The role of interleukin-17 in the pathogenesis of systemic sclerosis: Pro-fibrotic or anti-fibrotic?

Silvia Bellando-Randone, Emanuel Della-Torre, Andra Balanescu

doi : 10.1177/23971983211039421

First Published August 14, 2021; pp. 227–235

Systemic sclerosis is characterized by widespread fibrosis of the skin and internal organs, vascular impairment, and dysregulation of innate and adaptive immune system. Growing evidence indicates that T-cell proliferation and cytokine secretion play a major role in the initiation of systemic sclerosis, but the role of T helper 17 cells and of interleukin-17 cytokines in the development and progression of the disease remains controversial. In particular, an equally distributed body of literature supports both pro-fibrotic and anti-fibrotic effects of interleukin-17, suggesting a complex and nuanced role of this cytokine in systemic sclerosis pathogenesis that may vary depending on disease stage, target cells in affected organs, and inflammatory milieu. Although interleukin-17 already represents an established therapeutic target for several immune-mediated inflammatory diseases, more robust experimental evidence is required to clarify whether it may become an attractive therapeutic target for systemic sclerosis as well.

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Responsiveness of the University of California Los Angeles Scleroderma Clinical Trial Consortium gastrointestinal tract 2.0 (UCLA-SCTC-GIT 2.0)to change in scleroderma patients

Yossra Atef Suliman, Suzanne Kafaja, Mohamed Alemam, Yasser Shaweesh, Kasra Tavakoli, Daniel E Furst

doi : 10.1177/2397198321992197

First Published February 22, 2021; pp. 236–241

Gastrointestinal tract involvement in systemic sclerosis is the most common internal organ involvement. Among the few validated patient-reported outcome measures for gastrointestinal involvement are the University of California Los Angeles Scleroderma Clinical Trial Consortium gastrointestinal tract 2.0 (UCLA-GIT 2.0) and the gastrointestinal problems’ visual analog scale (SHAQ-VAS). The latter is a component of the Scleroderma Health Assessment Questionnaire Disability Index. Our aim is to compare the responsiveness of the UCLA-GIT 2.0 total score, single domains, upper and lower gastrointestinal domains, and gastrointestinal problems’ visual analog scale of the scleroderma HAQ(SHAQ-GI-VAS) to change in gastrointestinal medication. In addition, we evaluated the correlation between the UCLA-GIT 2.0 and SHAQ-GI-VAS scale in our systemic sclerosis population.

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A pilot study of dimethyl fumarate in pulmonary arterial hypertension associated with systemic sclerosis

Kristi Kong, Diane Koontz, Christina Morse, Eileen Roth, Robyn T Domsic, Marc A Simon, Eric Stratton, Connor Buchholz, Kimberly Tobin-Finch, Robert Simms, M Patricia George, Paul M Hassoun, Harrison Farber, Robert Lafyatis

doi : 10.1177/23971983211016196

First Published May 28, 2021; pp. 242–246

Given the poor treatment options for pulmonary arterial hypertension–associated systemic sclerosis patients, we sought to determine clinical safety and efficacy of dimethyl fumarate, an Nrf2 agonist, and the effects on biomarkers of oxidative stress on pulmonary arterial hypertension–associated systemic sclerosis in an exploratory interventional clinical trial.

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Fundoplication after lung transplantation in patients with systemic sclerosis–related end-stage lung disease

Miguel M Leiva-Juárez, Andreacarola Urso, Joseph Costa, Bryan P Stanifer, Joshua R Sonett, Luke Benvenuto, Megan Aversa, Hilary Robbins, Lori Shah, Selim Arcasoy, Frank D’Ovidio

doi : 10.1177/23971983211016210

First Published May 25, 2021; pp. 247–255

Gastroesophageal reflux and aspiration are risk factors for chronic lung allograft dysfunction in lung transplant recipients. Patients with systemic sclerosis are at an increased risk of aspiration due to esophageal dysmotility and an ineffective lower esophageal sphincter. The aim of this study is to understand the effect of fundoplication on outcomes in systemic sclerosis recipients.

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Autonomic nervous system dysfunction correlates with microvascular damage in systemic sclerosis patients

Francesco Masini, Raffaele Galiero, Pia Clara Pafundi, Klodian Gjeloshi, Emanuele Pinotti, Roberta Ferrara, Ciro Romano, Luigi Elio Adinolfi, Ferdinando Carlo Sasso, Giovanna Cuomo

doi : 10.1177/23971983211020617

First Published June 7, 2021; pp. 256–263

Cardiac autonomic neuropathy is among the known cardiovascular complications of systemic sclerosis and may affect the whole prognosis of the disease. The aim of our study was to assess cardiac autonomic neuropathy prevalence in our cohort of systemic sclerosis patients and compare its main features with clinical and epidemiological data, particularly with the severity of microvascular damage, as detected by nailfold videocapillaroscopy.

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Epidemiology and trends in survival of systemic sclerosis in Olmsted County (1980–2018): A population-based study

Caitrin M Coffey, Yasser A Radwan, Avneek S Sandhu, Cynthia S Crowson, Philippe R Bauer, Eric L Matteson, Ashima Makol

doi : 10.1177/23971983211026853

First Published June 23, 2021; pp. 264–270

To update the epidemiology of systemic sclerosis and evaluate the performance of the American College of Rheumatology/European League Against Rheumatism 2013 versus 1980 American Rheumatism Association classification criteria in a US population-based cohort.

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Mycophenolate in scleroderma-associated interstitial lung disease: Real-world data from rheumatology and pulmonology clinics in South Asia

Ramya Janardana, Aparna Irodi, Pramod P Chebbi, Ruchika Goel, Leena R Vimala, Shivraj Padiyar, Anoof Peediyakal, John Mathew, Aswin Nair, Devasahayam J Christopher, Debashish Danda

doi : 10.1177/23971983211024410

First Published June 30, 2021; pp. 271–276

There is a paucity of real-world data on mycophenolate mofetil/mycophenolate sodium in systemic sclerosis-related interstitial lung disease.

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Systemic sclerosis portends increased risk of conduction and rhythm abnormalities at diagnosis and during disease course: A US population-based cohort

Yasser A Radwan, Reto D Kurmann, Avneek S Sandhu, Edward A El-Am, Cynthia S Crowson, Eric L Matteson, Thomas G Osborn, Kenneth J Warrington, Rekha Mankad, Ashima Makol

doi : 10.1177/23971983211034074

First Published July 28, 2021; pp. 277–285

To study the incidence, risk factors, and outcomes of conduction and rhythm disorders in a population-based cohort of patients with systemic sclerosis versus nonsystemic sclerosis comparators.

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Circulating Gremlin-1 is elevated in systemic sclerosis patients

Steven O’Reilly

doi : 10.1177/23971983211036571

First Published August 2, 2021; pp. 286–289

Systemic sclerosis is an autoimmune connective tissue disease in which there is activation of the immune system, vascular disease and fibrosis. Activation of quiescent fibroblasts to myofibroblasts is key to disease pathogenesis. Gremlin-1 is a bone morphogenetic protein antagonist which is important in development and we recently reported in skin fibrosis. The aim of this study was to determine the serum circulating levels of Gremlin-1 in early diffuse systemic sclerosis.

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Fecal microbiome differs between patients with systemic sclerosis with and without small intestinal bacterial overgrowth

Daniel Levin, Giada De Palma, Hannah Zou, Ava Hadi Zadeh Bazzaz, Elena Verdu, Barbara Baker, Maria Ines Pinto-Sanchez, Nader Khalidi, Maggie J Larché, Karen A. Beattie, Premysl Bercik

doi : 10.1177/23971983211032808

First Published July 24, 2021; pp. 290–298

Gastrointestinal manifestations of systemic sclerosis affect up to 90% of patients, with symptoms including diarrhea and constipation. Small intestinal bacterial overgrowth is a condition associated with increased numbers of pathogenic bacteria in the small bowel. While currently unknown, it has been suggested that dysregulation of the fecal microbiota may play a role in the development of systemic sclerosis and small intestinal bacterial overgrowth.

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Limited cutaneous systemic sclerosis: Total rehabilitation with fixed prosthesis on dental implants

Miguel Angel Garcés Villalá, Carolina Zorrilla Albert

doi : 10.1177/23971983211004362

First Published April 1, 2021; pp. 299–305

Limited cutaneous systemic sclerosis with special manifestations (calcinosis cutis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) is part of the group of connective tissue diseases, these rare autoimmune systemic pathologies cause thickening and hardening of tissues in different parts of the body and can lead to complex disorders. Oral manifestations of systemic sclerosis may include limited ability to open the mouth, xerostomia, periodontal disease, enlarged periodontal ligament, and bone resorption of the jaw.

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New-onset systemic sclerosis and scleroderma renal crisis under docetaxel

Véronique Debien, Arthur Petitdemange, Dorothée Bazin, Carole Ederle, Benoit Nespola, Hamid Merdji, Jérome Olagne, Thierry Martin, Aurélien Guffroy, Carole Pflumio

doi : 10.1177/23971983211007669

First Published April 13, 2021; pp. 306–310

Systemic sclerosis is a rare systemic autoimmune disease characterized by microvascular impairment and fibrosis of the skin and other organs with poor outcomes. Toxic causes may be involved. We reported the case of a 59-year-old woman who developed an acute systemic sclerosis after two doses of adjuvant chemotherapy by docetaxel and cyclophosphamide for a localized hormone receptor + human epithelial receptor 2�breast cancer. Docetaxel is a major chemotherapy drug used in the treatment of breast, lung, and prostate cancers, among others. Scleroderma-like skin-induced changes (morphea) have been already described for taxanes. Here, we report for the first time a case of severe lung and kidney flare with thrombotic microangiopathy after steroids for acute interstitial lung disease probably induced by anti-RNA polymerase III + systemic sclerosis after docetaxel.

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Scleredema with biopsy-confirmed cardiomyopathy: A case report

Mei Nakatsuji, Naoto Ishimaru, Jun Ohnishi, Shimpei Mizuki, Yohei Kanzawa, Kei Kawano, Takahiro Nakajima, Masaru Kuroda, Nobuya Sano, Saori Kinami

doi : 10.1177/23971983211013977

First Published May 12, 2021; pp. 311–315

Scleredema is a rare cutaneous mucinosis characterized by diffuse swelling and non-pitting induration. A 63-year-old man reported a 5-year history of skin thickening of the trunk and a 3-week history of dyspnea. Echocardiography revealed diffuse hypokinesis. Skin biopsies obtained from the waist showed thickened dermis with mucin. Myocardial biopsies showed alcian blue-stained tissue between the muscle fibers. The patient was referred to a dermatologist for phototherapy. Cardiomyopathy should be considered in patients with scleredema. Scleredema usually has a good prognosis; however, the mortality risk could be high when accompanied by cardiomyopathy.

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Scleroderma associated with renal cell carcinoma: A case report and literature review

Hiral S. Patel, Vanya Aggarwal, Rupak Thapa

doi : 10.1177/23971983211013973

First Published May 18, 2021; pp. 316–319

Systemic sclerosis (aka scleroderma) is an autoimmune disease with no known definitive etiology, but genetic, infectious, and non-infectious exposures have been associated with its occurrence. Previous studies have shown that systemic sclerosis can be a paraneoplastic phenomenon and may be associated with increased risk of malignancy, most commonly lung, skin, and breast cancers. The association of renal cell carcinoma with systemic sclerosis is rare.

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Scleroderma renal crisis following Covid-19 infection

Doron Rimar, Itzhak Rosner, Gleb Slobodin

doi : 10.1177/23971983211016195

First Published May 28, 2021; pp. 320–321

Systemic sclerosis (SSc) is an autoimmune disease in which environmental exposure to substances and agents may trigger disease onset or exacerbation. The most fatal complication of SSc is scleroderma renal crisis (SRC), the incidence of which is 2-3%. SRC usually occurs in the first 5 years from disease onset in diffuse-SSc patients with anti-topoisomerase 1 (ATA) or RNA polymerase 3 antibodies [1]. Other risk factors for SRC are pericardial effusion, tendon friction rub and steroid use. We report herein a case of scleroderma renal crisis (SRC), following covid-19 infection, in a limited-SSc patient who was in long remission prior to the infection without any risk factors for SRC. the temporal relationship and lack of other risk factors combine to suggest covid-19 infection as a possible trigger for SRC. We discuss the shared pathophysiology of covid-19 infection and SRC, including, vasculopathy, endothelial activation, hypercoagulability, cytokines release as interleukin 6, that may explain the possible role of covid-19 infection, as a trigger for SRC in SSc patients.

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Ascending aorta aneurysm in scleroderma

Bhavya Chintala, Lalit Duggal, Neeraj Jain

doi : 10.1177/23971983211023322

First Published June 21, 2021; pp. 322–326

Ascending aorta aneurysm in scleroderma can be ascribed to its macrovascular involvement which is very less elucidated. We here describe a 56-year-old female with rapidly progressive diffuse cutaneous scleroderma. She presented with skin thickening involving all four limbs, thorax and abdomen. She had other features like arthritis, Raynaud’s phenomena, dyspnoea, heaviness of chest, and dysphagia. On investigation, she was strongly positive for antinuclear antibody and Scl 70. Imaging revealed interstitial lung disease (nonspecific interstitial pneumonia pattern) and a fusiform dilatation of ascending aorta of 6.5 cm. Patient was offered surgical correction for aneurysm, for which she refused. To the best of our knowledge, our case report adds up to the few cases of ascending aorta aneurysm in scleroderma available in world literature.

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Is axillary botulinum toxin efficient in controlling secondary Raynaud’s phenomenon? A case report

David DeMasters, Emily Sturgill, Alan Bartholomew

doi : 10.1177/23971983211034077

First Published July 29, 2021; pp. 327–329

Raynaud’s phenomenon when secondary to underlying systemic disease such as systemic sclerosis occurs early in the disease course and progression can bring significant morbidity such as pain, digital ulceration, and necrosis. Standard medical therapies are aimed at promoting distal arterial vasodilation but are often inadequate in managing Raynaud’s phenomenon. Options for refractory cases include surgical and chemical sympathectomy with Botulinum neurotoxin type A (BoNT/A) hand injections but the latter can be associated with transient hand weakness. We describe the case of a 35-year-old woman with undifferentiated connective tissue disease, Raynaud’s phenomenon, and concomitant primary focal axillary hyperhidrosis for which she received axillary BoNT/A therapy every 6 months who noted significant improvement in her Raynaud’s phenomenon and hand arthralgias for 5 months following the axillary injections. This effect remained durable after 24 months of therapy. This improvement in Raynaud’s phenomenon after axillary BoNT/A has not been previously described.

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Scleroderma as paraneoplastic disease: A new case of a rare association with renal cell carcinoma

Francesca Ruffilli, Melissa Padovan, Giovanni Ciancio, Veronica Venturelli, Beatrice Maranini, Marcello Govoni

doi : 10.1177/23971983211050810

First Published October 12, 2021; pp. 330–331

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