{"id":390683,"date":"2018-05-14T00:00:00","date_gmt":"2018-05-14T00:00:00","guid":{"rendered":"https:\/\/clarivate.com\/life-sciences-healthcare\/report\/nrlfim0003-2018-biopharma-scleroderma-systemic-sclerosis-niche-rare-disease-landscape-forecast-us-eu5-2018\/"},"modified":"2026-04-15T09:56:25","modified_gmt":"2026-04-15T09:56:25","slug":"nrlfim0003-2018-biopharma-scleroderma-systemic-sclerosis-niche-rare-disease-landscape-forecast-us-eu5-2018","status":"publish","type":"report","link":"https:\/\/clarivate.com\/life-sciences-healthcare\/report\/nrlfim0003-2018-biopharma-scleroderma-systemic-sclerosis-niche-rare-disease-landscape-forecast-us-eu5-2018\/","title":{"rendered":"Scleroderma (Systemic Sclerosis) | Niche &#038; Rare Disease Landscape &#038; Forecast | US\/EU5 | 2018"},"content":{"rendered":"<p>Scleroderma (systemic sclerosis [<abbr title=\"systemic sclerosis\">SSc<\/abbr>]) is a rare progressive autoimmune disorder characterized by skin fibrosis, systemic inflammation, and varying degrees of vasculopathy that manifest as Raynaud\u2019s disease and, often, painful digital ulcers. Risks of pulmonary arterial hypertension (<abbr title=\"pulmonary arterial hypertension\">PAH<\/abbr>), interstitial lung disease (<abbr title=\"interstitial lung disease\">ILD<\/abbr>), and renal crisis are higher in\u00a0<abbr title=\"systemic sclerosis\">SSc<\/abbr>\u00a0patients than in the general population\u00a0and underlie a high mortality rate. No disease-modifying therapy (<abbr title=\"disease-modifying therapy\">DMT<\/abbr>) has been approved to treat\u00a0<abbr title=\"systemic sclerosis\">SSc<\/abbr>. Systemic immunosuppressants are prescribed to treat\u00a0<abbr title=\"interstitial lung disease\">ILD<\/abbr>\u00a0and skin fibrosis, while symptoms of\u00a0<abbr title=\"pulmonary arterial hypertension\">PAH<\/abbr>\u00a0and vasculopathy are treated with varying combinations of calcium-channel blockers, endothelin receptor antagonists,\u00a0<abbr title=\"phosphodiesterase\">PDE<\/abbr>-5 inhibitors, and prostacyclin analogues. Renal crisis is treated acutely with\u00a0<abbr title=\"angiotensin-converting enzyme\">ACE<\/abbr>\u00a0inhibitors, which interviewed experts credit with having reduced mortality from this complication. Still, current\u00a0<abbr title=\"systemic sclerosis\">SSc<\/abbr>\u00a0treatments are only marginally effective, leaving tremendous need for effective\u00a0<abbr title=\"disease-modifying therapy\">DMT<\/abbr>s and antifibrotics to treat this debilitating condition. Several marketed drugs (e.g., Boehringer Ingelheim\u2019s Ofev, Roche\u2019s Actemra\/RoActemra) are in Phase III studies in\u00a0<abbr title=\"systemic sclerosis\">SSc<\/abbr>.<\/p>\n<p>QUESTIONS ANSWERED<\/p>\n<ul>\n<li>How many people are currentlydiagnosed with\u00a0<abbr title=\"systemic sclerosis\">SSc<\/abbr>in the United States and\u00a0<abbr title=\"France, Germany, Italy, Spain, UK\">EU5<\/abbr>; how many will be diagnosed over time?<\/li>\n<li>What is the commercial potential of key emerging therapies? How will emerging therapies fit into the\u00a0<abbr title=\"systemic sclerosis\">SSc<\/abbr>\u00a0treatment landscape?<\/li>\n<li>What is the current treatment landscape in\u00a0<abbr title=\"systemic sclerosis\">SSc<\/abbr>, and how will it change in the next ten years?<\/li>\n<li>What do key opinion leaders consider the greatest unmet medical needs in the management of\u00a0<abbr title=\"systemic sclerosis\">SSc<\/abbr>?<\/li>\n<\/ul>\n<p>PRODUCT DESCRIPTION<\/p>\n<p>Niche &#038; Rare Disease Landscape &#038; Forecast: Comprehensive market intelligence providing world-class epidemiology, keen insight into current treatment paradigms, in-depth pipeline assessments, and drug forecasts supported by detailed primary and secondary research.<\/p>\n","protected":false},"template":"","class_list":["post-390683","report","type-report","status-publish","hentry","report-gateway-biopharma","biopharma-therapy-areas-scleroderma-systemic-sclerosis","biopharma-geography-us","biopharma-date-920"],"acf":[],"publishpress_future_workflow_manual_trigger":{"enabledWorkflows":[]},"_links":{"self":[{"href":"https:\/\/clarivate.com\/life-sciences-healthcare\/wp-json\/wp\/v2\/report\/390683","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/clarivate.com\/life-sciences-healthcare\/wp-json\/wp\/v2\/report"}],"about":[{"href":"https:\/\/clarivate.com\/life-sciences-healthcare\/wp-json\/wp\/v2\/types\/report"}],"version-history":[{"count":2,"href":"https:\/\/clarivate.com\/life-sciences-healthcare\/wp-json\/wp\/v2\/report\/390683\/revisions"}],"predecessor-version":[{"id":576734,"href":"https:\/\/clarivate.com\/life-sciences-healthcare\/wp-json\/wp\/v2\/report\/390683\/revisions\/576734"}],"wp:attachment":[{"href":"https:\/\/clarivate.com\/life-sciences-healthcare\/wp-json\/wp\/v2\/media?parent=390683"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}