candidate for surgery according to our heart team’s decision. Lessons: Synthetic cannabinoids can trigger constrictive pericarditis, and clinicians should consider them when evaluating patients with compatible symptoms and exposure history. Further research on the cardiovascular effects of synthetic cannabinoids is needed and public education on potential harms is warranted.

From silence to sound – a case of autoimmune ear disease

Autoimmune inner ear disease (AIED) is a rare rheumatological cause of sensorineural hearing loss, accounting for less than 1% of worldwide hearing loss. It presents with rapidly progressive, unilateral or bilateral SNHL, often fluctuating. The progression is over weeks to months, along with vestibular symptoms. Diagnosing the condition is challenging because no adopted standardized tests are available. Hence, AIED is a challenging diagnosis based on exclusion. Clinical characteristics and how the patients respond to immunosuppressive agents are used for the diagnoses of AIED. Only a few cases have been reported in India till now. Here, we present a rare case of Autoimmune Ear Disease without any underlying systemic autoimmune disease.

A172 A CLASSIC CASE OF WHIPPLE'S DISEASE

Abstract Background Whipple’s disease is a systemic disorder caused by the Gram-positive bacillus Tropheryma whippelii. It is classically defined by the four cardinal clinical manifestations of arthralgias, weight loss, diarrhea and abdominal pain. Classic Whipple’s disease is considered extremely rare, with an estimated global incidence of 30 cases per year, though this may be an underestimate. Aims To present a case of classic Whipple’s disease, so as to raise awareness and clinical suspicion for this rare condition. Methods We performed a retrospective chart review of a case of classic Whipple’s disease diagnosed in Saint John, NB, in 2020. Results A 55-year-old female presented to hospital with intractable weakness and diarrhea. Her history was notable for several years of right leg weakness, myalgias and low-grade fevers. More acutely, she presented to hospital due to worsening secretory diarrhea resulting in 15kg weight loss. Initial investigations were remarkable for an elevated C-reactive protein. The patient was admitted for coordinated evaluation for possible rheumatologic and infectious causes. As part of that workup, colonoscopy was performed. Colonoscopy revealed an abnormal ileum, with villi described as edematous with a mosaic pattern alternating between erythematous and “white tipped”. On histologic assessment, ileal biopsies showed macrophages with PAS-positive diastase resistant granules suspicious for Whipple’s disease. A follow-up esophagogastroduodenoscopy with duodenal biopsies was thus performed to gain further tissue for confirmatory testing. The duodenal mucosa was also noted to have a mosaic pattern with hypertrophic villi alternating between pale and erythematous in appearance. Duodenal biopsies were taken and sent for PCR testing. This testing was positive for T. whippelii, confirming the diagnosis of Whipple’s disease. The patient was initiated on long-term antibiotic therapy with ceftriaxone transitioning to trimethoprim-sulfamethoxazole prior to discharge. She unfortunately re-presented one month post-discharge with recurrent fevers, malaise and arthralgias. Given suspicion that her disease may be resistant to trimethoprim-sulfamethoxazole, she was transitioned to doxycycline and hydroxychloroquine as second-line therapy. She clinically improved on this regimen, and was able to be de-escalated to long-term doxycycline monotherapy after one year. At a recent follow-up, nearly three years from her initial diagnosis, she was doing well with essentially no ongoing symptoms of her Whipple’s disease. Conclusions Whipple’s disease is a rare infection presenting with intractable diarrhea and abdominal discomfort. Our case highlights the excellent clinical response that can be achieved with successful diagnosis and management of this otherwise debilitating disease. Funding Agencies None

Abstract 13671: A Case of Diffuse Alveolar Hemorrhage While on Systemic Anticoagulation for Impella

A 57 year old male with PMH of non-ischemic cardiomyopathy (EF 30%), atrial fibrillation, and VT s/p ICD was admitted to the cardiac intensive care unit for cardiogenic shock. His condition deteriorated necessitating Impella 5.5 implantation as bridge to cardiac transplant. Systemic heparin was started per Impella protocol with a lower anti-Xa goal of 0.2 - 0.3 given post-op bleeding at the R axillary Impella site. Five days post Impella placement he developed bloody secretions from the endotracheal tube requiring multiple blood transfusions and discontinuation of systemic heparin, while maintaining heparin purge. Labs were notable for anti-Xa 0.22, hemoglobin 9.7, and platelets 221. CT Chest demonstrated parenchymal opacities concerning for diffuse alveolar hemorrhage (DAH) which was confirmed via bronchoalveolar lavage; he had no known history of lung disease. High-dose corticosteroids were administered as a high ANA titer (1:128) and low C3 suggested capillaritis. However, decreasing ANA (1: 320) and a negative autoimmune workup decreased the likelihood of a rheumatologic cause for DAH and steroids were discontinued. DAH secondary to acquired von Willebrand syndrome (aVWS) was considered, but factor VIII and vWF activity levels were normal. The bloody secretions improved with inhaled thromboxane and systemic heparin was restarted. Orthotopic heart and kidney transplant was performed with subsequent Impella removal 104 days after Impella placement. Discussion: We present the first documented case of DAH with isolated Impella 5.5 support. Previously reported cases of DAH post-Impella were observed in patients with concurrent ECMO or RVAD. As autoimmune and coagulopathic causes of DAH were excluded, bland DAH secondary to systemic anticoagulation is most likely in this case. This raises a question about how to balance the risk of bleeding with the risk of device thrombosis, stroke and embolic events and when to switch from heparin to sodium bicarbonate purge.

E035 An evaluation of the causes and outcomes of haemophagocytic lymphohistiocytosis in Sheffield Teaching Hospitals: 2016 to 2021

Abstract Background/Aims Haemophagocytic lymphiohistiocytosis (HLH) is a rare, underrecognised hyperinflammatory syndrome, characterised by immune dysregulation. Without treatment, the ensuing cytokine storm leads to high mortality. Secondary HLH (sHLH) is triggered by malignancy, infection, autoimmunity and medicines; treatment with immunosuppression is consensus- rather than evidence-based and extrapolated from primary HLH. Sheffield hosts a mature HLH multidisciplinary advisory group (MDAG). Here we evaluate the cause, treatment, requirement for critical care and mortality of people with HLH managed through the MDAG in a period including the coronavirus pandemic but prior to NHS England approval of anakinra (IL-1 antagonist) for HLH. Methods This retrospective evaluation (approved locally STH 10850) identified patients from MDAG records 1st October 2016 to 30th September 2021. Data from electronic/paper records was analysed using Microsoft Excel. Results HLH triggers were infection (viral 34%, bacterial 10%), haematological (35%), rheumatological (13%) and other (8%). Rheumatological causes were Still’s disease (n = 5); antiphospholipid syndrome (n = 2); JO1 dermatomyositis (n = 1); SLE (n = 1); and rheumatoid arthritis (n = 1). Other causes included unknown (n = 3); combined systemic JIA and sickle cell crisis (n = 1); medication (alemtuzumab) (n = 1); and primary HLH (n = 1). Overall mortality was 53% and highest in HLH with a haematological malignancy trigger (82%) Prior to the COVID19 pandemic (pre-March 2020), the commonest trigger of HLH was haematological malignancy (47%); after March 2020, the commonest trigger was infection (64%); COVID-19 explained 42% of cases. Mortality fell from 72% to 31%. Conclusion In this real-world series of people with HLH, mortality and critical care requirement was high. HLH triggers reflect published evidence as does poor prognosis in haematological malignancy-associated HLH. No-HLH associated with non-haematological malignancy was identified; we may need to improve MDAG reach into oncology. Seeming reduction in mortality following the COVID-19 pandemic may reflect increased recognition of COVID-19 induced hyperinflammation along with locally-agreed access to anakinra for COVID-19-induced HLH. The increase in infection related HLH cases since March 2020 is explained largely by COVID-19 cases. This has led to a relative reduction in cases related to haematological malignancy. HLH requires multidisciplinary management and better research to improve treatment. Disclosure B. Awdas: None. T. Nadin: None. R. Tattersall: None.

Adult-onset Still’s disease

Adult-onset Still's disease (AOSD) is arare autoinflammatory disease characterized by intermittent fever and acombination of symptoms, such as an evanescent rash synchronous with fever, arthralgia/arthritis, lymphadenopathy and hepatosplenomegaly. The diagnosis is based on acharacteristic constellation of symptoms and the exclusion of infections, hemato-oncological diseases, infectious diseases and alternative rheumatological causes. The systemic inflammatory reaction is reflected by high levels of ferritin and C‑reactive protein (CRP). The pharmacological treatment concept includes glucocorticoids often in combination with methotrexate (MTX) and ciclosporine (CSA) for reduction of steroids. The interleukin 1 (IL-1) receptor antagonist anakinra, the IL-1beta antibody canakinumab or an IL‑6 receptor blockage with tocilizumab (off label for AOSD) are used where there is no response to MTX or CSA. Anakinra or canakinumab can be used as aprimary option in AOSD in cases of moderate and severe disease activity.

Adult-onset Still's disease : Diagnosis and treatment according to the new S2e guidelines of the German Society of Rheumatology (DGRh)

Adult-onset Still's disease (AOSD) is arare autoinflammatory disease characterized by intermittent fever and acombination of symptoms, such as an evanescent rash synchronous with fever, arthralgia/arthritis, lymphadenopathy and hepatosplenomegaly. The diagnosis is based on acharacteristic constellation of symptoms and the exclusion of infections, hemato-oncological diseases, infectious diseases and alternative rheumatological causes. The systemic inflammatory reaction is reflected by high levels of ferritin and C‑reactive protein (CRP). The pharmacological treatment concept includes glucocorticoids often in combination with methotrexate (MTX) and ciclosporine (CSA) for reduction of steroids. The interleukin 1 (IL-1) receptor antagonist anakinra, the IL-1beta antibody canakinumab or an IL‑6 receptor blockage with tocilizumab (off label for AOSD) are used where there is no response to MTX or CSA. Anakinra or canakinumab can be used as aprimary option in AOSD in cases of moderate and severe disease activity.

Macrophage Activation Syndrome in Adult Onset Still’s Disease: A Life Threatening Complication

Adult onset Still’s disease (AOSD), a multi-systemic inflammatory disorder, is a rare disorder but an important differential to be considered in patients of Pyrexia of Unknown Origin, especially if patient presents with polyarthritis. Macrophage activation syndrome (MAS), a subset of Secondary Hemophagocytic Lymphohistiocytosis (HLH), considered as the most life threatening complication of AOSD, mostly develops around the onset of disease. Hence, in a previously undiagnosed case of AOSD, recognizing MAS as a presenting feature complicating underlying AOSD is essential for increased patient survival, as in our case.
 
 
 A 39 year old diabetic female presented with high grade fever with chills and multiple joint pains symmetrically involving proximal shoulder, knees and distal joints (involving MCP, PIP and DIP joints of hand) associated with swelling and early morning stiffness, relieved on activity, since past 6 months. No history of oral ulcers, rash, jaundice, weight loss, loss of appetite, foreign travel or close animal contact.
 
 On examination, Pallor was present, Blood Pressure was 120/70 mm Hg, pulse rate was 102/min and temperature was 100 F. On per abdomen, mild hepatomegaly was present and other system examination was unremarkable.
 
 Routine laboratory findings on day of presentation have been summarised (Table 1.1). Ultrasound Abdomen revealed hepatomegaly and Chest X-ray showed signs of old infective foci. Peripheral smear for Malarial parasite, Ns1Ag and dengue serology, Widal test and Montoux test were negative. Blood cultures and urine cultures were sterile. X-Ray of joints were normal. USG of B/L Knee joints showed mild joint effusion bilaterally with no internal echoes (non tappable). Trans-thoracic echo and Trans-esophageal echo were normal. CT chest, neck and abdomen showed old tubercular changes in lung. Tests for atypical bacterial infections which was normal (RK 39, Chickungunya, Brucella, Leptospirosis and Scrub typhus). Fever did not respond to Broad Spectrum Antibiotics, Antimalarials or Antitubercular therapy and patient continued to have persistent fever spikes.
 
 Autoimmune profile showed ANA and RA Factor negative but markers of inflammation were raised: CRP- 521, ESR -148, IL-6 – 84.0, Procalcitonin-6.9 and S.Ferritin >2000. During the work up for Anemia, Microcytic hypochromic anemia was found with Elevated NAP SCORE-165. Incidentally, her triglycerides were found to be elevated (356) and a reduced fibrinogen. With these results, we planned for a bone marrow aspirate and biopsy which showed increased myeloid preponderance (32:1), increased histiocytes and evidence of hemophagocytosis.
 
 The 2004 diagnostic criteria of HLH was fulfilled. After ruling out almost all infectious and malignant causes of secondary HLH, we searched for a rheumatologic cause. Thus diagnosis of AOSD (after fulfilling Yamaguchi’s criteria) with MAS (a subset of 2’ HLH) was made. Patient had dramatic improvement after receiving steroids with her fever episodes and joint pains settling completely thereafter.
 
 
 Prompt recognition of life threatening complications like MAS which pose diagnostic difficulty due to overlapping features in a patient of AOSD, should be done at the earliest to improve patient prognosis and survival. Serum Ferritin levels can be considered a useful marker to assess the disease activity and to predict MAS occurrence in such patients.

PSUN265 Metformin-Induced Severe Lactic Acidosis Requiring Urgent Dialysis Associated With Influenza A Infection in a Patient With Previous Preserved Renal Function: Case Report

Abstract Background Although rare, metformin-induced lactic acidosis it's a severe acute condition, that requires immediate recognition and management due to its high lethality. Clinical case Female, 70-years-old, presents to the E.R. with generalized malaise, weakness and myalgia for 4 days associated with diarrhea and vomits for 2 days. Denies fever or flu-like symptoms. History of hypertension and T2DM using metoprolol 100mg/day, perindopril 10mg/day, indapamide 2.5mg/day, amlodipine 5mg/day, linagliptin 5mg/day, rosuvastatin 10mg/day, gliclazide 120mg/day, aspirin 100mg/day, clonidine 0.3mg/day and metformin XR 1g/day. On clinical exam she was dehydrated and tachypneic with no other important findings. Laboratory showed glucose 31mg/dL, severe metabolic acidosis - pH 6,0; HCO3 5,4mmol/L; lactate 11.6mmol/L (n<1.6mmoL/L), acute renal failure KDIGO 3, creatinine 8.08mg/dL eGFR 5mL/min/1.73m2 (patient's baseline 0.9mg/dL), BUN 213mg/dL (n<50mg/dL), potassium 6.8mEq/L (3.5>n<5.1mEq/L), phosphor 14.9mg/dL (2.7>n<4.5mg/dL), chlorine 92mEq/L (98>n<107mEq/L), magnesium 1.7mg/dL (1.6>n<2.6mg/dL), calcium 0.95mmol/L (1.16>n<1.32mmol/L), and normal blood count. She was admitted to the ICU, hemodynamically stable with no need for vasoactive amine, breathing under nasal cannula and proceed with emergency hemodialysis. Acidosis was corrected after 6h of hemodialysis. Patient turned eupneic in room air and presented renal function improvement (BUN 44mg/dL; Creatinine 2.66mg/dL). She continued under intensive care and daily hemodialysis for another 4 days. The etiological investigation excluded glomerulopathies of rheumatological cause, blood cultures, serology for hepatitis, HIV and Dengue were all negative. However, respiratory viral panel was positive for influenza A. Although metformin serum dosage was not performed, renal function stability without the need for hemodialysis was only achieved after discontinuation of metformin. The main diagnostic hypothesis was metformin-induced severe metabolic acidosis. Finally, the patient was discharged on day 10, with stable renal function without hemodialysis (creatinine 2.19mg/dL eGFR 22mL/min/1.73m2) and no neurological deficits. The patient was referred to the endocrinologist and nephrologist to follow-up. Conclusion Metformin is affordable, safe and widely prescribed since the 50's. Although rare, metformin-induced lactic acidosis is a lethal toxicity, with identifiable risk factors that must be considered by prescribers. References Lazarus, Benjamin et al. "Association of Metformin Use With Risk of Lactic Acidosis Across the Range of Kidney Function: A Community-Based Cohort Study." JAMA internal medicine vol. 178,7 (2018): 903-910. doi: 10.1001/jamainternmed.2018.0292 Salpeter, Shelley R et al. "Risk of fatal and nonfatal lactic acidosis with metformin use in type 2 diabetes mellitus." The Cochrane database of systematic reviews vol. 2010,4 CD002967. 14 Apr. 2010, doi: 10.1002/14651858.CD002967.pub4 Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.

SINGLE CAVITARY LUNG LESION IN A HEALTHY YOUNG MALE

SINGLE CAVITARY LUNG LESION IN A HEALTHY YOUNG MALE

UNCOMMON CAUSES OF RECURRENT UNILATERAL PLEURAL EFFUSIONS

UNCOMMON CAUSES OF RECURRENT UNILATERAL PLEURAL EFFUSIONS

Anakinra for the treatment of adult secondary HLH: a retrospective experience.

Anti-cytokine therapies have been gaining attention as a means of improving outcomes in adult secondary HLH (asHLH), which currently has poor outcomes when treated with standard etoposide-based therapies. Anakinra is an interleukin-1 antagonist that is increasingly being used in the management of asHLH. Here is described a multi-hospital series of 16 adult patients with secondary HLH treated with anakinra. Provoking factors of secondary HLH included hematologic malignancy (n = 7, 44%), bacterial infection (n = 7, 44%), viral infection (n = 5, 31%), rheumatologic disorder (n = 4, 25%), and unknown (n = 1, 6%). Five patients remained alive at time of last follow-up (OS = 31%). Median OS was 1.7 months from initiation of anakinra (range 0.2–59). OS among patients with rheumatologic causes of secondary HLH was 75%, whereas only 17% of patients with other provoking factors survived (p = 0.0293). Anakinra was well tolerated, with only 1 patient experiencing associated toxicity (grade 3 liver injury). Anakinra may be useful in the management of asHLH provoked by rheumatologic conditions, although its benefit in asHLH provoked by other factors may be limited.

Gentle Giant: An Unusual Cause of Heart Block

<h3>Introduction</h3> Inflammatory cardiomyopathies can be a diagnostic dilemma. Early management can lead to reduced morbidity and mortality for patients. We describe a rare presentation of an unusual cardiomyopathy. <h3>Case Report</h3> A 58-year-old female presented with a 10-day prodrome of cough, NYHA class III dyspnea, and fatigue with minimal symptoms of orthopnea, paroxysmal nocturnal dyspnea or peripheral edema. She was previously healthy with no cardiac medications. Family history was significant for granulomatosis with polyangiitis. COVID-19 swab was negative. She was symptomatic with transient complete heart block and junctional escape of 20bpm. A temporary transvenous pacemaker was placed. Echocardiogram showed biventricular dysfunction with left ventricular ejection fraction < 30%. Troponin and brain-natriuretic peptide were elevated. Coronary angiogram showed no significant occlusions. CT excluded pulmonary embolism, pneumonia, or adenopathy. She was initiated on heart failure medications but beta blocker was not started given heart block. Six days into admission, her heart failure improved but she developed transient complete heart block without junctional escape. There was no ventricular ectopy. Evaluation for rheumatologic, infectious, and inflammatory causes showed elevated C-reactive protein and antineutrophil cytoplasmic antibodies. The remainder of the workup was negative. Leading diagnoses were idiopathic giant cell myocarditis or cardiac sarcoidosis. An endomyocardial biopsy revealed multinucleated giant cells and myocyte necrosis. She was diagnosed with giant cell myocarditis. Prior to discharge, she had defibrillator insertion and was initiated on prednisone and tacrolimus. Shortly after this time, she returned with critical cardiogenic shock despite intensification of immunosuppressive therapies and was listed for cardiac transplant. <h3>Summary</h3> Giant cell myocarditis (GCM) is a rare and often fatal autoimmune cause of heart failure. Patients frequently present with congestive heart failure, ventricular arrhythmia and a rapid progression of symptoms. In GCM, it is rare to present with atrioventricular conduction delays. Given the crossover in symptoms with sarcoidosis and GCM, diagnosis may be challenging. In this case of an acute presentation of heart failure and complete heart block, endomyocardial biopsy was central to the diagnosis and management of GCM.

Hemophagocytic Lymphohistiocytosis Associated With Endocarditis: A Case Years in the Making.

Hemophagocytic Lymphohistiocytosis Associated With Endocarditis: A Case Years in the Making.

105 An intriguing case of pericardial effusion after acute coronary syndrome

Abstract Aims Left ventricular free wall rupture is one of the mechanical complications of myocardial infarction with an incidence of 2–4%. Sometimes the myocardial rupture hasn’t an immediate fatal evolution, nor is easy to be diagnosed because it is contained by the pericardium and thrombus formation, leading to pseudoaneurysm of the left ventricle. Pseudoaneurysms need a prompt surgical correction for their high risk of rupture both in the acute phase and later. Methods and results A 57 years old, smoker, woman with no previous cardiological history was admitted to our cardiological department for acute coronary syndrome with persistent ST segment elevation involving the postero-lateral wall (door to balloon time about 10 h, Killip class I and peak hs-TnI value was 27.67 ng/ml n.v. ˂0.02 ng/ml). The echocardiography showed mild left ventricular disfunction (LV EF 45%), postero lateral akinesia and moderate mitral regurgitation; ubiquitous pericardial effusion (1 cm) was present, particularly along the anterior left ventricular wall, with irregular echo-dense aspect. She underwent urgent coronarography that showed a critical stenosis of the distal third of the left circumflex and a thrombotic occlusion of the first marginal branch. The distal circumflex was treated with angioplasty and stent implantation but we couldn’t obtain the reperfusion of the marginal branch. Post procedural echocardiogram was unchanged and no free wall rupture was detected. 7 days after the admission, the patient showed persistent elevation of white blood count and CRP and developed fever, promptly empirically treated with a cephalosporin (blood cultures collected before were negative). After two new episode of fever with persisting biochemical flogistic parameters, a rheumatologic cause of the pericardial effusion was considered in the presence of positive antinuclear antibodies suggesting the diagnosis of Systemic Lupus Erythematosus. Steroidal therapy was prescribed which caused clinical improvement without complete resolution of the pericardial effusion. On day 20 of hospital stay a new echocardiographic evaluation showed a discontinuation of the postero lateral myocardial wall (Figure 1), about 1 cm in width, widely communicating with the left ventricular cavity and suggestive for a left postero-lateral ventricular pseudoaneurysm. The patient underwent surgical intervention and it was possible to expose a big clot occluding the pseudoaneurysmatic cavity communicating with the left ventricular chamber through an inlet about 1 cm in diameter, that was repaired with a bovine pericardial patch (Bard Sauvage technique). After surgery the patient was supported with an IABP and inotropes and was discharged to a rehabilitation structure on day 29 of hospital stay. Four months after the hospital discharge the patient died for a recently diagnosed pancreatic cancer. Conclusions Pseudoaneurisms are life-threatening complications of myocardial infarction that sometimes are hardly diagnosed. When correctly recognized surgical correction can lead to a good prognosis.

Post-cardiac injury syndrome triggered by radiofrequency ablation for AVNRT

BackgroundPost-cardiac injury syndrome (PCIS) is an inflammatory condition following myocardial or pericardial damage. In response to catheter ablation, PCIS most frequently occurs after extensive radiofrequency (RF) ablation of large areas of atrial myocardium. Minor myocardial injury from right septal slow pathway ablation for atrioventricular nodal reentrant tachycardia (AVNRT) is not an established cause of the syndrome.Case presentationA 62-year-old women with a 6-year history of symptomatic narrow-complex tachycardia was referred to perform an electrophysiological study. During the procedure AVNRT was recorded and a total of two RF burns were applied to the region between the coronary sinus and the tricuspid annulus. Pericardial effusion was routinely ruled out by focused cardiac ultrasound. In the following days, the patient developed fever, elevated inflammatory and cardiac markers, new-onset pericardial effusion, characteristic ECG changes, and complained of pleuritic chest pain. An extensive workup for infectious, metabolic, rheumatologic, neoplastic, and toxic causes of pericarditis and myocarditis was unremarkable. Cardiac magnetic resonance imaging showed no signs of ischemia, infiltrative disease or structural abnormalities. The patient was diagnosed with PCIS and initiated on aspirin and low-dose colchicine. At a 1-month follow-up visit the patient was free of symptoms but still had a small pericardial effusion. After three months of treatment the pericardial effusion had resolved completely.ConclusionsInflammatory pericardial reactions can occur after minor myocardial damage from RF ablation without involvement of structures in close proximity to the pericardium.

P097 Raised inflammatory markers with unclear cause; what do they tell us? An audit of patients presenting to rheumatology service at RNHRD, Bath

Abstract Background/Aims Unexplained persistently raised inflammatory response in patients’ leads to concern for clinicians. Different studies have demonstrated increased risk of mortality in patients with persistently raised inflammatory response. Rheumatology input is often sought in these cases when cause of inflammatory response is unclear. We conducted this project in our department to see the progress of new patients with raised inflammatory markers presenting to the rheumatology service. Methods Patients referred to the rheumatology outpatients with raised inflammatory markers (Plasma viscosity &amp;gt;1.85 or CRP&amp;gt;100) from April to November 2018 were included. Patient demographic information was gathered through patient’s records and imaging information was gathered through PACS system. Information about investigative modalities was included. Treatment recommendations were also included. Patients were followed up for 6 months and any change in their diagnosis was also incorporated into the data. Results 169 patients satisfied the inclusion criteria. Median age was 58 years. Majority were females (72%). Mean referral to seen time was 3.43 weeks. Mean elevation in CRP and PV was 10 and 1.93 respectively. A diagnosis of mechanical/non rheumatological cause was made in 36% patients of those 11% were Fibromyalgia. Inflammatory arthritis diagnosis made in 34% patients. Autoimmune testing and radiological tests were performed in 97% and 90% patients respectively. 67% patients had malignancy or infection work up. Diagnosis was changed in 25% patients on follow up with investigation. With PV &amp;gt; 2.0 (44 patients), only 20% had non inflammatory conditions. There were 6 deaths in this cohort of patients (13% mortality). 3 new cases of malignancy were diagnosed. P097 Table 1:Investigative modalitiesInflammatory disorders N=number of scansNon-inflammatory conditionsCT100MRI1812PET CT71NM scan14 Conclusion Patients with persistently elevated inflammatory markers had a higher mortality rate than the other group over 6 months. Three quarters of patients with raised inflammatory markers remain under follow up of Rheumatology team for surveillance. Patients were reviewed in Rheumatology clinics in reasonable time. Appropriate investigative modalities were considered while investigating for inflammatory disorders. Further large scale studies are required to find an association between raised inflammatory response with mortality in rheumatology patients. Disclosure M. Jamal: None. R. Peck: None. R. Sengupta: None.

P105 Auto-inflammatory idiopathic serositis: an under-characterised phenomenon?

Abstract Background/Aims Serositis is a broad term encompassing inflammation of the pericardium, pleural or peritoneum. Serositis can be secondary to various conditions ranging from systemic lupus erythematous, inflammatory bowel disease and familial Mediterranean fevers. Due to the multitude of precipitating causes, idiopathic serositis is a diagnosis of exclusion and requires extensive medical work-up. This case series details patients who have developed inflammatory serositis without a clear underlying precipitating cause over the past 12 years at the Royal Free Hospital, a tertiary rheumatology centre based in London. Methods Patients were identified retrospectively through electronic records by searching through clinic letters and discharge letters from 2008 to 2020. Search terms including serositis, pericarditis, pleuritis and peritonitis were used. Data collected included their key demographic details, laboratory and pathology investigations as well as the course of treatment and follow up data, including rate of recurrence, complication and survival outcomes. Patients with underlying rheumatological, immunological, or malignant causes were excluded. Results Four hundred and twenty patients over 12 years with serositis were identified with 22 patients classified as idiopathic, auto-inflammatory serositis. Patient demographics indicate a 5:2 female preponderance for the condition with an average age of 61 (range 19-87) and Caucasian ethnicity (68.2%, 15/22). Patients were investigated for underlying causes, including screening for infection and autoimmune conditions, and imaging was performed including computer tomography and positive emission tomography. Autoimmune screening revealed weakly positive autoantibodies of low sensitivity (anti-neutrophil antibodies and rheumatoid factor) in 18.2% (4/22) patients. Treatment options varied, typically with immunosuppression being initiated acutely with steroid therapy, either oral prednisolone or intravenous methylprednisolone, depending on severity of disease presentation before progression to steroid-sparing agents, including mycophenolate motefil (50%, 11/22), cyclophosphamide (13.6%, 3/22) and azathioprine (9.1%, 2/22). Other therapies including colchicine (40.9%, 9/22) were used. Relapse was relatively uncommon once patients were established on steroid-sparing agents (21.7%, 5/22). However, Anakinra (interleukin-1 receptor antagonist) was initiated in patients with recurrent relapses (9.1%, 2/22) with good response. One patient died through bronchopneumonia following cyclophosphamide therapy. Of note, pericardial stripping was required in patients with haemodynamic compromise during admission in 3/22 (13.6%) with 1 patient in this subset subsequently requiring Anakinra therapy. Conclusion Auto-inflammatory idiopathic serositis appears to be distinct category of serositis affecting predominantly middle aged to elderly female Caucasian patients, without underlying connective tissue disease, requiring prolonged therapy with immunosuppressive agents, which can be characterised by involvement of multiple serous membranes and/or recurrent episodes of serositis. This condition appears to respond well to immune suppression with steroids, steroid-sparing agents and Anakinra. Further work will be required to characterise and classify this condition and to establish treatment guidelines. Disclosure K. Pillai: None. J. Perera: None. D. Kurzeja: None. S. Amin: None. M. Choudhury: None. R. Stratton: None.