Recovery from severe dysphagia in systemic sclerosis - myositis overlap: a case report

Background: Dysphagia is common in inflammatory myopathies and usually responds to corticosteroids. Severe dysphagia requiring feeding by percutaneous endoscopic gastrostomy is associated with significant morbidity and high mortality.Clinical case: A 56-year old African Black woman initially presented with systemic sclerosis (SSC) - myositis overlap and interstitial lung disease. She responded to high dose corticosteroids and cyclophosphamide followed by azathioprine, with improvement in her lung function and regression of the skin changes. Six years later she had a myositis flare with severe dysphagia. Her myositis improved after high doses of corticosteroids, azathioprine and two doses of intravenous immunoglobulin (IVIG). As her dysphagia persisted, she was fed via a percutaneous endoscopic gastrostomy (PEG) tube and given a course of rituximab. Her dysphagia gradually resolved and the PEG tube was removed within two months. She received another dose of rituximab six months later and continued low dose prednisone and azathioprine. Her muscle power improved, weight returned to normal and she remained well 20 months after hospital discharge.Conclusion: Our patient with SSC-myositis overlap and severe dysphagia requiring PEG feeding, improved with high dose corticosteroids, azathioprine, two courses of IVIG and rituximab, and remained in remission 20 months after hospital discharge.Keywords: Severe dysphagia, systemic sclerosis, myositis overla

Letter from Jeremiah W. Bronaugh to George Sibley, January 27, 1814

Transcript of Letter from Jeremiah W. Bronaugh to George Sibley, January 27, 1814. Bronaugh discusses the status of trade houses; and how the war in Euroipe will affect the US/British war

Recovery from severe dysphagia in systemic sclerosis - myositis overlap: a case report.

Background: Dysphagia is common in inflammatory myopathies and usually responds to corticosteroids. Severe dysphagia requiring feeding by percutaneous endoscopic gastrostomy is associated with significant morbidity and high mortality. Clinical case: A 56-year old African Black woman initially presented with systemic sclerosis (SSC) - myositis overlap and interstitial lung disease. She responded to high dose corticosteroids and cyclophosphamide followed by azathioprine, with improvement in her lung function and regression of the skin changes. Six years later she had a myositis flare with severe dysphagia. Her myositis improved after high doses of corticosteroids, azathioprine and two doses of intravenous immunoglobulin (IVIG). As her dysphagia persisted, she was fed via a percutaneous endoscopic gastrostomy (PEG) tube and given a course of rituximab. Her dysphagia gradually resolved and the PEG tube was removed within two months. She received another dose of rituximab six months later and continued low dose prednisone and azathioprine. Her muscle power improved, weight returned to normal and she remained well 20 months after hospital discharge. Conclusion: Our patient with SSC-myositis overlap and severe dysphagia requiring PEG feeding, improved with high dose corticosteroids, azathioprine, two courses of IVIG and rituximab, and remained in remission 20 months after hospital discharge

Real-world Comparative Effectiveness of Tocilizumab Monotherapy vs. Tumor Necrosis Factor Inhibitors with Methotrexate in Patients with Rheumatoid Arthritis

INTRODUCTION: Controlled clinical studies have shown that the efficacy of tocilizumab (TCZ) monotherapy is superior to that of tumor necrosis factor inhibitor (TNFi) monotherapy and comparable to that of TCZ plus methotrexate (MTX) for the treatment of rheumatoid arthritis (RA). This study compared the real-world effectiveness of TCZ monotherapy vs. TNFis plus MTX in US patients with RA. METHODS: TCZ-naive patients from the Corrona RA registry with prior exposure to \u3e /= 1 TNFi who initiated TCZ monotherapy or TNFi + MTX were included. Outcomes included mean change in Clinical Disease Activity Index (CDAI), achievement of low disease activity (LDA; CDAI \u3c /= 10), achievement of modified American College of Rheumatology (mACR) 20/50 responses, and mean change in modified Health Assessment Questionnaire (mHAQ) at 6 months. Patients initiating TNFi + MTX were grouped by MTX dose ( \u3c /= 10 mg; \u3e 10 to \u3c /= 15 mg; \u3e 15 to \u3c /= 20 mg; \u3e 20 mg); outcomes in each group were compared with TCZ monotherapy using trimmed populations (excluding patients outside the propensity score distribution overlap). RESULTS: Patients in all groups experienced improvement in CDAI at 6 months (mean change, - 6.9 to - 9.7), with no significant differences between the TCZ monotherapy and TNFi + MTX groups. Achievement of LDA and mACR responses at 6 months were comparable between the TCZ monotherapy and TNFi + MTX groups; overall, 26.8-38.0% of patients achieved LDA, 24.3-37.6% achieved mACR20 response and 13.2-20.8% achieved mACR50 response. The mean change in mHAQ at 6 months was - 0.1 in all groups. CONCLUSIONS: In this real-world population of US patients with RA who had prior TNFi exposure, there was no evidence of a difference in the effectiveness of TCZ monotherapy compared with that of TNFi + MTX, regardless of MTX dose, at 6 months for improving RA disease activity. FUNDING: Corrona, LLC. Plain language summary available for this article

The Dark Side of SAPHO Syndrome

SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) is a relatively rare entity. The therapeutic approach of patients with SAPHO syndrome has included multiple drugs with varying success and incoherence responses. The therapy is still empirical today. SAPHO syndrome is commonly treated with non-steroidal anti-inflammatory drugs, bisphophonates and non-biologic disease modifying antirheumatic drugs. Recent reports showed successful treatment with tumour necrosis factor α (TNF α) antagonists, but there is still a dark side of SAPHO syndrome including a subgroup of patient's refractory to all the treatments that have been empirically experienced. A clinical report of a patient with SAPHO syndrome with 12 years of evolution is described. All the therapeutic approaches, including anti TNF α therapy, have not prevented the clinical and radiographic progression of the disease. Given that the disease affects mostly younger patients, new therapeutic strategies are necessary in order to avoid potentially irreversible joint and bone lesions.info:eu-repo/semantics/publishedVersio

RNA sequencing from human neutrophils reveals distinct transcriptional differences associated with chronic inflammatory states

Background The transcriptional complexity of mammalian cells suggests that they have broad abilities to respond to specific environmental stimuli and physiologic contexts. These abilities were not apparent a priori from the structure of mammalian genomes, but have been identified through detailed transcriptome analyses. In this study, we examined the transcriptomes of cells of the innate immune system, human neutrophils, using RNA sequencing (RNAseq). Methods We sequenced poly-A RNA from nine individual samples corresponding to specific phenotypes: three children with active, untreated juvenile idiopathic arthritis (JIA)(AD), three children with the same disease whose disease was inactive on medication (CRM), and three children with cystic fibrosis (CF). Results We demonstrate that transcriptomes of neutrophils, typically considered non-specific in their responses and functions, display considerable specificity in their transcriptional repertoires dependent on the pathologic context, and included genes, gene isoforms, and long non-coding RNA transcripts. Furthermore, despite the small sample numbers, these findings demonstrate the potential of RNAseq approaches to biomarker development in rheumatic diseases. Conclusions These data demonstrate the capacity of cells previously considered non-specific in function to adapt their transcriptomes to specific biologic contexts. These data also provide insight into previously unrecognized pathological pathways and show considerable promise for elucidating disease and disease-state specific regulatory networks

Use and Significance of Anti CCP Antibodies in Rheumatoid Arthritis.

INTRODUCTION : Rheumatoid Arthritis (RA) is the most common systemic inflammatory, auto immune Rheumatic disease of unknown etiology affecting nearly 1% of the adult population worldwide. It is characterized by chronic and erosive polyarthritis, (usually involving small, peripheral joints in a symmetric distribution) caused by abnormal growth of synovial tissue or pannus, and causes irreversible joint deformity that can lead to severe disability with considerable morbidity. Although the precise aetiology of RA remains unknown, there is strong evidence for autoimmunity, since several auto antibodies are associated with the disease. The potential of the synovial inflammation to cause cartilage damage and bone erosions and subsequent changes in joint integrity is the hallmark of the disease. Despite its destructive potential, the course of RA can be quite variable. Some patients may experience only a mild oligo articular illness of brief duration with minimal joint damage, but most will have a relentless progressive polyarthritis with marked functional impairment. AIMS AND OBJECTIVES : Aim: The aim of the present study is to evaluate the use and significance of AntiCCP antibodies in Rheumatoid Arthritis, and to compare it with other Arthritis - Early Synovitis (ES), Connective Tissue Disorders (CTD) including SLE, and Osteo Arthritis (OA), and in Healthy Blood Donors as control. Objectives: • To evaluate the diagnostic utility of Anti- CCP (cyclic citrullinated peptide) antibody in Rheumatoid arthritis. • To study & compare the presence of Anti- CCP antibody in Rheumatoid arthritis (RA) with other arthritis. – Early Synovitis (ES),Connective Tissue Disorders (CTD) including SLE, and Osteo Arthritis (OA). • To evaluate the significance of Anti- CCP antibody in Sero Negative Rheumatoid Arthritis. • To assess the sensitivity and specificity of the Anti CCP antibody test with RF test in RA and other arthritis. MATERIALS AND METHODS : The total number of subjects in this study for evaluation were 250, which included both males and females.The study subjects were selected according to the inclusion criteria’s mentioned below, from the patients who attended the Out Patient Clinics at the Rheumatology and Orthopaedics Department and Healthy Blood Donors who attended the Blood bank, Coimbatore Medical College Hospital, Coimbatore. The study subjects, in both genders were divided into five groups. Each group include 50 patients. Four groups based upon the clinical conditions and fifth group, healthy individuals (blood donors) as control, as follows: Group I: Rheumatoid Arthritis (RA) - 50 patients. Group II: Early Synovitis (ES) - 50 patients. Group III: Connective Tissue Disorders including Systemic Lupus Erythematosus (SLE) - 50 patients. Group IV: Osteo Arthritis (OA) - 50 patients, and Group V: Healthy Blood Donors (HBD) - 50 patients. Inclusion Criteria: Early Synovitis: Patients with complaints of joint pain. (Synovitis – joint pain, blotted feeling, redness, fever for > 6wks & < 12 months duration.) • Joint pain with no h/o injury or sepsis. • Without any bony deformity. • Not already on treatment for RA. (Inclusion19of patients fulfiling ≥ 2 clinical and ≥1 laboratory criterion and duration of symptoms ≤ 12 weeks. Exclusion Criteria: RA: If does not fit in to the ACR criteria. Early Synovitis: • Complaints of joint pain (synovitis 12months). • Joint pain with h/o injury or sepsis. • With bone and joint deformity. • Already on treatment for RA. RESULTS : The present study was conducted with the patients who attended the Out Patient Clinics at the Rheumatology and Orthopaedics Department of Coimbatore Medical College Hospital, Coimbatore. 200 subjects (including both males and females) were recruited for the study. The subjects were categorized into four groups – RA, ES, CTD, and OA (each group 50) based upon the clinical conditions, and 50 Healthy Blood Donors who attended the Blood bank, Coimbatore Medical College Hospital, Coimbatore, were selected as control and categorized into fifth group. CONCLUSION : In conclusion, based upon the higher sensitivity and specificity of the Anti CCP test in RA, the current study is of diagnostic, and public importance, because it suggests that Anti CCP test should be included in the investigation of undifferentiated arthritis, since a considerable amount of Rheumatic disease associated work disability starts in the first few years of the disease. Anti-CCP antibodies have all the hallmarks of establishing themselves firmly in the diagnostic algorithm of Rheumatoid Arthritis providing additive sensitivity to Rheumatoid Factor. So it should also be included among the diagnostic criteria of RA along with RF. It can also be used as a prognostic indicator in RA patients on treatment