Management Of Rheumatic Diseases Research Articles

AB1181 SHOULD A COMBINED RHEUMATOLOGY-PULMONOLOGY INTERSTITIAL LUNG DISEASE SERVICE BE CONFINED TO TERTIARY CENTRES - A SERVICE EVALUATION

Background:Interstitial lung disease is a well described extra-articular manifestation in a range of rheumatic diseases. It carries significant morbidity and mortality. Management of rheumatic diseases associated ILD (r-ILD) requires expertise as the needs of such patients are complex and treatment options limited. Historically, such complex ILD has been managed in tertiary referral centres.Objectives:We set up a combined service incorporating both rheumatology and respiratory domains in a district general hospital (DGH) to help patients avoid long journeys and improve their experience whilst focusing on an integrated care pathway. We evaluated the outcomes of the first set of patients managed in this proof-of-concept service model.Methods:Referrals were accepted from any hospital specialist involved in the management r-ILD. They were triaged by lead ILD pulmonologist to monthly ILD MDT comprising a rheumatologist, respiratory physician, a radiologist and ILD specialist nurse. Appropriate patients were booked into combined clinic, run by the respective rheumatology and chest specialists with ILD interest, attracting a multi-speciality tariff. All the data was recorded electronically with full access to demographics, disease parameters, investigations and drug management.Results:89 patients were included in this proof-of-concept. Mean age was 66.1 yrs (19-90 yrs) and 44% (n=39) were male. 35 (40%) had RA, 34 (39%) had CTD, eight (10%) had sarcoidosis, five had IPAF and seven others. Most predominant HRCT pattern was NSIP (n=53,60%) followed by UIP (n=23, 21%), sarcoid (n=10, 12%) and miscellaneous (LIP and mixed). Mean FVC was 2.64 L/min (1.93-4.13) with DLCOc of 52.7% (28.9-90.1%) predicted. Only two patients had all antibodies negative whilst 87 had at least one antibody positive with ANA being the most common (n=28).Most (83%) patients were treated with immunomodulators including nine with rituximab. 39 (44.3%) patients had significant improvement in clinical, imaging and pulmonary parameters with DLCOc improving to 56.57% and FVC to 2.70 L/min. There were similar improvements in six minute walk test. 17 patients died and 20 patients required long term oxygen therapy.Conclusion:This proof-of-concept real world study confirms the utility of a combined specialist service in a district general hospital. Nearly half of this complex and resource intensive patient cohort had good clinical outcomes and derived benefit from the expertise in one room. Feedback from both patients and referrers was unanimously positive. No patient required tertiary centre referral and all could be managed adequately in the clinical setting.Our report confirms that r-ILD can be managed in a DGH setting with a stream-lined service offering clear benefits to patients. We would argue that r-ILD service, congruent to satellite pulmonary hypertension clinics in secondary care with hub-and-spoke model liaison with tertiary centre, can be established on similar principles and could help over-stretched tertiary care with repatriation of services whilst helping develop local expertise in the management of chronic ILD.Disclosure of Interests:Karim Salama: None declared, Natasha Ramsundar: None declared, Vijay Joshi: None declared, Muhammad Khurram Nisar Grant/research support from: Muhammad Nisar undertakes clinical trials and received support (including attendance at conferences, speaker fees and honoraria) from Roche, Chugai, MSD, Abbvie, Pfizer, BMS, Celgene, Novartis and UCB, Consultant of: Muhammad Nisar undertakes clinical trials and received support (including attendance at conferences, speaker fees and honoraria) from Roche, Chugai, MSD, Abbvie, Pfizer, BMS, Celgene, Novartis and UCB, Speakers bureau: Muhammad Nisar undertakes clinical trials and received support (including attendance at conferences, speaker fees and honoraria) from Roche, Chugai, MSD, Abbvie, Pfizer, BMS, Celgene, Novartis and UCB

Reproductive health professional's reported knowledge on diagnosis and management of rheumatic heart disease in pregnant women in Maputo, Mozambique

BackgroundRheumatic heart disease (RHD) poses a threat to African women in their reproductive years, being an important cause of maternal mortality and poor foetal outcomes. Timely diagnosis and adequate management reduce significantly obstetric complications. Our study aimed to describe the knowledge of diagnosis and management of RHD in pregnant women among reproductive health professionals (RHP) working in a highly endemic area. MethodsThe study that took place in May/2017 in two conveniently selected health facilities. Doctors (residents and specialists) and mid-level (maternal and child health nurses/technicians, MLRHP) were invited to respond to an anonymous, self-administered and standardized survey (electronic and paper-based questionnaires), which contained closed and open-ended questions on pregnancy-related RHD diagnosis, treatment and complications. The responses were coded and analysed using SPSS version 20. ResultsSeventy-three RHP participated (27 doctors, 46 MLRHP). While RHP understand the fetal 49 (67%) and maternal 57 (53%) outcomes in presence of RHD, they are unprepared to diagnose, manage and refer them adequately. ConclusionRHP constitute a group that can be targeted for decentralization of diagnosis and management of RHD, a strategy that may be crucial to reduce maternal mortality by indirect causes in low-middle income countries.

Rheumatologic Medication Use During Pregnancy.

Chronic rheumatic diseases often occur in women of reproductive age, and the effect rheumatic disease has on pregnancy varies depending on the condition. Medical management of rheumatic diseases during pregnancy may prevent joint or organ damage and minimize the adverse effects of the disease itself on pregnancy outcomes. Each patient requires individual assessment to control disease activity while minimizing or avoiding medications with potential maternal or fetal toxicity. An open discussion with shared decision making between patients, obstetricians, rheumatologists, and pharmacists is imperative to create an individualized treatment plan that meets patients' goals. This article will review the current literature for use of disease modifying antirheumatic drugs and biologics during pregnancy and lactation, providing health care professionals with the most up-to-date information available.

Educational needs of patients with rheumatic and musculoskeletal diseases attending a large health facility in Austria.

Patient education is an important part of the management of rheumatic and musculoskeletal diseases. Given that patients with diverse diseases do not have the same needs, it is crucial to assess the educational requirements of targeted groups to provide tailored educational interventions. The aim of our study was to assess educational needs of a large cohort of patients with different rheumatic and musculoskeletal diseases attending a health facility in Austria. We assessed educational needs, via an online survey of patients with fibromyalgia (FMS), rheumatoid arthritis (RA), and ankylosing spondylitis (AS) recruited from an Austrian health-care facility, using the Austrian version of the Educational Needs Assessment Tool (OENAT). For our sample of 603 patients, AS (62%), RA (15%), and FMS (24%), there were no educational need differences for the domains of movements, disease process, and self-help measures. Patients with FMS had less need for pain management education and greater need for education about feelings, than other disease groups. Patients with RA had a greater need for education related to treatments than other groups, and patients with AS had a greater need for treatment education than patients with FMS. Patients with AS reported greater need for support system education than other patient groups. Educational needs vary by disease groups, suggesting that health-care professionals should assess disease-specific needs for education to provide optimal assistance in disease management for patients.

Acceptance and commitment therapy for people with rheumatic disease: Existing evidence and future directions.

Despite advances in the management of rheumatic diseases, many patients experience persistent pain and fatigue. Psychological interventions are useful adjunctive treatments, but improvements tend to be small, and progress in this area is needed. The aim of this review was to evaluate the effectiveness of acceptance and commitment therapy (ACT) for the treatment of chronic pain and fatigue in patients with rheumatic disease. A systematic literature search was conducted in MEDLINE, EMBASE, and PsycINFO for randomised controlled trials (RCTs) of ACT for the treatment of chronic pain and fatigue in patients with rheumatic disease. Ten RCTs that included people with rheumatic disease were identified, rated for risk of bias, and summarised in terms of effects on relevant outcomes (disability, emotional functioning, pain, and quality of life). Treatment with ACT was associated with improvements in physical and emotional functioning, pain, and quality of life in chronic pain patients. The favourable effects of ACT were evident when compared with those of treatment as usual or active control conditions (e.g., pain education and novel medication). There was consistent evidence to show that ACT is beneficial in improving physical and emotional functioning in patients with fibromyalgia but a lack of evidence pertaining to patients with other forms of rheumatic disease. ACT provides benefit for fibromyalgia patients. Further high-quality research is needed to develop ACT interventions for people with inflammatory rheumatic diseases and to evaluate their effectiveness for managing the pain and fatigue associated with these conditions.

Interstitial lung disease in rheumatic diseases - newtherapeuticapproaches

Pulmonary involvement is an important aspect in the management of rheumatic diseases. Connective tissue disease-associated interstitial lung diseases (CTD-ILD) are of particular importance, as their occurrence is often decisive for patient outcome. We use corticosteroids, immunosuppressants and eventually also biologicals in the therapy of CTD-ILD.Except for the "Scleroderma Lung Study" (SLS) I and II, which confirm the effectiveness of the immunosuppressive drugs cyclophosphamide (CYC) and mycophenolate mofetil (MMF) in pulmonary involvement of scleroderma (SSc-ILD), there is little data on the treatment of other CTD-ILD. Within the group of biologicals the use of Rituximab (RTX) increases in importance. The currently expected study results compare the efficacy of immunosuppressive drugs (in particular MMF and CYC) with RTX. Other investigated biologicals include Tocilizumab in SSc-ILD and Abatacept in pulmonary involvement in rheumatoid arthritis (RA-ILD). Autologous stem cell transplantation is a potent but potentially risky therapy for severe scleroderma.For the group of pulmonary interstitial diseases of different dignity, including CTD-ILD, with a increasingly fibrosing course despite adequate therapy, (e. g. chronic hypersensitivity pneumonitis, sarcoidosis) the term and concept of "fibrosing interstitial lung diseases with progressive phenotype" (PF-ILD) is being established. In the last months studies were published, which show a positive effect of antifibrotic drugs (Nintedanib, Pirfenidon) in such constellations. Currently a number of other studies regarding the effectiveness of antifibrotics in CTD-ILD are expected to be published. The studies in this field bring new aspects in the understanding of interstitial diseases and have the potential of expanding treatment options in CTD-ILD.The topic of CTD-ILD is a difficult, but at the same time exciting field.

P13 An analysis of multidisciplinary care in systemic auto-immune rheumatic disease in a single centre

Abstract Background Auto-immune rheumatic diseases are multisystem conditions with high morbidity and mortality, where co-ordinated multidisciplinary (MDT) care is necessary to ensure effective and timely treatment. An NHS England Commissioning for Quality and Innovation was implemented in 2016 to support development of MDTs to achieve earlier diagnosis and intervention and standardise quality of care and access to high-cost drugs. Methods A regional network for MDTs was established in one centre, led by Rheumatology with input from other specialities. MDT outcomes are recorded in a bespoke database. We analysed MDT outcomes over a two-year period from September 2017 to August 2019 for the following: disease activity assessments, investigations requested, referrals to other specialists, treatment changes and the reasons for these. Results During this period, 2,750 MDT discussions were recorded for 1,270 patients. The most common diagnoses were giant cell arteritis (30%), systemic lupus erythematosus (9%) and granulomatosis with polyangiitis (6%). Disease activity scores were recorded on 2,744 (99.8%) occasions with BVAS (n = 1,658, mean 0.82, range 0-21) and SLEDAI (n = 379, mean 2.04, range 0-20) used most. This underlines that decisions were consistently based on validated outcome measures. Investigations were ordered on 1165 (42%) occasions. Imaging was requested in 1,124 cases, biopsy in 37, functional tests in 221 and other tests in 18. Ultrasound was requested most often (614). Onward referrals were made in 237 (9%), most frequently to respiratory (32) and ENT (32). On 1550 occasions (56%) a treatment change was made: 1049 (38%) had a new change and 501 (18%) had a previously planned change. The most common reasons were good response to therapy (354 [23%]), inefficacy (345 [22%]) or starting additional therapy (214 [14%]). This shows that a significant number of treatment changes were approved by the MDT, both for treatment reduction as well as escalation. A biologic was added on 65 occasions (4% of treatment changes), most commonly in GCA (8 started on tocilizumab), SLE (5) and GPA (5). Biologics were repeated in 121 (8%) and stopped in 25 (2%). Where biologics were added, mean pre-treatment BVAS was 4.7 and post-treatment was 0.6. Mean reduction in BVAS after treatment was 5.2. The equivalent mean scores for SLEDAI were 7 (pre-treatment), 2 (post-treatment) and 7.7 (reduction). On average, the addition of biologics resulted in reductions in disease activity scores, which provides justification for their use. Conclusion Our data suggest that the large number of MDT reviews over this two-year period contributed significantly to management of systemic auto-immune rheumatic diseases by approving investigations, referrals and treatment changes. This facilitated timely intervention and avoidance of overtreatment. The auditable recording of these reviews and disease activity scores ensured decisions were guided by objective assessments and the use of high-cost drugs was standardised and justifiable. Disclosures K. Sandhu None. N. Ahmad None. A. Verdiyeva None. R. Luqmani None.

Перигравидарный менеджмент ревматических заболеваний

Management of inflammatory rheumatic diseases in preconception period, pregnancy and breastfeeding has undergone significant changes over the past few years. Modern therapy, including biological and targeted synthetic disease modifying drugs, has significantly improved the control of rheumatic diseases, which has led to an increase in the patients’ number planning a pregnancy with serious diseases. When consulting such patients, it is necessary to discuss the possible time of conception (regarding the activity of the disease), the effect of the disease on pregnancy and pregnancy on the disease, as well as the potential need to change the regimen of medications during pregnancy and breastfeeding. This review summarizes information on the effects of pregnancy on various rheumatic diseases and vice versa, changes in therapy and monitoring of patients with rheumatic diseases before, during and after pregnancy. Women with inflammatory rheumatic diseases need advice on drug therapy before planning pregnancy, during pregnancy, and breastfeeding. Safe disease-modifying drugs that can be taken during pregnancy are hydroxychloroquine, sulfosalazine, azathioprine, and cyclosporine. Glucocorticoids and non-steroidal anti-inflammatory drugs can also be taken up to 32 weeks of gestation. Most inhibitors of tumor necrosis factor (anti-TNF) are also safe during pregnancy. During pregnancy, a clear monitoring of the activity of the disease is necessary, control of the level of autoantibodies, especially anti-SSA / Ro and anti-SSB / La and antiphospholipid antibodies, an assessment of the degree of organ dysfunction, especially kidney damage. Presented are modern approaches to optimizing the management of inflammatory rheumatic disease during pregnancy. For patients with inflammatory rheumatic diseases, a successful pregnancy outcome is optimized by creating an individual plan to suppress disease activity using a targeted approach. Key words: pregnancy, rheumatic diseases, treatment, lactation, management tactics, drugs.

ROMATİZMAL HASTALIKLARDA KAPLICA TEDAVİSİ

Kaplıca tedavisi birçok hastalığın tedavisinde olduğu gibi kronik romatizmal hastalıkların yönetiminde de önemli bir rol oynamaktadır. Etki mekanizması kimyasal, termal, mekanik ve immünmodülatör olmak üzere geniş bir spektrum içermektedir. Son yıllarda balneoterapinin tek başına veya diğer geleneksel tıbbi tedavilere tamamlayıcı olarak önemi giderek artmaktadır. Çeşitli araştırmalardan elde edilen sonuçlar balneoterapinin faydalı etkilerini ortaya koymaktadır. Bu çalışmada, laboratuar parametreleri ve klinik açıdan yapılan çalışmalar gözden geçirilerek, romatizmal hastalıklarda kaplıca tedavisinin etkisi güncel bilgiler ışığında sunulmuştur.

The Role of Cardiovascular Magnetic Resonance in Inflammatory Arthropathies and Systemic Rheumatic Diseases

In this review, the role of cardiovascular magnetic resonance (CMR), magnetic resonance angiography (MRA) and magnetic resonance spectroscopy (MRS) in the diagnosis, risk stratification, management and prognostication of inflammatory arthropathies and systemic rheumatic diseases (IASRD) is discussed. There have been several recent publications and it is timely to place into perspective the rapidly evolving concepts of advanced imaging in IASRDs through review and critical appraisal of the literature. IASRD are multisystemic autoimmune disorders with predilection for cardiovascular involvement and complications. Persons with IASRD have increased risk of coronary artery disease, stroke, peripheral vascular disease, myocarditis, cardiomyopathy and heart failure that may not be fully explained by traditional CVD risk factors, and inflammation is presumed to be an important determinant of CVD. There is increasing evidence that lowering systemic inflammation reduces prevalence of CVD in IASRD patients. CMR is an advanced imaging modality, free of ionising radiation, with high spatial and temporal resolution, performed via excitation of atomic nuclei with uneven spin and capable of providing detailed anatomical, functional and haemodynamic information on the heart and vasculature. CMR is the single imaging modality capable of assessing noninvasively global and regional cardiac function, fibrosis, perfusion, flow, vascular function and oedema. The unique strengths of CMR are its ability to noninvasively image the entire cardiovascular tree (myocardium and vessels) with high resolution and to reveal tissue characteristics for the study of inflammation, infarction, fibrosis and infiltration. CMR combined with MRA is capable of visualising vascular complications of IASRD as well as to track disease progression or regression following therapy. MRS reported no abnormalities in IASRD on proton MRS, but impaired myocardial energetics on phosphorous MRS. CMR, MRA and MRS have significantly improved our understanding of disease mechanisms, diagnosis, risk stratification, management and prognostication of IASRD. Further CMR can be used for the study of inflammation and fibrosis and to track disease progression and regression following therapeutic interventions.

Reflections on 'older' drugs: learning new lessons in rheumatology.

The management of rheumatic and musculoskeletal diseases has transformed over two decades of exciting discoveries regarding pathogenesis and innovative drug development. The introduction of sophisticated immunomodulatory therapies has given renewed hope to many patients, but notable challenges remain within the field of rheumatology. Before the advent of biologic therapies, conventional synthetic DMARDs (csDMARDs) provided effective disease control for some patients, although a comprehensive understanding of the mechanisms by which these drugs exert their effects remained elusive. Reflecting upon the efficacy and mechanisms of action of csDMARDs can provide intriguing insights. First, contemplating past approaches brings our remarkable current approaches concerning pathogenesis-driven discovery and drug development into sharper focus. Second, increased understanding of the mode of action of these older drugs might in turn provide exciting opportunities for the understanding of disease and for the development of future therapies.

Challenges in the Management of Rheumatic Valvular Heart Disease in the West African Sub-Region

Challenges in the Management of Rheumatic Valvular Heart Disease in the West African Sub-Region

Rheumatology practice and training in India – A perspective from rheumatology consultants

Background: We surveyed rheumatology consultants regarding their perceptions about rheumatology practice and training in India. Materials and Methods: A structured questionnaire was administered during two national rheumatology meetings to evaluate practice settings and demographic characteristics of patients seen. We also assessed perceptions regarding junior doctors, auxiliary health professionals (AHPs), ancillary facilities (daycare, intra-articular injections, musculoskeletal ultrasound [MSKUS]), and possible government-driven strategies for improving rheumatology care delivery. Results: The response rate was 56% (70/125); 68 complete responses were further analyzed. Most respondents were in the private sector (56/68), had been in practice for a mean of 11.5 (±8.4) years, attended to a mean of 150 (±91) patients every week, a majority of whom were educated upto intermediate level and belonged to the middle class. About 60% had junior doctors to assist them in care delivery; 82.5% (52/63) felt that junior doctors had limited competence in managing rheumatic diseases. More than 90% felt the need for AHPs in the clinic, although less than one-half had access to such personnel. Most utilized ancillary services like intra-articular injections (97%), daycare facilities for infusions (92%), and MSKUS (71%). More than 90% respondents felt that government-funded programs for the management of rheumatic diseases, coupled with a structured referral system, would be useful. Conclusion: Rheumatology practice in India may improve by better training junior doctors in skills related to rheumatology at the undergraduate and postgraduate internal medicine levels. The development of specialist AHPs may help enhance service delivery. Government programs for community management of rheumatic diseases and management guidelines suited to the needs of a developing economy majorly reliant on out-of-pocket expenditure for healthcare are significant areas for development.

Attitude of Iraqi Rheumatologists While Managing Rheumatic Diseases in the Era of COVID-19: An Online Survey.

Background:The current crisis COVID-19 has affected the already challenging management of rheumatic diseases. Since no evidence-based approach is yet available, this survey was conducted to explore the Iraqi rheumatologists’ attitude in the era of COVID-19 and how they have been managing their patients, especially with the continuously updating and sometimes conflicting reports.Patients and methods:A cross-sectional survey was distributed online via telephone and social media to rheumatologists in Iraq. A questionnaire was conducted about methods of patient education, preventive measures, and methods of arranging visits and treatment. In addition, an inquiry about the similarity of the practice between their local institutions and global ones.Results:144 rheumatologists answered the 14 obligatory questions, of which the majority were specialists. 122 rheumatologists participated in patient education. Half of participants used online information, social media and websites as a source to communicate and interact with their patients for education about COVID-19-related issues.Conclusion:Despite the lack of solid guidelines regarding the management of rheumatic diseases during the COVID-19 crisis, this survey showed the majority of Iraqi rheumatologists to be familiar with the updating recommendations. Also, as the majority are waiting for stronger evidence before attempting to embrace controversial issues, surely this reflects a responsible and scientific attitude.

Underuse of Injectable Penicillin in Secondary Prophylaxis of Rheumatic Heart Disease in Nepal

Background and Aims: Secondary prophylaxis has remained the mainstay of rheumatic fever and rheumatic heart disease management. Despite the proven ef􀏐icacy and superiority of injectable penicillin in rheumatic heart disease patients, it has been underused in Nepal. Materials and Methods: This is a hospital based cross-sectional study during June 2014 to October 2018 over a period of 52 months at College of Medical Sciences-Bharatpur including 350 patients with clinical and/or echocardiographic evidence of de􀏐inite rheumatic heart disease. Data was collected from both cardiology outpatients and inpatients (admitted in cardioward/coronary care unit). Relevant data and information were entered into the pre-structured proforma and then analyzed by SPSS-16 software. Results: The age of the patients ranged from 6 to 80 years with mean age 36.76±4.6years with female preponderance (F:M=1.26:1). The predominantly involved isolated valve was mitral in 152 patients (44.43%) followed by aortic valve in 70 patients (20.00%) and rest 90 (25.71%) had dual valvular involvement. The common complications encountered were heart failure in 200(57.14%) and arrhythmias in 155(44.29%) patients. Two hundred ten (60.00%) of the patients received penicillin (oral and injectable) and erythromycin. Majority 180/210=85.71%) were prescribed on oral penicillin whereas only 46/210=21.90% received injectable penicillin; the ratio being 3.35:1. Conclusion: RHD is a leading cause of heart failure and death among young population. There is underuse of penicillin with very minimal focus on use of injectable penicillins currently. Hence, Nepal government and other non-governmental organizations should consider implementation of use of penicillin broadly and moreover focus on use and adherence of injectable penicillin. Keywords: Rheumatic Heart Disease, Penicillin, Underuse, Secondary Prophylaxis.

Detection of Anti-Extractable Nuclear Antigens in Patients with Systemic Rheumatic Disease via Fluorescence Enzyme Immunoassay and Its Clinical Utility.

PurposeTesting for autoantibodies to extractable nuclear antigens (ENAs) plays an important role in the diagnosis and management of systemic rheumatic disease. Currently, no gold standard tests are available for detecting anti-ENAs. To address this gap, we aimed to identify an assay that exhibits satisfactory diagnostic performance in the detection of five common anti-ENAs by comparing two commonly used assays, an automated fluorescent enzyme immunoassay (FEIA) and a microplate ELISA assay.Materials and MethodsSera from 100 patients with systemic rheumatic disease were collected and assayed with FEIA and microplate ELISA to detect anti-ENAs. Statistical analyses were performed to check the agreement rate between the two platforms using kappa coefficients. Analytical sensitivity and specificity for each assay were calculated.ResultsThe concordance rates between ELISA and FEIA ranged from 89% for anti-RNP to 97% for anti-Scl-70, and the kappa coefficients of the two assays were in the range of 0.44 to 0.82. Between the two assays, a significant difference in sensitivity and specificity was seen only for anti-Sm and anti-RNP, respectively.ConclusionIn this study, FEIA and ELISA showed comparable efficiency for detecting anti-ENAs.

Management of Rheumatic Heart Diseases in Burundi

Background: The surgical management of rheumatic heart disease (RHD) in Burundi is almost impossible because of the absence of the technical plateau. Aim: To describe the therapeutic and evolutionary aspects of RHD in Burundi. Patients and methods: This is a retrospective and analytical study of patients' records in surgical indication and/or operated for RHD by facilitation of “Maison du Bon Samaritain du Burundi (MBSB)” from February 2015 to February 2017. Results: Among 89 patients consulting for RHD, 45 (50.56%) were operated. The average age of the patients was 25 years. Females predominated with 64.04% of cases. The age of most of patients was between 4 and 40 years with 74 cases (83.14%). All patients had been received at the heart failure stage including 83 (93.25%) at stage III or IV according to the New York Heart Association (NYHA) classification. The ejection fraction was ≤ 50% in 61 patients (68.53%). Pulmonary arterial hypertension was recorded in 79 patients (88.76%). On chest X-ray, cardiomegaly was found in 85 patients (95.50%). Electrical abnormalities were dominated by dilatation of the left atrium in 48 cases (53.93%). Isolated valvular lesions were the most numerous with 67 cases (75.28%). The operative management was mainly valvular replacement (93.47%) with an operative success of 100% six months after the intervention. Conclusion: The diagnosis of RHD was late. Young people were more vulnerable. The postoperative prognosis at six months was good.

Adverse Cutaneous Reactions of Common Biologic Medications for Rheumatic Diseases

Within the last two decades, the application of biologic medications for rheumatic diseases has greatly expanded. Therefore, it is important for clinicians to be knowledgeable about potential adverse reactions of this medication class. The goal of this review is to discuss the current understanding of cutaneous adverse reactions that may occur with common biologic medications utilized in the management of rheumatic diseases. A variety of cutaneous reactions have been reported in association with biologics commonly used to treat rheumatic diseases. Here, we review cutaneous reactions reported with tumor necrosis factor-α inhibitors, abatacept (co-stimulation blockade), CD20 inhibitors, IL-17 inhibitors, IL-12/23 inhibitors, and IL-6 inhibitors. The side effects, in most cases, can be managed without discontinuation of the associated biologic medication. However, there are identified reactions that warrant discontinuation or adjustments in order to prevent additional and possibly permanent patient morbidity. While causation is often difficult to prove when cutaneous side effects are observed in relation to biologic therapies, it is important for clinicians to be aware of characteristic or distinguishing cutaneous reactions in order to treat patients with rheumatic diseases safely and effectively.

Medications in pregnancy and breastfeeding

Advances in therapeutics, including a wider use of biologics and targeted synthetic disease-modifying anti-rheumatic drugs (DMARDs) have substantially improved the management of rheumatic diseases, resulting in more women with severe disease considering pregnancy. Every clinical rheumatologist has encountered a woman who wishes to have a pregnancy - or who presents already pregnant - and who values the importance of reliable information on rheumatic diseases and safety of medications in pregnancy. This chapter summarises current evidence and knowledge on the use of 'Medications in pregnancy and breastfeeding in women with rheumatic diseases' and considers paternal medication use at the time of conception.

Paternal safety of anti-rheumatic medications.

There is lack of research on the safety of medications used to treat rheumatic diseases in men wishing to conceive. When evaluating medication safety for potential fathers, two major reproductive issues are to be considered: first, whether a drug induces infertility, and second, whether a drug can cause adverse pregnancy outcomes. Cyclophosphamide is the only medication used in rheumatic disease management that causes irreversible infertility. All men prescribed cyclophosphamide should be counseled on fertility preservation including sperm banking. Sulfasalazine can cause reversible azoospermia; when conception is delayed, this medication should be held for three months and semen analysis should be performed. There are limited data on the teratogenic risk of paternal exposure to medications. Men wanting to conceive should avoid cyclophosphamide and thalidomide. Methotrexate; NSAIDs; glucocorticoids; sulfasalazine; the immunosuppressive agents azathioprine, 6-mercaptopurine, cyclosporine, tacrolimus, and mycophenolate mofetil; colchicine; TNF-alpha blockers; hydroxychloroquine; IVIG; rituximab; abatacept; and anakinra are compatible with paternal exposure. There are insufficient data to conclude the safety of other biologics and small molecules in men seeking to father a child.