Diagnostic criteria, severity classification and guidelines of systemic sclerosis

Abstract

Several effective drugs have been identified for the treatment of systemic sclerosis (SSc). However, in advanced cases, not only their effectiveness is reduced but they may be also harmful due to their side-effects. Therefore, early diagnosis and early treatment is most important for the treatment of SSc. We established diagnostic criteria for SSc in 2003 and early diagnostic criteria for SSc in 2011, for the purpose of developing evaluation of each organ in SSc. Moreover, in November 2013, the American College of Rheumatology and the European Rheumatology Association jointly developed new diagnostic criteria for increasing their sensitivity and specificity, so we revised our diagnostic criteria and severity classification of SSc. Furthermore, we have revised the clinical guideline based on the newest evidence. In particular, the clinical guideline was established by clinical questions based on evidence-based medicine according to the New Minds Clinical Practice Guideline Creation Manual (version 1.0). We aimed to make the guideline easy to use and reliable based on the newest evidence, and to present guidance as specific as possible for various clinical problems in treatment of SSc.

Successful therapy for diffuse cutaneous systemic sclerosis‐induced digital ulcer treated with hyperbaric oxygen therapy

“…3 Thus, the distribution of morphea on the forearms may be relevant to NCA in our case, because morphea lesions alter surface blood flow which would affect nearby cutaneous conditions. 4,5 Nail fold capillary abnormalities preceded skin sclerosis for 10 years in our case. Sato et al 1 reported that 13 NFB-positive and anti-Scl70 antibody-positive patients did not have skin sclerosis initially, and developed skin sclerosis in an average of 6.5 years after NFB appearance.…”

“…4 Wound care and, in severe cases, surgical interventions are also essential components in treatment of BU. 5 Rehabilitation is important especially when the joints are involved, like in our case. 5…”

“…The Japanese guideline states that HBOT is a treatment option and useful for treating dcSSc skin ulcers (recommendation level, 2D). 5 Hyperbaric oxygen therapy provides patients with 100% oxygen at pressures greater than atmospheric pressure. Although the mechanism of HBOT is not well understood, recent evidence indicates that hyperbaric oxygen inhibits the actions of certain cytokines, acting as an immune modulator.…”

Novel disease-modifying drugs against skin fibrosis of systemic sclerosis

“…Furthermore, belimumab (antibody for B-lymphocyte stimulator), dasatinib, and nilotinib (Abl tyrosine kinase and PDGF receptor inhibitors) (49), antibody against lymphotoxin-α, -β, CD40L, CD27L, FasL, OX40L, or tumor necrosis factor superfamily (TRAIL) (8) are speculated to have anti-fibrotic effects. Guideline of SSc was published by a Japanese group in 2018 (64), and evidence of various treatments has been analyzed and evaluated. Further investigation of the effects of novel drugs will contribute to the treatment of skin sclerosis in SSc.…”

‘Narrow-sense’ and ‘broad-sense’ vascular abnormalities of systemic sclerosis

“…Because of color changes, pain, cryaesthesia, and numbness, patients often complain that Raynaud's phenomenon is the most stressful among various symptoms of SSc. In the Japanese guidelines of SSc, calcium antagonists are the most strongly recommended to treat Raynaud's phenomenon (Recommendation level: 1 A), followed by antiplatelet drugs, beraprost sodium, and alprostadil (1 C) [4]. However, it is difficult to completely control this phenomenon using drug therapy alone, and lifestyle guidance, involving avoidance of temperature change and smoking, should be combined.…”