Anti-polymyositis/Scl antibody-positive overlap syndrome of diffuse cutaneous systemic sclerosis, dermatomyositis, systemic lupus erythematosus, and antiphospholipid syndrome.

A 37-year-old Japanese man with a 3-year history of diffuse cutaneous systemic sclerosis was admitted to our hospital with high fever, arthralgia, myalgia, and muscle weakness. A physical examination revealed facial erythema, Gottron's sign, and mechanic's hands in addition to skin sclerosis. Laboratory data revealed significantly elevated levels of creatine kinase and decreased complement.

Clinical features and poor prognostic factors of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis with rapid progressive interstitial lung disease.

Anti-melanoma differentiation-associated gene 5 (MDA5) antibody has been recognized to be significantly associated with a subset of dermatomyositis patients with rapidly progressive interstitial lung disease (RP-ILD). To elucidate the clinical characteristics and poor prognostic factors in Japanese dermatomyositis patients with anti-MDA5 antibody. Clinical features of anti-MDA5 antibody-positive dermatomyositis patients and risk factors, potentially associated with a poor prognosis, were retrospectively analysed.

Two cases of chronic oral ulcers effectively treated with systemic corticosteroid therapy: Circumorificial plasmacytosis and traumatic ulcerative granuloma with stromal eosinophilia.

Chronic oral ulcers are induced by various causative factors. Biopsy from an active site around ulceration is critical for both the definitive diagnosis and proper treatment. We report two cases of chronic oral ulcers, circumorificial plasmacytosis (CP) and traumatic ulcerative granuloma with stromal eosinophilia (TUGSE).

Mechanistic insight into the norepinephrine-induced fibrosis in systemic sclerosis.

Raynaud's phenomenon is frequently observed in systemic sclerosis (SSc) patients, and cold- or stress-induced norepinephrine (NE) has been speculated to be associated with vasoconstriction. Objective was to elucidate the role of NE in fibrosis in SSc. IL-6 is a potent stimulator of collagen production in fibroblasts.

Possible association of elevated serum collagen type IV level with skin sclerosis in systemic sclerosis.

Collagen type IV is the primary collagen in the basement membranes around blood vessels and in the dermoepidermal junction in the skin. Perivascular collagen type IV is synthesized by endothelial cells and pericytes, and contributes to the homeostasis and remodeling of blood vessels. It has been well recognized that elevated serum collagen type IV levels are associated with the liver fibrosis.

Beneficial effect of botulinum toxin A on Raynaud's phenomenon in Japanese patients with systemic sclerosis: A prospective, case series study.

Currently, there is no satisfactory treatment for Raynaud's phenomenon (RP) in systemic sclerosis (SSc). Recently, it has been reported that botulinum toxin A (BTX-A) injection was effective for the treatment of RP in SSc patients. The objective was to assess the efficacy and safety of BTX-A on RP in Japanese SSc patients.

Lichen planus complicated with thymoma: Report of three Japanese cases and review of the published work.

Thymoma is recognized to be complicated with autoimmune diseases, such as myasthenia gravis, pemphigus vulgaris and bullous pemphigoid. Abnormal regulation of autoreactive lymphocytes may be involved in the pathogenesis of the autoimmune diseases. The association of thymoma and lichen planus (LP) is relatively rare.

Clinical and laboratory features of systemic sclerosis complicated with localized scleroderma.

Localized scleroderma (LSc) primarily affects skin, whereas systemic sclerosis (SSc) affects skin and various internal organs. LSc and SSc are considered to be basically different diseases, and there is no transition between them. However, LSc and SSc have several common characteristics, including endothelial cell dysfunction, immune activation, and excess fibrosis of the skin, and there exist several SSc cases complicated with LSc during the course of SSc.

Demographic and clinical features of systemic sclerosis patients with anti-RNA polymerase III antibodies.

Anti-RNA polymerase III antibody (RNAP) is primarily detected in diffuse cutaneous type systemic sclerosis (dcSSc) patients and strongly associated with renal crisis. Additionally, there has been increasing evidence that cancer in SSc patients is associated with RNAP. The aim of this study was to examine the demographic and clinical features of SSc patients with RNAP.

Demographic and clinical features of autoimmune thyroid disorder in Japanese patients with systemic sclerosis.

Autoimmune thyroid disorders (AITD) are characterized by the impairment of the thyroid gland as a result of systemic or organ-specific autoimmune disorders, and the presence of antithyroid autoantibodies, such as antithyroglobulin antibody (AbTg) and antithyroid peroxidase antibody (AbTPO). Several studies have reported the association of AITD with systemic sclerosis (SSc). However, none of those studies analyzed the association between AITD and skin sclerosis in SSc patients.

Elevated plasma homocysteine level is possibly associated with skin sclerosis in a series of Japanese patients with systemic sclerosis.

Homocysteine is a sulfhydryl-containing amino acid that is derived from dietary methionine, and there has been increasing evidence that elevated plasma homocysteine levels are associated with increased risk of cardiovascular diseases, including carotid, coronary and peripheral arterial disease (PAD). The association of plasma homocysteine levels with peripheral vascular involvements, such as Raynaud phenomenon (RP), digital ulcers (DU) in systemic sclerosis (SSc) patients has not been well studied. The objective of this study was to examine plasma homocysteine levels and their clinical associations in patients with SSc.

No association of atherosclerosis with digital ulcers in Japanese patients with systemic sclerosis: evaluation of carotid intima-media thickness and plaque characteristics.

Patients with systemic sclerosis (SSc) usually develop Raynaud's phenomenon, persistent digital ischemia and sometimes develop digital ulcers (DU). Several studies have reported an association of carotid artery atherosclerosis with SSc by evaluating carotid intima-media thickness (IMT) in SSc patients. However, none of those studies analyzed the association between DU and carotid artery atherosclerosis in SSc patients.