Lauren M Pachman, Brian E Nolan, Deidre DeRanieri, Amer M Khojah
{"title":"Juvenile Dermatomyositis: New Clues to Diagnosis and Therapy.","authors":"Lauren M Pachman, Brian E Nolan, Deidre DeRanieri, Amer M Khojah","doi":"10.1007/s40674-020-00168-5","DOIUrl":"10.1007/s40674-020-00168-5","url":null,"abstract":"<p><strong>Purpose of review: </strong>To identify clues to disease activity and discuss therapy options.</p><p><strong>Recent findings: </strong>The diagnostic evaluation includes documenting symmetrical proximal muscle damage by exam and MRI, as well as elevated muscle enzymes-aldolase, creatine phosphokinase, LDH, and SGOT-which often normalize with a longer duration of untreated disease. Ultrasound identifies persistent, occult muscle inflammation. The myositis-specific antibodies (MSA) and myositis-associated antibodies (MAA) are associated with specific disease course variations. Anti-NXP-2 is found in younger children and is associated with calcinosis; anti-TIF-1γ+ juvenile dermatomyositis has a longer disease course. The diagnostic rash-involving the eyelids, hands, knees, face, and upper chest-is the most persistent symptom and is associated with microvascular compromise, reflected by loss of nailfold (periungual) end row capillaries. This loss is associated with decreased bioavailability of oral prednisone; the bioavailability of other orally administered medications should also be considered. At diagnosis, at least 3 days of intravenous methyl prednisolone may help control the HLA-restricted and type 1/2 interferon-driven inflammatory process. The requirement for avoidance of ultraviolet light exposure mandates vitamin D supplementation.</p><p><strong>Summary: </strong>This often chronic illness targets the cardiovascular system; mortality has decreased from 30 to 1-2% with corticosteroids. New serological biomarkers indicate occult inflammation: ↑CXCL-10 predicts a longer disease course. Some biologic therapies appear promising.</p>","PeriodicalId":11096,"journal":{"name":"Current Treatment Options in Rheumatology","volume":"7 1","pages":"39-62"},"PeriodicalIF":0.0,"publicationDate":"2021-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8336914/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39282552","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Soluble Biomarkers for Prediction of Vascular and Gastrointestinal Disease Severity in Patients with Systemic Sclerosis.","authors":"Miruna Carnaru, Monique Hinchcliff","doi":"10.1007/s40674-021-00171-4","DOIUrl":"10.1007/s40674-021-00171-4","url":null,"abstract":"<p><strong>Purpose of review: </strong>Disease severity biomarkers in patients with systemic sclerosis (SSc) provide an early and noninvasive screening tool to identify patients at increased risk for internal organ involvement that may impact diagnostic testing and treatment decisions. This review will focus on soluble SSc vascular and gastrointestinal disease biomarkers.</p><p><strong>Recent findings: </strong>Due to high morbidity and mortality associated with SSc pulmonary hypertension, multiple biomarkers are currently under investigation including serum autoantibodies, chemistries [such as N-terminal pro-brain natriuretic peptide (NT-proBNP)], proteins [midkine (MDK) and follistatin-like 3 (FSTL3)], chemokines [C-X-C motif ligand 4 (CXCL4) and C-C motif ligand 21 (CCL21)], plasma growth factors [vascular endothelial growth factor (VEGF) and placental growth factor (PlGF)], cell adhesion molecules [vascular cell adhesion molecule 1 (VCAM-1)], and endothelial microparticles [CD144+ endothelial microparticle (CD144+ EMP)]. A subset of these have also been proposed as SSc digital ulcer biomarkers [anti-endothelin-1 type A receptor (anti-ETAR), PlGF, and NT-proBNP]. A combination of NT-proBNP and high sensitivity cardiac troponins T (hs-cTnT) and I (hs-cTnI) may be useful for assessing primary SSc cardiac involvement. Putative SSc renal disease biomarkers include VEGF and endostatin levels; while anti-U1 and U3 ribonucleoprotein (anti-U1- and anti-U3-RNP) antibodies and fecal-calprotectin (F-calprotectin) are associated with GI involvement.</p><p><strong>Summary: </strong>Serum autoantibodies are the mainstay SSc severity biomarkers, but new biomarkers are under investigation.</p>","PeriodicalId":11096,"journal":{"name":"Current Treatment Options in Rheumatology","volume":"1 1","pages":"21-38"},"PeriodicalIF":0.0,"publicationDate":"2021-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12017339/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91357537","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Quantity and Quality of Rheumatoid Arthritis and Osteoarthritis Clinical Practice Guidelines: Systematic Review and Assessment Using AGREE II","authors":"J. Y. Ng, Ashlee M. Azizudin","doi":"10.1007/s40674-021-00172-3","DOIUrl":"https://doi.org/10.1007/s40674-021-00172-3","url":null,"abstract":"","PeriodicalId":11096,"journal":{"name":"Current Treatment Options in Rheumatology","volume":"113 1","pages":"134 - 149"},"PeriodicalIF":0.0,"publicationDate":"2021-02-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88910050","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Evaluation and Treatment of Pediatric Localized Scleroderma: Pearls and Updates","authors":"D. Glaser, K. Torok","doi":"10.1007/s40674-021-00170-5","DOIUrl":"https://doi.org/10.1007/s40674-021-00170-5","url":null,"abstract":"","PeriodicalId":11096,"journal":{"name":"Current Treatment Options in Rheumatology","volume":"85 1","pages":"1-20"},"PeriodicalIF":0.0,"publicationDate":"2021-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81086666","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"In Pursuit of an Effective Treatment: the Past, Present and Future of Clinical Trials in Inclusion Body Myositis","authors":"A. Snedden, J. Lilleker, H. Chinoy","doi":"10.1007/s40674-020-00169-4","DOIUrl":"https://doi.org/10.1007/s40674-020-00169-4","url":null,"abstract":"","PeriodicalId":11096,"journal":{"name":"Current Treatment Options in Rheumatology","volume":"55 1","pages":"1-19"},"PeriodicalIF":0.0,"publicationDate":"2021-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77403729","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Non-infectious Chronic Uveitis in Childhood: Assessment and Treatment in the Biological Era","authors":"R. Ponti, M. Mastrolia, G. Simonini","doi":"10.1007/s40674-020-00166-7","DOIUrl":"https://doi.org/10.1007/s40674-020-00166-7","url":null,"abstract":"","PeriodicalId":11096,"journal":{"name":"Current Treatment Options in Rheumatology","volume":"220 3 1","pages":"82-97"},"PeriodicalIF":0.0,"publicationDate":"2021-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86176190","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A Selva-O'Callaghan, F Romero-Bueno, E Trallero-Araguás, A Gil-Vila, J C Ruiz-Rodríguez, O Sánchez-Pernaute, I Pinal-Fernández
{"title":"Pharmacologic Treatment of Anti-MDA5 Rapidly Progressive Interstitial Lung Disease.","authors":"A Selva-O'Callaghan, F Romero-Bueno, E Trallero-Araguás, A Gil-Vila, J C Ruiz-Rodríguez, O Sánchez-Pernaute, I Pinal-Fernández","doi":"10.1007/s40674-021-00186-x","DOIUrl":"https://doi.org/10.1007/s40674-021-00186-x","url":null,"abstract":"<p><strong>Purpose of the review: </strong>Idiopathic inflammatory myopathies are a heterogeneous group of autoimmune disorders. The presence of different autoantibodies allows clinicians to define distinct phenotypes. Antibodies against the melanoma differentiation-associated protein 5 gene, also called anti-MDA5 antibodies, are associated with a characteristic phenotype, the clinically amyopathic dermatomyositis with rapidly progressive interstitial lung disease. This review aims to analyze the different pharmacological options for the treatment of rapidly progressive interstitial lung disease in patients with anti-MDA5 antibodies.</p><p><strong>Recent findings: </strong>Evidence-based therapeutic recommendations suggest that the best initial approach to treat these patients is an early combination of immunosuppressive drugs including either glucocorticoids and calcineurin inhibitors or a triple therapy adding intravenous cyclophosphamide. Tofacitinib, a Janus kinase inhibitor, could be useful according to recent reports. High ferritin plasma levels, generalized worsening of pulmonary infiltrates, and ground-glass opacities should be considered predictive factors of a bad outcome. In this scenario, clinicians should consider rescue therapies such as therapeutic plasma exchange, polymyxin-B hemoperfusion, veno-venous extracorporeal membrane oxygenation, or even lung transplantation.</p><p><strong>Summary: </strong>Combined immunosuppressive treatment should be considered the first-line therapy for patients with anti-MDA5 rapidly progressive interstitial lung disease. Aggressive rescue therapies may be useful in refractory patients.</p>","PeriodicalId":11096,"journal":{"name":"Current Treatment Options in Rheumatology","volume":" ","pages":"319-333"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8476986/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39483078","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sicong Huang, Vanessa L Kronzer, Paul F Dellaripa, Kevin D Deane, Marcy B Bolster, Vivek Nagaraja, Dinesh Khanna, Tracy J Doyle, Jeffrey A Sparks
{"title":"Rheumatoid arthritis-associated interstitial lung disease: Current update on prevalence, risk factors, and pharmacologic treatment.","authors":"Sicong Huang, Vanessa L Kronzer, Paul F Dellaripa, Kevin D Deane, Marcy B Bolster, Vivek Nagaraja, Dinesh Khanna, Tracy J Doyle, Jeffrey A Sparks","doi":"10.1007/s40674-020-00160-z","DOIUrl":"https://doi.org/10.1007/s40674-020-00160-z","url":null,"abstract":"<p><strong>Purpose of review: </strong>Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is one of the most serious extra-articular RA manifestations. RA-ILD is associated with worse physical function, lower quality of life, and increased mortality. RA-ILD is comprised of heterogeneous subtypes characterized by inflammation and fibrosis. Diagnosis can be difficult since the presentation of RA-ILD is characterized by non-specific symptoms and imaging findings. Management of RA-ILD is also challenging due to difficulty in precisely measuring pulmonary disease activity and response to treatment in patients who may also have articular inflammation. We provide a current overview of RA-ILD focusing on prevalence, risk factors, and treatment.</p><p><strong>Recent findings: </strong>Research interest in RA-ILD has increased in recent years. Some studies suggest that RA-ILD prevalence may be increasing; this may be due to underlying biologic drivers or increases in imaging and recognition. Novel RA-ILD risk factors include the <i>MUC5B</i> promotor variant, articular disease activity, autoantibodies, and biomarkers of damaged pulmonary parenchyma. Treatment should focus on controlling RA disease activity, which emerging data suggest may reduce RA-ILD risk. Immunomodulatory and antifibrotic drugs may also treat RA-ILD.</p><p><strong>Summary: </strong>RA-ILD is an underrecognized and serious manifestation of RA, but important progress is being made in identifying risk factors and treatment.</p>","PeriodicalId":11096,"journal":{"name":"Current Treatment Options in Rheumatology","volume":"6 4","pages":"337-353"},"PeriodicalIF":0.0,"publicationDate":"2020-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1007/s40674-020-00160-z","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38341048","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"An Update on Imaging in Rheumatoid Arthritis","authors":"M. Isbel, S. Paramalingam, P. Conaghan, H. Keen","doi":"10.1007/s40674-020-00165-8","DOIUrl":"https://doi.org/10.1007/s40674-020-00165-8","url":null,"abstract":"","PeriodicalId":11096,"journal":{"name":"Current Treatment Options in Rheumatology","volume":"45 1","pages":"370 - 381"},"PeriodicalIF":0.0,"publicationDate":"2020-11-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86581626","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}