Overlap syndrome is defined as a disease entity that satisfies the classification criteria of at least two connective tissue diseases occurring concurrently or separately in a single patient. Here, we report a rare case of a 59-year-old woman with diffuse systemic sclerosis with lung involvement-rheumatoid arthritis overlap syndrome accompanied by cutaneous sarcoidosis. Although there is no consensus for the optimal treatment of overlap syndrome to date, this case of co-existing rheumatoid arthritis and systemic sclerosis with interstitial lung disease successfully responded to abatacept.
Abatacept ; Arthritis ; Arthritis, Rheumatoid ; Classification ; Connective Tissue Diseases ; Consensus ; Female ; Humans ; Lung ; Lung Diseases, Interstitial ; Middle Aged ; Sarcoidosis ; Scleroderma, Diffuse ; Scleroderma, Systemic

OBJECTIVETo examine the efficacy and safety of bathing therapy with Taohong Siwu Decoction (, TSD) in the treatment of early-stage, mild-moderate diffuse cutaneous systemic sclerosis (dcSSc).

METHODSThis randomized, placebo-controlled trial enrolled 148 men and women (18-60 years) with dcSSc (disease duration 12 months) and baseline modified Rodnan skin score (MRSS) 10. Patients were randomized into a TSD group (71 cases bathing with TSD plus oral prednisone) or control group (71 cases bathing with placebo plus oral prednisone). Bathing (40 °C, 30 min) of the upper and lower limbs was carried out once daily for 12 consecutive weeks. The primary outcome measure was MRSS; secondary outcomes were Raynaud's phenomenon (RP) score, quality of life (QOL), physician visual analogue scale (VAS), patient VAS, percent predicted diffusing capacity for carbon monoxide (DLCO), percent predicted forced vital capacity (FVC), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) level and overall treatment effect.

RESULTSThe final analysis included 135 patients (control group, 68 cases; TSD group, 67 cases). Primary and secondary outcome measures after 2 weeks of treatment showed no improvement (versus baseline) in both groups, with no differences between groups. At 12 weeks, QOL, physician VAS, patient VAS, ESR and CRP were improved in both groups, but MRSS and RP score were improved only in the TSD group (all P<0.05). MRSS, RP score, QOL, physician VAS, patient VAS, ESR and CRP differed significantly between groups (all P<0.05). Meanwhile, the overall treatment effect was significantly higher in the TSD group than in the control group (P<0.05). Adverse events in the two groups were similar (P>0.05).

CONCLUSIONSBathing with TSD plus oral prednisone achieves better outcomes than oral prednisone alone in patients with dcSSc and is not associated with serious adverse events.

Adult ; Drugs, Chinese Herbal ; adverse effects ; therapeutic use ; Female ; Humans ; Hygiene ; Intention to Treat Analysis ; Male ; Middle Aged ; Outcome Assessment (Health Care) ; Placebos ; Scleroderma, Diffuse ; drug therapy ; Treatment Outcome

Systemic sclerosis is an intractable clinical subset characterized by diffuse fibrosis, vasculopathy, and immune abnormalities. Diffuse cutaneous systemic sclerosis has a variety of symptoms depending on the internal organs involved, such as the lungs, heart, kidneys, and gastrointestinal system. Gastrointestinal involvement is frequent and one of the major cause of morbidity and mortality. There is no sustained, effective therapy for treating the active gastrointestinal involvement of systemic sclerosis. Although immunoglobulin is commonly used in the treatment of other autoimmune diseases, its effects in the treatment of the gastrointestinal involvement in systemic sclerosis are unclear. Here, we report a patient with a severe cough caused by uncontrolled gastrointestinal involvement in diffuse cutaneous systemic sclerosis treated with immunoglobulin.
Autoimmune Diseases ; Cough ; Fibrosis ; Heart ; Humans ; Immunoglobulins* ; Kidney ; Lung ; Mortality ; Scleroderma, Diffuse ; Scleroderma, Systemic*

BACKGROUND: Systemic sclerosis (SSc) is a rare, connective tissue  disease  with  multisystem involvement.This is due to immunological processes,vascular endothelial cell injury  and extensive activation of fibrolast that commonly affects the skin and other internal organs such as the esophagus, lungs, heart, and kidneys. SSc has one of the highest mortality among  the  autoimmune  rheumatic  diseases,  hence  the  emphasis  on  the  early  recognition  and  management  to  prevent significant progression of the disease.
CASE: A 22-year-old  female  presented  with  a  one-year history of multiple hard and hypopigmented patches on the face, neck, trunk and upper extremities. Further examination revealed mask-like facies, microstomia, frenulum sclerosis, Raynaud's phenomenon, pitted scars on the digital pulp of hands  and  sclerodactyly.Baseline  blood  chemistry,chest radiograph and electrocardiography  were  all  negative  for systemic involvement. Autoantibodies were positive for dsDNA, SS-A/Ro and Scl-70. Skin biopsy revealed sclerosing dermatitis, which was consistent with SSc.
OUTCOME: The  patient  was  initially  started  with  oral prednisone 0.5 mg/kg/day and was increased to 0.75 mg/kg/day for eight weeks. Prednisone was slowly tapered to 5.0 mg/day and methotrexate 15.0 mg/week was included in  the  management for eight weeks which resulted in decreased joint pains, halted the progression of skin induration, decreased in pruritus and palmar edema.
CONCLUSION: The characteristic dermatological findings of SSc are not only important signs to dermatologists, but these serves as diagnostic clues for clinicians from other disciplines as well. In our case, the presence of the autoantibody Scl-70 indicated the potential risk of pulmonary fibrosis and pulmonary arterial hypertension that accounts with high mortality.Hence,physicians should be aware of the possible risk of organ damage,even when asymptomatic because there is a high risk of disease progression. The importance of early recognition and a multidisciplinary approach lead to the good outcome in this case.

Human ; Female ; Adult ; Autoantibodies ; Prednisone ; Methotrexate ; Cicatrix ; Microstomia ; Sclerosis ; Raynaud Disease ; Pulmonary Fibrosis ; Hypertension, Pulmonary ; Edema ; Rheumatic Diseases ; Scleroderma, Systemic ; Scleroderma, Diffuse

PURPOSE: The aim of this article is to review a group of lesions associated with periodontal ligament (PDL) widening. MATERIALS AND METHODS: An electronic search was performed using specialized databases such as Google Scholar, PubMed, PubMed Central, Science Direct, and Scopus to find relevant studies by using keywords such as “periodontium”, “periodontal ligament”, “periodontal ligament space”, “widened periodontal ligament”, and “periodontal ligament widening”. RESULTS: Out of nearly 200 articles, about 60 were broadly relevant to the topic. Ultimately, 47 articles closely related to the topic of interest were reviewed. When the relevant data were compiled, the following 10 entities were identified: occlusal/orthodontic trauma, periodontal disease/periodontitis, pulpo-periapical lesions, osteosarcoma, chondrosarcoma, non-Hodgkin lymphoma, progressive systemic sclerosis, radiation-induced bone defect, bisphosphonate-related osteonecrosis, and osteomyelitis. CONCLUSION: Although PDL widening may be encountered by many dentists during their routine daily procedures, the clinician should consider some serious related conditions as well.
Chondrosarcoma ; Dentists ; Humans ; Ligaments ; Lymphoma, Non-Hodgkin ; Osteomyelitis ; Osteonecrosis ; Osteosarcoma ; Periapical Periodontitis ; Periodontal Ligament* ; Radiotherapy ; Scleroderma, Diffuse

Extensive tumoral calcinosis affecting a large joint is uncommon in patients with systemic sclerosis. We report the case of a 52-year-old female patient referred for a growing calcified mass in the shoulder. She was diagnosed with interstitial lung disease and progressive systemic sclerosis. Although the pain and disability associated with the affected joint was not severe, the patient underwent surgical excision because the mass continued to grow and was likely to produce shoulder dysfunction and skin ulceration. The patient appeared well 10 months after surgery with no signs of recurrence. This report highlights the timing and indication of surgical excision in similar cases.
Calcinosis* ; Female ; Humans ; Joints ; Lung Diseases, Interstitial ; Middle Aged ; Quality of Life ; Recurrence ; Scleroderma, Diffuse ; Scleroderma, Systemic* ; Shoulder ; Skin Ulcer

Animals ; Cannabinoid Receptor Antagonists ; therapeutic use ; Cannabinoids ; pharmacology ; Fibrosis ; metabolism ; Humans ; Liver Cirrhosis ; etiology ; metabolism ; therapy ; Piperidines ; therapeutic use ; Pyrazoles ; therapeutic use ; Receptor, Cannabinoid, CB1 ; metabolism ; Receptor, Cannabinoid, CB2 ; metabolism ; Receptors, Cannabinoid ; metabolism ; Scleroderma, Diffuse ; metabolism ; Signal Transduction ; drug effects ; Skin ; metabolism ; Smad Proteins ; metabolism ; Transforming Growth Factor beta1 ; metabolism

Systemic sclerosis is often associated with other autoimmune diseases such as autoimmune thyroiditis, primary Sjogren syndrome, dermatomyositis and autoimmune hepatitis, as the so-called scleroderma overlap syndrome. However, coexistence of scleroderma and discoid lupus erythematosus (DLE) is extremely rare. Association of scleroderma and DLE has not been described definitely, but it is suggested that various factors such as genetic factor, sex hormone, environmental factor, immunologic factor, viral infection and HLA typing may contribute. A 40-year-old woman presented with an 18-year history of Raynaud's phenomenon and generalized hypopigmented sclerotic patches over the entire body. Skin biopsy and other examinations showed characteristics of scleroderma. The diagnosis was diffuse systemic sclerosis. During treatment, erythematous and erosive patches and plaques on face and arms appeared. The clinical features, histopathologic presentation, and laboratory findings were consistent with DLE.
Adult ; Arm ; Autoimmune Diseases ; Biopsy ; Dermatomyositis ; Female ; Hepatitis, Autoimmune ; Histocompatibility Testing ; Humans ; Lupus Erythematosus, Discoid ; Scleroderma, Diffuse ; Scleroderma, Systemic ; Sex Factors ; Sjogren's Syndrome ; Skin ; Thyroiditis, Autoimmune

Autoimmune polyendocrinopathy syndrome is a heterogeneous group of rare diseases characterized by autoimmune activity against more than one endocrine organ, although non-endocrine organs can be affected. We present a case of autoimmune polyendocrinopathy syndrome type 2 in a 42-year-old woman with Guillain-Barre syndrome and scleroderma. This combination of syndromes has not been reported and warrants further investigation.
Adult ; Female ; Guillain-Barre Syndrome ; complications ; diagnosis ; Humans ; Polyendocrinopathies, Autoimmune ; diagnosis ; Scleroderma, Diffuse ; complications ; diagnosis

OBJECTIVETo investigate the effects of ethanolic extract from Capparis spinosa (ECS) on the fibroblast proliferation and type I collagen production in normal and progressive systemic sclerosis (PSS).

METHODCellular activity was determined by the MTT method. Apoptosis was detected by flow cytometry analysis of Annexin V-stained cells. The expression levels of type I collagen messenger RNA and protein were analyzed by RT-PCR and western blot analysis.

RESULTECS could significantly inhibit the proliferation of fibroblast and reduced the expression of alpha2 (I) collagen mRNA and type I collagen protein in PSS in a dose-and time-dependent manner. ECS did not affect the proliferation of fibroblast and expression of type I collagen mRNA and protein in normal human. ECS could counteract the harmful effects on fibroblast by H2O2.

CONCLUSIONECS can effectively inhibit the fibroblast proliferation and type I collagen production in PSS.

Capparis ; chemistry ; Cell Proliferation ; drug effects ; Cells, Cultured ; Collagen Type I ; metabolism ; Drugs, Chinese Herbal ; pharmacology ; Fibroblasts ; cytology ; drug effects ; Humans ; Scleroderma, Diffuse ; drug therapy ; metabolism ; pathology