Rheumatic Involvement Research Articles

Degenerative mitral stenosis by echocardiography: presentation and outcome

Abstract Aims Degenerative mitral stenosis (DMS) is due to degenerative mitral annular calcification (MAC) and valvular calcification. However, DMS impacts on the outcome, and therefore, potential treatment needs are poorly known. We aimed at evaluating survival after DMS diagnosis by Doppler echocardiography in routine practice. Methods and results A cohort of 2937 (75 ± 12 years, 67% women) consecutive patients were diagnosed between 2003 and 2014 with DMS (diastolic mean gradient ≥ 5 mmHg), with analysis of short- and long-term survival. All patients had overt mitral annular/valvular degenerative calcification without rheumatic involvement. Mean gradient was 6.5 ± 2.4 mmHg, and DMS was considered mild in 50%, moderate in 44%, and severe in 6%. DMS was associated with left atrial enlargement (52 ± 23 mL/m2) and elevated pulmonary pressure (49 ± 16 mmHg) despite generally normal ejection fraction (61 ± 13%). DMS was associated with frequent comorbid conditions (74% hypertension, 58% coronary disease, and 52% heart failure) and humoural alterations (haemoglobin 11.3 ± 1.8 g/dL and creatinine 1.5 ± 1.4 mg/dL). One-year mortality was 22%, most strongly related to older age, higher comorbidity, and abnormal haemoglobin/creatinine but only weakly to DMS severity (with anaemia 42% irrespective of DMS severity, P = 0.99; without anaemia 18, 23, and 28% with mild, moderate, and severe DMS, respectively, P < 0.0004). Long-term mortality was high (56% at 5 years) also mostly linked to aging and weakly to DMS severity [with anaemia P = 0.90; without anaemia: adjusted-hazard ratio: 1.30 (1.19–1.42), P < 0.0001, for moderate vs. mild DMS and 1.63 (1.34–1.98), P < 0.0001, for severe vs. mild DMS]. Conclusion DMS is a condition of the elderly potentially resulting in severe mitral obstruction and haemodynamic alterations. However, DMS is frequently associated with severe comorbidities imparting considerable mortality following diagnosis, whereas DMS severity is a weak (albeit independent) determinant of mortality. Hence, patients with DMS should be carefully evaluated and interventional/surgical treatment prudently considered in those with limited comorbidity burden, particularly without anaemia. Keywords: Degenerative Mitral Stenosis; Outcome; Natural history; Ecocardiography; Mitral Stenosis.

SEVERE MITRAL INSUFFICIENCY FROM RHEUMATIC DISEASE: A CASE REPORT

Abstract Acute rheumatic fever continues to be a major health problem in many parts of thw world. It’s more common in low–income or developing parts of the world. Antibiotics for bacterial infections may not be available in these areas. In developed countries, cardiac involvement is quite rare. Although traditionally considered to be a disease associated with poverty, rheumatic fever continues to persist even among the middle–class population in developed countries. Children and teenagers with intreated strep infections are the most likely to get rheumatic fever. Signs of heart damage can develop years after the infection. Rheumatic heart disease is the result of valvular damage caused by an abnormal immune response to Streptococcus pyogenes infection. After multiple episodes of rheumatic fever, progressive fibrosis of the heart valves can occur: mitral regurgitation is the most common valvular lesion. We report the case of a 13–year–old, Italian, middle–class girl with rheumatic mitral valve involvement. A 13–years–old comes to the clinic for onset of worsening dyspnea ad easy fatigability. History turns out to be negative for cardiovascular disease and history of recent infections. Echocardiographic examination showed a dilated left ventricle (VTD 148ml, 98 m/mq) with preserved chinese. Severe dilatation of the left atrium with atrio–aortic ratio 2.3 and atrial volume 91 ml/mq. The mitral valve presented a myxomatous, thickened leaflets, predominantly the posterior which appeared retracted and hypomobile with thickening of the subvalvular apparatus with coaptation deficit along the entire coaptation rhyme realizing severe degree insufficiency (CV 7mm, ERO 0.8 cm2, CO 39 l/min). A small cleft at the level of A1, main lesion jet (A1–P3–P2) was also visible. The Echocardiographic appearance suggested outcomes from rheumatic disease so antistreptolysin titer and anti–DNase B antibodies were assayed with high levels found. Penicillin prophylaxis was therefore initiated. For the continuation of the therapeutic course, the case was discussed collegially and a valve replacement rather tan repair was advocated because the valvular and subvalvular apparatus were far too damaged.

Does rheumatic fever-induced myocardial involvement improve? Three-dimensional echocardiography and the answer to this question.

Does rheumatic fever-induced myocardial involvement improve? Three-dimensional echocardiography and the answer to this question.

How Do We Identify and Manage Progressive CTD-ILD?-Update in Treatment and Guideline

The lung is a frequent target of autoimmune-mediated injury in patients with rheumatic diseases. Not infrequently, respiratory involvement may be the presenting manifestation of connective tissue diseases (CTDs). The prevalence of interstitial lung disease (ILD) in rheumatoid arthritis is about 20 to 30% of patients, while ILD may occur in 20 to 50% of patients with idiopathic inflammatory myositis, 40-50% or more of patients with systemic sclerosis, 2 to 5% of patients with systemic lupus erythematosus, 20 to 60% of patients with mixed connective tissue disease, and up to 25% of patients with Sjögren’s syndrome. Rheumatic disease-associated lung involvement is a major cause of morbidity and mortality in these patients. It shows a considerable heterogeneity in incidence and prevalence, clinical course, and the involved lung structure depending on the underlying rheumatic disease. Each rheumatic disease is associated with a characteristic pattern of their lung disease. The standard therapy has traditionally been immunosuppressant. With the evolvement of treatment, anti-fibrotic is now indicated for chronic fibrosing ILDs with a progressive phenotype, including autoimmune disease-associated ILD. By targeting the underlying mechanism of pulmonary fibrosis, it is shown that the annual rate of FVC decline can be reduced by more than 50%. In this lecture, Prof. Oliver Distler and Prof. Toby Maher will review the importance of timely treatment, the management practices and treatment guidelines for CTD-ILD.

VEXAS syndrome: Current clinical, diagnostic and treatment approaches.

VEXAS syndrome, is a hemato-inflammatory chronic disease characterized with predominantly rheumatic and hematologic systemic involvement. It was first described in 2020 by a group of researchers in the United States. VEXAS syndrome is a rare condition that primarily affects adult males and is caused by a mutation in the UBA1 gene located on the X chromosome. Its pathogenesis is related to the somatic mutation affecting methionine-41 (p.Met41) in UBA1, the major E1 enzyme that initiates ubiquitylation. Mutant gene lead to decreased ubiquitination and activated innate immune pathways and systemic inflammation occur. The specific mechanism by which the UBA1 mutation leads to the clinical features of VEXAS syndrome is not yet fully understood. VEXAS is a newly define adult-onset inflammatory syndrome manifested with treatment-refractory fevers, arthritis, chondritis, vasculitis, cytopenias, typical vacuoles in hematopetic precursor cells, neutrophilic cutaneous and pulmonary inflammation. Diagnosing VEXAS syndrome can be challenging due to its rarity and the overlap of symptoms with other inflammatory conditions. Genetic testing to identify the UBA1 gene mutation is essential for definitive diagnosis. Currently, there is no known cure for VEXAS syndrome, and treatment mainly focuses on managing the symptoms. This may involve the use of anti-inflammatory medications, immunosuppressive drugs, and supportive therapies tailored to the individual patient's needs. Due to the recent discovery of VEXAS syndrome, ongoing research is being conducted to better understand its pathogenesis, clinical features, and potential treatment options. In this review article, the clinical, diagnostic and treatment approaches of VEXAS syndrome were evaluated in the light of the latest literature data.

Cardiovascular accident unveiling rheumatic heart disease

Infection with group A Streptococci causes an autoimmune reaction known as acute rheumatic fever (ARF) which leads to chronic cardiac condition called rheumatic heart disease (RHD). Rheumatic involvement of the cardiac valves is one of the sequelae of ARF. The valvular lesions begin as verrucae along the borders of heart valves and tend to resolve leaving a scar once the inflammation subsides. Cardio embolism is seen as the main pathophysiological mechanism of ischemic stroke in RHD.

Long-term results of the Ross operation in rheumatic valve disease.

The aim of this study was to evaluate the impact of rheumatic heart disease (RHD) on the long-term outcomes of the Ross operation when compared to patients with bicuspid aortic valves (BAV). We analysed patients with rheumatic disease and BAV who underwent a Ross operation between 1995 and 2020. The primary outcomes were late survival and reoperations on the pulmonary autograft and the right ventricular allograft. We also performed a propensity score analysis to adjust for differences in baseline characteristics. The cumulative incidence of the events in the matched cohorts was compared using the Fine-Gray model. There were 147 patients with rheumatic disease and 314 with bicuspid valves. In the unmatched population, patients with rheumatic disease were younger, more symptomatic, had worse left ventricular function and were more likely to present a preoperative diagnosis of aortic insufficiency. Propensity matching resulted in 86 pairs with similar baseline characteristics. Compared with those with bicuspid valves, rheumatic patients had similar 20-year survival both in the unmatched (RHD = 67.8% vs BAV = 79.7%, P = 0.27] and matched cohorts (RHD = 72.5% vs BAV = 82.9%, P = 0.46). Cumulative incidence of reoperations on the pulmonary autograft after 20 years was also comparable between them in the unmatched (RHD 13.4% vs BAV 14.4%, P = 0.67) and matched patients (RHD 11.6% vs BAV 21.1%, P = 0.47). Pulmonary autograft dysfunction due to rheumatic involvement was detected in only three occasions. Cumulative incidence of reoperations on the right-sided allograft was higher in the unmatched cohort (RHD = 5.5% vs BAV = 16.7%, P = 0.035) but became nonsignificant after propensity score matching (RHD = 9.9% vs BAV = 32.3%, P = 0.051). The cumulative incidence of all Ross-related reoperations was similar in both groups. Among patients undergoing Ross operation, patients with RHD had similar long-term survival and rates of reoperations, when compared to patients with BAV.

Rheumatic Heart Disease with Quadrivalvular Stenosis: A Case Report and Review of Literature

Multivalvular heart disease (MVD), particularly of rheumatic nature, continues to be a widespread condition in developing countries such as Morocco, despite implemented preventive measures. The intricate hemodynamic interactions between coexisting valve lesions may influence the clinical expression of each individual lesion. It makes diagnosis difficult and management often at the discretion of the physician given the lack of specific guidelines to assist in strategy. In the available literature, rheumatic quadrivalvular stenosis is exceptional. Here we describe a very rare case of rheumatic involvement of all four heart valves discovered in a 34-year-old woman with a history of untreated recurrent tonsillitis and joint pain in childhood, who presented with progressively worsening dyspnea and fatigue. Transthoracic echocardiography revealed severe left valves stenosis in addition to severe tricuspid and mild pulmonary stenosis, with evidence of congestive heart failure. Our case highlights the diagnostic difficulties encountered in the context of a developing country with limited access to primary care and treatment options.

A novel approach of tricuspid valve repair: mitralization of tricuspid valve

OBJECTIVESThis study presents the mid-term results of a novel tricuspid valve (TV) repair strategy defined as ‘mitralization of TV’ (resection and plication of the posterior leaflet, ring implantation, optional leaflet procedures) applied for the correction of tricuspid regurgitation (TR).METHODSBetween 2017 and 2020, a total of 22 patients underwent concomitant TV repair using mitralization of the TV. Fourteen of the patients had functional TR (2 of them had severe tethering), 5 patients had prolapse and 3 patients had rheumatic involvement.RESULTSThere was no in-hospital mortality. Moderate or severe TR was not observed in any patient in echocardiographic evaluations before discharge. The mean follow-up duration was 30.9 + 6.2 months. Moderate-to-severe TR-free survival was 100% in the second year and 94.7% in the third year.CONCLUSIONSMitralization of the TV is a safe and effective treatment modality in terms of its mid-term results. This new technique provides an innovative perspective for the treatment of TR, especially in complex TV pathologies.

Cytokine Release Syndrome in Cancer Patients Receiving Immune Checkpoint Inhibitors: A Case Series of 25 Patients and Review of the Literature.

Cytokine release syndrome (CRS) is a phenomenon of immune hyperactivation described in the setting of immunotherapy. Unlike other immune-related adverse events, CRS triggered by immune checkpoint inhibitors (ICIs) is not well described. The clinical characteristics and course of 25 patients with ICI-induced CRS from 2 tertiary hospitals were abstracted retrospectively from the medical records and analyzed. CRS events were confirmed by 2 independent reviewers and graded using the Lee et al. scale. The median duration of CRS was 15.0 days (Q1; Q3 6.3; 29.8) and 10 (40.0%) had multiple episodes of CRS flares. Comparing the clinical factors and biomarkers in Grades 1-2 and 3-5 CRS, we found that patients with Grades 3-5 CRS had following: (i) had longer time to fever onset [25.0 days (Q1; Q3 13.0; 136.5) vs. 3.0 days (Q1; Q3 0.0; 18.0), p=0.027]; (ii) more cardiovascular (p=0.002), neurologic (p=0.001), pulmonary (p=0.044) and rheumatic (p=0.037) involvement; (iii) lower platelet count (p=0.041) and higher urea (p=0.041) at presentation compared to patients with Grades 1-2 CRS. 7 patients (28.0%) with Grades 1-2 CRS were rechallenged using ICIs without event. 9 patients (36.0%) were treated with pulse methylprednisolone and 6 patients (24.0%) were treated with tocilizumab. Despite this, 3 patients (50%) who received tocilizumab had fatal (Grade 5) outcomes from ICI-induced CRS. Longer time to fever onset, lower platelet count and higher urea at presentation were associated with Grade 3-5 CRS. These parameters may be used to predict which patients are likely to develop severe CRS.

The Diagnosis and Treatment of Rheumatoid and Juvenile Idiopathic Arthritis of the Temporomandibular Joint.

Involvement of the temporomandibular joint can be shown in 40-90% of patients with rheumatoid arthritis and juvenile idiopathic arthritis (JIA), although it is often asymptomatic. Restricted jaw mobility and jaw pain can be found in approximately 20% of patients with JIA (prevalence: 70 per 100 000 persons). Early diagnosis and treatment of the underlying disease are essential for a good outcome, but uniform, consensus-based management is still lacking. The clinical practice guideline is based on the findings of a systematic literature review in multiple databases and a Delphi procedure to obtain consensus on the recommendations. Most of the identified studies were retrospective. Patients with JIA should undergo clinical screening with a structured examination protocol once per year in childhood and adolescence, and thereafter as well if the temporomandibular joint is involved. The diagnosis of chronic rheumatoid arthritis of the temporomandibular joint is established with contrast-enhanced magnetic resonance imaging. Conservative treatment (antirheumatic basal therapy, local measures) is unsuccessful in less than 10% of patients. In such cases, arthroscopy and arthrocentesis can be used for temporary symptom relief and functional improvement. Intra-articular corticosteroid injections should be given only once, and only in otherwise intractable cases. In severe cases where all other options have been exhausted (<1%), open surgical treatment can be considered, including alloplastic joint replacement. Oligosymptomatic and asymptomatic cases are common even with radiologic evidence of marked joint damage. The possibility of rheumatic involvement of the temporomandibular joint must be kept in mind so that serious complications can be avoided. Regular clinical evaluation of the temporomandibular joint is recommended, particularly for patients with juvenile idiopathic arthritis.

Effect of Percutaneous Transvenous Mitral Commissurotomy in Severe Mitral Stenosis: A Cross Sectional Study

Introduction: The inflow obstruction from the left atrium to the left ventricle due to rheumatic involvement of apparatus is the main cause of Mitral stenosis. The patients with mitral valve involvement are found to have a combination of stenosis and regurgitation. Due to the thromboembolic phenomenon, left atrial dilatation and stasis predispose thrombus formation. So, even after percutaneous treatment, the elevated pressure in MS patients causes morbidity and mortality. Aim: To assess the effect of percutaneous transvenous mitral commissurotomy (PTMC) in reducing pulmonary hypertension in patients with severe mitral stenosis. Methodology: A total of 134 patients were included in the study. All of them had severe mitral stenosis with elevated pressure of more than 55 mmHg. The age range of patients was between 16 to 62 years. These patients were assessed by echocardiography post-PTMC. Age, gender, and other demographic details were also noted. Results: Mean age of patients was 36.44±11.37 years. Hypertension was found in 49 patients, and diabetes in 48 patients. Mean baseline systolic pressure was found to be 73.62+9.10 mmHg and mean artery systolic pressure was 44.09±5.06 mmHg. After the commissurotomy, the pulmonary artery systolic pressure reduced from baseline by 38.90+5.20. In 125 patients the Transvenous mitral commissurotomy was effective and in 9 patients it was ineffective. Conclusion: This study was found to be successful because it had fewer patients with pulmonary hypertension after the completion of treatment. For the reduction in mean pulmonary artery, systolic pressure balloon valvotomy is a useful technique irrespective of age and gender.

Anesthesia management in patient with nonobstructive hypertrophic cardiomyopathy with severe mitral valvular regurgitation with severe pulmonary artery hypertension for mitral valve replacement

Hypertrophic cardiomyopathy (HCM) is a genetic disorder that is characterized by left ventricular hypertrophy unexplained by secondary causes and a nondilated left ventricle with preserved or increased ejection fraction. Mitral regurgitation is common in HCM secondary to distortion of mitral valve apparatus leading to systolic anterior motion. We report a case of HCM with rheumatic involvement of mitral valve with severe pulmonary artery hypertension and atrial fibrillation. With regard to hemodynamic management in patients with postcardiac surgery status, anesthetic management seems to be a “walk a tight rope.”

Incidence &amp; Pattern of Valvular Heart Disease in Patients attended in Echo Lab at a tertiary care Hospital: A single Centre Study

Background: This echocardiographic study was undertaken to establish incidence, pattern &amp; complications in patients of valvular heart disease.&#x0D; Methods: A total of 97 patients were enrolled among the patients attended in echo lab at Ibrahim cardiac hospital &amp; Research Institute over a period of 1 year.&#x0D; Results: Mean age was 53.8±1.5 years. Male were predominant (58.76% vs 41.23%). Most common symptom was shortness of breath (60.8%) followed by palpitation (43.3%). 39.2% of patients had normal ECG &amp; 12.4% revealed AF. The most common complication was pulmonary hypertension (54.6%) followed by pulmonary edema (26.8%), Stroke (10.3%), Left atrial thrombus (7.3%), &amp; infective endocarditis (6.2%). MV was most commonly involved (72.2%) followed by aortic valve (AV) (66.0%), tricuspid valve (TV) (54.6%) &amp; pulmonary valve (PV) was (20.6%). Rheumatic involvement (52.6%) constituted the dominant cause than degenerative &amp; congenital (24.7% in each). Mitral stenosis (MS) was predominant (52.6%) &amp; was rheumatic in origin; then aortic stenosis (AS) (48.5%) which was mostly degenerative followed by bicuspid aortic valve (8.2%). Mitral regurgitation (MR) was the most common valvular lesion (64.9%); then aortic regurgitation (AR) (51.5%) which was also rheumatic in etiology. Multiple valve involvement was also present. The most common variety was MS+MR (43.3%). Tricuspid stenosis (TS) was in association with MR+AR (1.03%). Tricuspid regurgitation (TR) was 58.8%, mostly secondary to rheumatic involvement of other valves. Severe TR (5.2%) was due to Ebstain anomaly and prolapse (4.1% &amp; 3.1%). ventricular systolic &amp; diastolic functions were normal mostly.&#x0D; Conclusion: Among the rheumatic heart disease patients’ mitral valve was the most commonly affected valve. Mitral regurgitation was the most common valvular lesion. Rheumatic involvement remains the dominant cause of valvular heart disease in Bangladesh.&#x0D; Cardiovasc. j. 2021; 13(2): 144-153

Normal values of echocardiographic parameters and incidence of valvular heart findings in 2229 iranian peaple: Results from the “HAMRAH survey”

Introduction: It is crucial to define the normal ranges of echocardiographic parameters and their relationships with age, sex, race, and geographical differences given the failure of the existing literature to reflect the diversity of the world's populations. This study aimed to determine the normal ranges of echocardiographic systolic and diastolic values and the incidence of valvular heart lesions with or without rheumatic involvement in a sample of Iranian population. Methods: The Echocardiography Heart Assessment and Monitoring in Rajaie Hospital study is a population-based investigation conducted in Rajaie Cardiovascular Medical and Research Center. A total of 2229 Iranian individuals between 30 and 75 years of age without clinical cardiovascular diseases were invited through a multistage random sampling process, and they underwent two-dimensional and Doppler echocardiography. The left ventricular (LV) ejection fraction, the interventricular septal thickness, the ascending aortic size, the LV diastolic function, the valvular heart disease (VHD) severity, and rheumatic valvular involvement were assessed. Results: The study population comprised 2229 patients: 882 men (40%) and 1347 women (60%) at an age range of 39–58 years and a mean age of 48 years. The average body mass index was 28.8 kg/m2 (25.9–32 kg/m2), and the median body surface area was 1.86. Mildsystolic dysfunction was reported in 119 patients (5.7%) and moderate systolic dysfunction in 19 (0.9%). LV systolic dysfunction was significantly correlated with the male sex, age, and hypertension (P < 0.001). The mean interventricular septal thickness was 8 mm. Mild LV hypertrophy (LVH) was detected in 5.5% of all the patients and moderate LVH in 0.4%. The mean interventricular septal thickness significantly increased with age (P < 0.001), and it was higher in the men than in the women (8.2 mm [7.8–9.2] vs. 8 mm [7–8.25]; P < 0.001). Mild LVH and moderate LVH were significantly correlated with hypertension, diabetes mellitus, hyperlipidemia, chronic kidney disease, and diastolic dysfunction (P < 0.001). Diastolic dysfunction was found in 29% of the patients (28.6% mild and 0.2% moderate). Significant correlations were found between LV diastolic dysfunction and age, the female sex, diabetes mellitus, hypertension, hyperlipidemia, and hypercholesterolemia (P < 0.001). The mean ascending aortic size was 31 mm in the men and 29 mm in the women. The ascending aortic size exceeded 37 mm in 5% of the men and 3.5% of the women. The indexed ascending aortic size was 1.54 cm/m2 in the men and 1.66 cm/m2 in the women. Valvular heart lesions were diagnosed in about half of the study population. The most common findings were mild regurgitation in the mitral and tricuspid valves. Multiple valvular disorders were found in 55% of the patients. Among significant (moderate and more-than-moderate) VHDs, tricuspid regurgitation (36%), mitral regurgitation (33%), and aortic regurgitation (19%) were the most frequent VHDs. Rheumatic valvular involvement was diagnosed in 31 (1.4%) patients. Conclusions: Echocardiographic parameters vary with age, sex, and race, and they should be population adjusted. We recommend further research in different areas of Iran to obtain sufficient data for the creation of an applicable guideline regarding echocardiographic parameters.

Prophylaxis of Rheumatic Fever/Rheumatic Heart Disease–Penicillin or Azithromycin, Who Wins?

Abstract For eradication of rheumatic fever (RF)/rheumatic heart disease (RHD), we must have a simplified diagnosis, and a treatment which is painless, easily available and safe; prophylaxis must be painless, safe, easily available, readily administered, and comprising a weekly oral single dose. Arati's regime for management of RF/RHD (ARMOR) consists of diagnosis and management of RF/RHD in today's context in a very easy and simplified way. ARMOR criteria: Arthritis or arthralgia with typical features suggestive of RF, carditis or cardiac involvement, typical of RF or RHD, and echocardiographic evidence of rheumatic heart valve involvement should essentially be the criteria to diagnose RF and RHD with high specificity and sensitivity. With regard to treatment of RF/RHD, we need a drug which is highly efficacious against Group A Beta Hemolytic Streptococcus (GABHS), which is the causative agent for primary prevention and treatment and secondary prophylaxis. The best drug discovered, to date, for GABHS is azithromycin. ARMOR for primary prevention, treatment and secondary prophylaxis of RF/RHD is as follows: Azithromycin must be given in a dose of 500 mg 1 tablet daily for 5 days, followed by 1 tablet once a week for 1 year. Penicillin for treatment and prophylaxis must be given up due to its lack of availability, side effects, risk of anaphylaxis, parenteral preparation, hazards of administration, need for sensitivity test each time, etc. and replaced by azithromycin.

Rheumatic involvement and bone scan features in Schnitzler syndrome: initial and follow-up data from a single-center cohort of 25 patients

ObjectiveTo report on the characteristics and long-term course of rheumatic manifestations in Schnitzler syndrome (SchS).MethodsA retrospective cohort study of patients with SchS followed between 2000 and 2020. Inclusion criteria included a diagnosis of SchS (Strasbourg criteria). All available bone scans were reviewed and scored according to the intensity and number of pathological sites. The scintigraphic score was compared with the clinical activity score, CRP level, and treatments.ResultsTwenty-five patients were included. Median age at diagnosis was 68 years. Eighty patients (72%) had SchS-related rheumatic pain. Most patients had a long-standing isolated rash before constitutional and/or rheumatic symptoms appeared. The monoclonal component level was usually very low (IgMκ in 22/25). Rheumatic pain predominated around the knees. Bone scans revealed abnormal tracer uptake in 15/18 (85%). The scintigraphic score correlated with clinical activity (r = 0.4, p < 0.02) and CRP level (r = 0.47, p < 0.01). The scintigraphic score was lower in patients receiving corticosteroids or IL1Ra (interleukin 1 receptor antagonist) than in untreated patients (median scores:2, 0, and 13, respectively; p < 0.05). Two patients developed Waldenström macroglobulinemia. Of the 22 surviving patients, median age at follow-up was 76 years. IL1Ra was used in 13 patients, with dramatic efficacy on both symptoms and bone scan features.ConclusionsRheumatic manifestations are very prevalent in SchS. However, bone pain can be misleading and contribute to misdiagnosis. Bone scan abnormalities are very prevalent and correlate with disease activity and treatments. IL1-Ra has a dramatic and durable efficacy but may not be required in every patient early on.

A Rare Cause of Interstitial Lung Disease in Rheumatology Clinic: Case Report for Sulfasalazine-Induced Acute Pulmonary Injury

Infection, primary lung pathology, rheumatic involvement, malignancy and drug-induced involvement can be suggested as differential diagnosis of a case with rheumatic disease who applied to an emergency service with pulmonary symptoms.The drugs cause 2.5-3% of interstitial lung diseases.Sulfasalazine has been widely used in the treatment of inflammatory rheumatic conditions and it is an extremely rare reason for interstitial lung disase. Here, we aimed to present the rarely seen sulfasalazine-induced interstitial pulmonary disease and its treatment. Sulphasalazine -induced lung disease can mimic the symptoms of infectious and rheumatic lung involvement and can cause serious diagnostic confusion.

Left and right ventricular deformation in patients with severe mitral stenosis and pulmonary hypertension undergoing percutaneous balloon mitral valvuloplasty: A two dimensional speckle-tracking echocardiographic study

Seventy-five patients with isolated severe MS (mitral valve area: 1.10 ± 0.15 cm2) and pulmonary hypertension underwent regional and global longitudinal strain (GLS) measurements of left (LV) and right ventricle (RV) at baseline and within 48 h after percutaneous balloon mitral valvuloplasty (PBMV). PBMV resulted in significant improvement in LV GLS (−16.35 ± 1.67% vs −19.98 ± 2.17%) and RV GLS (−10.34 ± 2.38% vs −13.83 ± 2.04%), p < 0.001 for both. Absolute increase in strain of basal segments of LV was more compared to mid and apical segments. We also found significant positive correlation between decrease in mean LA pressure (pre PBMV 28.91 ± 4.21 mm Hg vs post PBMV 10.55 ± 3.04 mm Hg, difference of 16.36 mm Hg; p < 0.001) obtained invasively during PBMV for 62 patients with improvement in LV GLS (r = 0.257, p = 0.048), RV GLS (r = 0.267, p = 0.043), and fall in right ventricular systolic pressure (r = 0.308, p = 0.022) that occurred post PBMV. The LV dysfunction is predominantly because of altered hemodynamics due to restricted LV filling with additional contribution from rheumatic involvement of basal LV myocardial segments. The improvement in LV deformation after PBMV is likely due to increase in preload. RV afterload reduction because of LA pressure decrease improved RV deformation.

Periprosthetic and interprothetic fractures in patients with rheumatism : Characteristics of conservative and surgical treatment

Periprosthetic fractures in patients with underlying rheumatic diseases can be a special challenge depending on the extent, bone quality and septic complications. Does the underlying rheumatic disease influence the treatment strategy for periprosthetic fractures? What effect do the many extra-articular and local joint-specific rheumatic involvement patterns have on the surgical strategy? How should the potential immunosuppressive side effects of continuously new biologicals be perioperatively taken into account? How can less conspicuous infections under immunosuppression be recognized clinically and using laboratory parameters? Rheumatic disease manifestations that must be perioperatively considered are presented based on the literature. The results are supplemented by many years of experience in a hospital with more than 1500 operations on patients with an underlying rheumatic disease. The treatment of periprosthetic fractures with underlying rheumatic diseases necessitates the specific consideration of the multiple accompanying conditions. In general, extra-articular manifestations, average younger age, increased susceptibility to infections, multiple joint involvement and the basic medication must be taken into consideration. The local poorer bone quality can require an adaptation of the surgical technique. These factors are summarized in a treatment algorithm for periprosthetic fractures in patients with rheumatism taking the stability of the prosthesis, the infection status, the etiology, the localization and extent of the fracture into consideration.