DCSSC
MCID: DFF035
MIFTS: 50

Diffuse Cutaneous Systemic Sclerosis (DCSSC)

Categories: Bone diseases, Nephrological diseases, Rare diseases, Respiratory diseases, Skin diseases

Aliases & Classifications for Diffuse Cutaneous Systemic Sclerosis

MalaCards integrated aliases for Diffuse Cutaneous Systemic Sclerosis:

Name: Diffuse Cutaneous Systemic Sclerosis 53 59
Progressive Cutaneous Systemic Scleroderma 53 59
Progressive Cutaneous Systemic Sclerosis 53 59
Diffuse Cutaneous Systemic Scleroderma 53 59
Diffuse Scleroderma 73
Dcssc 53

Characteristics:

Orphanet epidemiological data:

59
diffuse cutaneous systemic sclerosis
Inheritance: Multigenic/multifactorial,Not applicable; Age of onset: Adult; Age of death: adult;

Classifications:



Summaries for Diffuse Cutaneous Systemic Sclerosis

NIH Rare Diseases : 53 Diffuse cutaneous systemic sclerosis (dcSSc) is a subtype of systemic scleroderma (systemic sclerosis) characterized by skin hardening (fibrosis) and problems in many organs of the body. The disease can occur at any age but mainly affects people between 40 and 50 years of age. Symptoms include Raynaud�??s phenomenon; skin fibrosis beginning on the fingers and face that rapidly becomes generalized; "spider veins" (telangiectasias) on the thorax, face, lips, tongue, and fingers; gastroesophageal reflux; and difficulty eating (dysphagia) along with weight loss, vomiting, diarrhea or constipation. Dry mouth and dental involvement can occur. Joint pain (arthralgias), muscular pain, weakness, cramps, and destruction of the tips of the fingers or toes (acroosteolysis) are frequent. Severe problems involving the lung (fibrosis or pulmonary hypertension) and kidney problems may also occur. The exact cause of the condition is unknown. There is currently no cure. Treatment depends of the symptoms, but may include medication and surgery.

MalaCards based summary : Diffuse Cutaneous Systemic Sclerosis, also known as progressive cutaneous systemic scleroderma, is related to arthritis and limited scleroderma. An important gene associated with Diffuse Cutaneous Systemic Sclerosis is IRF5 (Interferon Regulatory Factor 5), and among its related pathways/superpathways are Innate Immune System and NF-kappaB Signaling. The drugs Iloprost and Acetylcysteine have been mentioned in the context of this disorder. Affiliated tissues include skin, lung and kidney, and related phenotypes are muscle weakness and dysphagia

Related Diseases for Diffuse Cutaneous Systemic Sclerosis

Graphical network of the top 20 diseases related to Diffuse Cutaneous Systemic Sclerosis:



Diseases related to Diffuse Cutaneous Systemic Sclerosis

Symptoms & Phenotypes for Diffuse Cutaneous Systemic Sclerosis

Human phenotypes related to Diffuse Cutaneous Systemic Sclerosis:

59 32 (show all 26)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 muscle weakness 59 32 frequent (33%) Frequent (79-30%) HP:0001324
2 dysphagia 59 32 frequent (33%) Frequent (79-30%) HP:0002015
3 nausea and vomiting 59 32 occasional (7.5%) Occasional (29-5%) HP:0002017
4 arthritis 59 32 frequent (33%) Frequent (79-30%) HP:0001369
5 carious teeth 59 32 frequent (33%) Frequent (79-30%) HP:0000670
6 malabsorption 59 32 frequent (33%) Frequent (79-30%) HP:0002024
7 flexion contracture 59 32 frequent (33%) Frequent (79-30%) HP:0001371
8 gastroesophageal reflux 59 32 hallmark (90%) Very frequent (99-80%) HP:0002020
9 renal insufficiency 59 32 occasional (7.5%) Occasional (29-5%) HP:0000083
10 pulmonary fibrosis 59 32 frequent (33%) Frequent (79-30%) HP:0002206
11 arthralgia 59 32 frequent (33%) Frequent (79-30%) HP:0002829
12 dyspnea 59 32 hallmark (90%) Very frequent (99-80%) HP:0002094
13 pulmonary arterial hypertension 59 32 occasional (7.5%) Occasional (29-5%) HP:0002092
14 congestive heart failure 59 32 occasional (7.5%) Occasional (29-5%) HP:0001635
15 autoimmunity 59 32 hallmark (90%) Very frequent (99-80%) HP:0002960
16 skin ulcer 59 32 frequent (33%) Frequent (79-30%) HP:0200042
17 telangiectasia of the skin 59 32 frequent (33%) Frequent (79-30%) HP:0100585
18 xerostomia 59 32 frequent (33%) Frequent (79-30%) HP:0000217
19 hypertensive crisis 59 32 occasional (7.5%) Occasional (29-5%) HP:0100735
20 osteolysis 59 32 frequent (33%) Frequent (79-30%) HP:0002797
21 dyspareunia 59 32 frequent (33%) Frequent (79-30%) HP:0030016
22 pulmonary infiltrates 59 32 hallmark (90%) Very frequent (99-80%) HP:0002113
23 oliguria 59 32 hallmark (90%) Very frequent (99-80%) HP:0100520
24 narrow foramen obturatorium 59 32 hallmark (90%) Very frequent (99-80%) HP:0100958
25 abnormal bowel sounds 59 32 occasional (7.5%) Occasional (29-5%) HP:0030142
26 abnormality of the skin 59 Very frequent (99-80%)

MGI Mouse Phenotypes related to Diffuse Cutaneous Systemic Sclerosis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.9 BANK1 CAV1 CCR6 FBN1 ICOS IRF5
2 homeostasis/metabolism MP:0005376 9.73 CAV1 CCN2 CCR6 COMP FBN1 ICOS
3 immune system MP:0005387 9.4 BANK1 CAV1 CCR6 COMP FBN1 ICOS

Drugs & Therapeutics for Diffuse Cutaneous Systemic Sclerosis

Drugs for Diffuse Cutaneous Systemic Sclerosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 106)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Iloprost Approved, Investigational Phase 2, Phase 3 78919-13-8 6443959
2
Acetylcysteine Approved, Investigational Phase 2, Phase 3 616-91-1 12035
3
Methocarbamol Approved, Vet_approved Phase 3,Phase 2 532-03-6 4107
4
Cyclophosphamide Approved, Investigational Phase 3,Phase 2,Phase 1 6055-19-2, 50-18-0 2907
5
Lenograstim Approved, Investigational Phase 3,Phase 2 135968-09-1
6 Antiviral Agents Phase 2, Phase 3
7 Vasodilator Agents Phase 2, Phase 3
8 Platelet Aggregation Inhibitors Phase 2, Phase 3
9 Antioxidants Phase 2, Phase 3
10 N-monoacetylcystine Phase 2, Phase 3
11 Free Radical Scavengers Phase 2, Phase 3
12 Expectorants Phase 2, Phase 3
13 Respiratory System Agents Phase 2, Phase 3
14 Anti-Infective Agents Phase 2, Phase 3,Phase 1
15 Protective Agents Phase 2, Phase 3
16 Antidotes Phase 2, Phase 3
17 Neuromuscular Agents Phase 3,Phase 2
18 Peripheral Nervous System Agents Phase 3,Phase 2
19 Immunosuppressive Agents Phase 3,Phase 2,Phase 1
20 Alkylating Agents Phase 3,Phase 2,Phase 1
21 Immunologic Factors Phase 3,Phase 2,Phase 1,Not Applicable
22 Antirheumatic Agents Phase 3,Phase 2,Phase 1
23 Antineoplastic Agents, Alkylating Phase 3,Phase 2,Phase 1
24
Abatacept Approved Phase 2,Phase 1 332348-12-6 10237
25
Mycophenolic acid Approved Phase 2,Phase 1 24280-93-1 446541
26
belimumab Approved Phase 2 356547-88-1 5957 10451420
27
leucovorin Approved Phase 1, Phase 2 58-05-9 6006 143
28
Miconazole Approved, Investigational, Vet_approved Phase 1, Phase 2 22916-47-8 4189
29
Everolimus Approved Phase 1, Phase 2 159351-69-6 6442177
30
Sirolimus Approved, Investigational Phase 1, Phase 2 53123-88-9 46835353 6436030 5284616
31
Methotrexate Approved Phase 1, Phase 2,Phase 2 59-05-2, 1959-05-2 126941
32
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
33
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5
34
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
35
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
36
Thiotepa Approved, Investigational Phase 2 52-24-4 5453
37
rituximab Approved Phase 2 174722-31-7 10201696
38
Mesna Approved, Investigational Phase 2 3375-50-6 598
39
alemtuzumab Approved, Investigational Phase 2,Phase 1 216503-57-0
40
Tofacitinib Approved, Investigational Phase 1, Phase 2 477600-75-2
41
Azathioprine Approved Phase 2 446-86-6 2265
42
Folic Acid Approved, Nutraceutical, Vet_approved Phase 1, Phase 2 59-30-3 6037
43
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7
44 Ifetroban Investigational Phase 2 143443-90-7
45 Antitubercular Agents Phase 2,Phase 1
46 Antibodies Phase 2,Phase 1,Not Applicable
47 Immunoglobulins Phase 2,Phase 1,Not Applicable
48 Antibiotics, Antitubercular Phase 2,Phase 1
49 Anti-Bacterial Agents Phase 2,Phase 1
50 Antibodies, Monoclonal Phase 2,Phase 1

Interventional clinical trials:

(show all 49)
# Name Status NCT ID Phase Drugs
1 Serial Night Time Position Splint on Systemic Sclerosis Unknown status NCT01586663 Phase 3 Drug treatment
2 High Dose Intravenous N-Acetylcysteine Versus Iloprost for Early, Rapidly Progressive Diffuse Systemic Sclerosis Unknown status NCT00428883 Phase 2, Phase 3 N-acetylcysteine (NAC)
3 Recombinant Human Relaxin in the Treatment of Diffuse Scleroderma Completed NCT00704665 Phase 3 Relaxin;Relaxin
4 High Dose Cyclophosphamide for Treatment of Scleroderma Completed NCT00501995 Phase 3 IV Cyclophosphamide
5 Trial to Evaluate Efficacy and Safety of Lenabasum in Diffuse Cutaneous Systemic Sclerosis Recruiting NCT03398837 Phase 3 Lenabasum 5 mg;Lenabasum 20 mg
6 AIMSPRO in Established Diffuse Cutaneous Systemic Sclerosis Unknown status NCT00769028 Phase 2 Hyperimmune caprine serum;Albumin
7 A Study of Subcutaneous Abatacept to Treat Diffuse Cutaneous Systemic Sclerosis Completed NCT02161406 Phase 2 Abatacept;Placebo
8 Belimumab for the Treatment of Diffuse Cutaneous Systemic Sclerosis Completed NCT01670565 Phase 2 Belimumab
9 Proof-of-concept Trial of IVA337 in Diffuse Cutaneous Systemic Sclerosis Completed NCT02503644 Phase 2 IVA337;Placebo
10 Safety, Tolerability, and Pharmacokinetics of CAT-192 (Human Anti-TGF-Beta1 Monoclonal Antibody) in Patients With Early Stage Diffuse Systemic Sclerosis Completed NCT00043706 Phase 1, Phase 2 Human Anti-Transforming Growth Factor Beta-1 Monoclonal Antibody
11 Proof of Biological Activity of SAR100842 in Systemic Sclerosis Completed NCT01651143 Phase 2 SAR100842;Placebo (for SAR100842)
12 Phase II Study of Recombinant Relaxin for Progressive Systemic Sclerosis Completed NCT00004380 Phase 2 relaxin
13 Phase II Pilot Study of Cyclophosphamide and Rabbit Anti-Thymocyte Globulin as Salvage Therapy in Patients With Diffuse Systemic Sclerosis Completed NCT00016458 Phase 2 anti-thymocyte globulin;cyclophosphamide
14 A Study to Evaluate the Safety and Efficacy of Abatacept in Patients With Diffuse Systemic Sclerosis (Scleroderma) Completed NCT00442611 Phase 1, Phase 2 Abatacept;Placebo
15 Oral Type I Collagen for Relieving Scleroderma Completed NCT00005675 Phase 2 Oral bovine type I collagen;Placebo
16 IL1-TRAP, Rilonacept, in Systemic Sclerosis Completed NCT01538719 Phase 1, Phase 2 Rilonacept
17 Rapamycin vs Methotrexate in Diffuse SSc Completed NCT00241189 Phase 1, Phase 2 rapamycin;methotrexate
18 Prednisolone in Early Diffuse Systemic Sclerosis Recruiting NCT03708718 Phase 2 Prednisolone 5 mg;Placebo oral capsule
19 Brentuximab Vedotin for Systemic Sclerosis Recruiting NCT03222492 Phase 1, Phase 2
20 Atorvastatin for Microvascular Endothelial Function and Raynaud in Early Diffuse Scleroderma Recruiting NCT02370784 Phase 2 atorvastatin;Placebo
21 Proof of Mechanism Study of GSK2330811 in Diffuse Cutaneous Systemic Sclerosis Recruiting NCT03041025 Phase 1, Phase 2 GSK2330811;Placebo
22 Oral Ifetroban to Treat Diffuse Cutaneous Systemic Sclerosis (SSc) or SSc-associated Pulmonary Arterial Hypertension Recruiting NCT02682511 Phase 2 Oral Ifetroban;Oral Placebo
23 Autologous Stem Cell Transplantation for Progressive Systemic Sclerosis Recruiting NCT01895244 Phase 2 Autologous stemcell transplantation with CD (cluster of differentiation) 34 selected stem cells
24 Autologous Stem Cell Transplantation in Patients With Systemic Sclerosis Recruiting NCT03630211 Phase 2 Cyclophosphamide;Mesna;Rituximab;Alemtuzumab;Thiotepa;GM-CSF
25 Safety, Tolerability, Efficacy, and Pharmacokinetics of JBT-101 in Systemic Sclerosis Active, not recruiting NCT02465437 Phase 2 JBT-101;Placebo;Part B Open-Label Extension
26 Evaluation of Tofacitinib in Early Diffuse Cutaneous Systemic Sclerosis (dcSSc) Active, not recruiting NCT03274076 Phase 1, Phase 2 Tofacitinib;Placebo Oral Tablet
27 Effectiveness and Safety of SAR156597 in Treating Diffuse Systemic Sclerosis Active, not recruiting NCT02921971 Phase 2 SAR156597 (ACT14604);Placebo
28 Efficacy and Safety of Riociguat in Patients With Systemic Sclerosis Active, not recruiting NCT02283762 Phase 2 Riociguat (Adempas, BAY63-2521);Placebo
29 Brentuximab Vedotin in Early Diffuse Cutaneous Systemic Sclerosis Not yet recruiting NCT03198689 Phase 2 Brentuximab Vedotin
30 Assessment of the Efficacy and Tolerance of Sub-cutaneous Re-injection of Autologous Adipose-derived REGEnerative Cells in the Local Treatment of Neuropathic Diabetic Foot ulcERs Not yet recruiting NCT02866565 Phase 2 adipose-derived regenerative cells (ADRCs)
31 Proof of Concept Trial of Gleevec (Imatinib) in Active Diffuse Scleroderma Terminated NCT01545427 Phase 2 Imatinib mesylate
32 A Double-Blinded Study to Evaluate the Safety, Tolerability, and Efficacy of BMS-986020 Versus Placebo in Diffuse Cutaneous Systemic Sclerosis (dcSSc) Withdrawn NCT02588625 Phase 2 BMS-986020
33 Mycophenolate Mofetil in Systemic Sclerosis Completed NCT00433186 Phase 1 Mycophenolate Mofetil
34 Fresolimumab In Systemic Sclerosis Completed NCT01284322 Phase 1 Fresolimumab
35 Thalidomide for Decreasing Collagen Biosynthesis in People With Progressive Systemic Sclerosis Terminated NCT00418132 Phase 1 Thalidomide;Placebo thalidomide
36 Nonmyeloablative Allo Stem Cell Transplant for Severe Autoimmune Diseases Terminated NCT00849745 Phase 1
37 Autologous SCT for Progressive Systemic Sclerosis: a Prospective NIS Study Unknown status NCT02264405
38 Treatment Outcome in Early Diffuse Cutaneous Systemic Sclerosis Completed NCT02339441 Methotrexate;Mycophenolate mofetil;Cyclophosphamide
39 Study of Ambrisentan With Antifibrotic Agent Combination Therapy in Diffuse Systemic Sclerosis Completed NCT01093885 Not Applicable Ambrisentan
40 EBMT ADWP Prospective Non Interventional Study : AutoHSCT in SSc Patients Completed NCT02516124
41 A Non-Interventional Pilot Study Assessing Whether Lysyl Oxidase-like 2 (LOXL2) is Present in Subjects With Scleroderma Completed NCT01881529
42 Skin Biopsy Specimens as Biomarkers of Systemic Sclerosis and Response to Mycophenolate Mofetil Completed NCT00853788
43 Idiopathic Pulmonary Fibrosis--Pathogenesis and Staging - SCOR in Occupational and Immunological Lung Diseases Completed NCT00005317
44 Angiogenic/Angiostatic Mediators in Patients With Systemic Sclerosis Completed NCT00668473
45 Stimulatory Autoantibodies to the Platelet-Derived Growth Factor Receptor (PDGFR) in Patients With Systemic Sclerosis Completed NCT00667134
46 Itch and Clinacal Caracteristics in Systemic Sclerosis Completed NCT03297580
47 IVIG Treatment in Systemic Sclerosis Active, not recruiting NCT01785056 Not Applicable
48 The Effects of Exercise in Patients With Systemic Sclerosis Active, not recruiting NCT03058887 Not Applicable
49 Systemic Sclerosis Clinical and Biomarker Study Withdrawn NCT02531009

Search NIH Clinical Center for Diffuse Cutaneous Systemic Sclerosis

Genetic Tests for Diffuse Cutaneous Systemic Sclerosis

Anatomical Context for Diffuse Cutaneous Systemic Sclerosis

MalaCards organs/tissues related to Diffuse Cutaneous Systemic Sclerosis:

41
Skin, Lung, Kidney, Tongue, Bone, Heart, Myeloid

Publications for Diffuse Cutaneous Systemic Sclerosis

Articles related to Diffuse Cutaneous Systemic Sclerosis:

(show top 50) (show all 100)
# Title Authors Year
1
Patient acceptable symptom state in scleroderma: results from the tocilizumab compared with placebo trial in active diffuse cutaneous systemic sclerosis. ( 29077900 )
2018
2
Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study. ( 29207002 )
2018
3
Factors influencing early referral, early diagnosis and management in patients with diffuse cutaneous systemic sclerosis. ( 29415230 )
2018
4
Skin Gene Expression Is Prognostic for the Trajectory of Skin Disease in Patients With Diffuse Cutaneous Systemic Sclerosis. ( 29858547 )
2018
5
Lysophosphatidic Acid Receptor 1 Antagonist SAR100842 for Patients With Diffuse Cutaneous Systemic Sclerosis: A Double-Blind, Randomized, Eight-Week Placebo-Controlled Study Followed by a Sixteen-Week Open-Label Extension Study. ( 29732731 )
2018
6
UVA1 for diffuse cutaneous systemic sclerosis in a Fitzpatrick skin type VI patient: outcomes in the modified Rodnan skin score. ( 28375193 )
2017
7
Successful treatment of continuous intra-arterial administration of prostaglandin E1, urokinase and heparin for intractable digital ulcers by upper extremity arterial occlusion in diffuse cutaneous systemic sclerosis patient. ( 28691232 )
2017
8
A preliminary study of skin ultrasound in diffuse cutaneous systemic sclerosis: Does skin echogenicity matter? ( 28339492 )
2017
9
An MIF Promoter Polymorphism Is Associated with Susceptibility to Pulmonary Arterial Hypertension in Diffuse Cutaneous Systemic Sclerosis. ( 28668810 )
2017
10
Clinical efficacy and safety of bathing with Chinese medicine Taohong Siwu Decoction () for treatment of diffuse cutaneous systemic sclerosis: A randomized, placebo-controlled trial. ( 28197938 )
2017
11
Proteomic Analysis of Sera from Individuals with Diffuse Cutaneous Systemic Sclerosis Reveals a Multianalyte Signature Associated with Clinical Improvement during Imatinib Mesylate Treatment. ( 28298564 )
2017
12
Two years follow-up of an open-label pilot study of treatment with rituximab in patients with early diffuse cutaneous systemic sclerosis. ( 28891418 )
2017
13
Clinical utility of hypo- and hyperpigmentation of skin in diffuse cutaneous systemic sclerosis. ( 28261995 )
2017
14
Reliability and Validity of the Tender and Swollen Joint Counts and the Modified Rodnan Skin Score in Early Diffuse Cutaneous Systemic Sclerosis: Analysis from the Prospective Registry of Early Systemic Sclerosis Cohort. ( 28298560 )
2017
15
Treatment outcome in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study (ESOS). ( 28188239 )
2017
16
Replacement myocardial fibrosis at the site of late gadolinium enhancement on magnetic resonance imaging in a patient with diffuse cutaneous systemic sclerosis: An autopsy report. ( 30279795 )
2017
17
Watermelon stomach and colon in a patient with diffuse cutaneous systemic sclerosis. ( 27414211 )
2016
18
The American College of Rheumatology Provisional Composite Response Index for Clinical Trials in Early Diffuse Cutaneous Systemic Sclerosis. ( 26808827 )
2016
19
Whole Exome Sequencing for Identification of Potential Causal Variants for Diffuse Cutaneous Systemic Sclerosis. ( 27111861 )
2016
20
The American College of Rheumatology Provisional Composite Response Index for Clinical Trials in Early Diffuse Cutaneous Systemic Sclerosis. ( 26806474 )
2016
21
Comments on: "Domsic, Robyn T., et al. "Derivation and External Validation of a Prediction Rule for Five-Year Mortality in Patients With Early Diffuse Cutaneous Systemic Sclerosis." Arthritis & Rheumatology 68.4 (2016): 993-1003." ( 27588383 )
2016
22
Classical case of diffuse cutaneous systemic sclerosis. ( 27728683 )
2016
23
Perivascular Cells in Diffuse Cutaneous Systemic Sclerosis Overexpress Activated ADAM12 and Are Involved in Myofibroblast Transdifferentiation and Development of Fibrosis. ( 27252423 )
2016
24
Trigeminal neuropathy responding to systemic corticosteroids and methotrexate in a patient with diffuse cutaneous systemic sclerosis. ( 27748258 )
2016
25
RISE-SSc: Riociguat in diffuse cutaneous systemic sclerosis. ( 27746061 )
2016
26
Prediction of improvement in skin fibrosis in diffuse cutaneous systemic sclerosis: a EUSTAR analysis. ( 27016052 )
2016
27
An easy prediction rule for diffuse cutaneous systemic sclerosis using only the timing and type of first symptoms and auto-antibodies: derivation and validation. ( 27550299 )
2016
28
Prediction of worsening of skin fibrosis in patients with diffuse cutaneous systemic sclerosis using the EUSTAR database. ( 24981642 )
2015
29
Rituximab in diffuse cutaneous systemic sclerosis: should we be using it today? ( 25573841 )
2015
30
A Longitudinal Biomarker for the Extent of Skin Disease in Patients With Diffuse Cutaneous Systemic Sclerosis. ( 26240058 )
2015
31
Intravenous immunoglobulin may be an effective therapy for refractory, active diffuse cutaneous systemic sclerosis. ( 25433527 )
2015
32
Disease progression in systemic sclerosis-overlap syndrome is significantly different from limited and diffuse cutaneous systemic sclerosis. ( 24389298 )
2015
33
A preliminary study of acoustic radiation force impulse quantification for the assessment of skin in diffuse cutaneous systemic sclerosis. ( 25593239 )
2015
34
Reliability, validity and responsiveness to change of the Saint George's Respiratory Questionnaire in early diffuse cutaneous systemic sclerosis. ( 25667436 )
2015
35
Gene expression changes reflect clinical response in a placebo-controlled randomized trial of abatacept in patients with diffuse cutaneous systemic sclerosis. ( 26071192 )
2015
36
Pityriasis rubra pilaris-like eruption with dermatomyositis and diffuse cutaneous systemic sclerosis overlap. ( 25758527 )
2015
37
High reactive oxygen species in fibrotic and nonfibrotic skin of patients with diffuse cutaneous systemic sclerosis. ( 26143738 )
2015
38
Derivation and external validation of a 5-year mortality prediction rule for patients with early diffuse cutaneous systemic sclerosis. ( 26554637 )
2015
39
Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. ( 25058083 )
2014
40
Imatinib mesylate (GleevecA9) in the treatment of diffuse cutaneous systemic sclerosis: results of a 24-month open label, extension phase, single-centre trial. ( 25152211 )
2014
41
Serum levels of soluble carbonic anhydrase IX are decreased in patients with diffuse cutaneous systemic sclerosis compared to those with limited cutaneous systemic sclerosis. ( 25030848 )
2014
42
Derivation and validation of a prediction rule for two-year mortality in early diffuse cutaneous systemic sclerosis. ( 24470430 )
2014
43
A patient with diffuse cutaneous systemic sclerosis complicated by antineutrophil-cytoplasmic antibody-associated vasculitis exhibiting honeycomb lung without volume loss. ( 24694501 )
2014
44
Increased expression of S100A8 and S100A9 in patients with diffuse cutaneous systemic sclerosis. A correlation with organ involvement and immunological abnormalities. ( 23754244 )
2013
45
Increased production of soluble inducible costimulator in patients with diffuse cutaneous systemic sclerosis. ( 23053221 )
2013
46
A randomized, double-blind, placebo-controlled trial: intravenous immunoglobulin treatment in patients with diffuse cutaneous systemic sclerosis. ( 23910617 )
2013
47
Case of diffuse cutaneous systemic sclerosis with anti-Ku and anti-centromere antibodies. ( 23663134 )
2013
48
Augmented ICOS expression in patients with early diffuse cutaneous systemic sclerosis. ( 23024058 )
2013
49
Significance of palpable tendon friction rubs in early diffuse cutaneous systemic sclerosis. ( 23371412 )
2013
50
A possible contribution of visfatin to the resolution of skin sclerosis in patients with diffuse cutaneous systemic sclerosis via a direct anti-fibrotic effect on dermal fibroblasts and Th1 polarization of the immune response. ( 23443327 )
2013

Variations for Diffuse Cutaneous Systemic Sclerosis

Expression for Diffuse Cutaneous Systemic Sclerosis

Search GEO for disease gene expression data for Diffuse Cutaneous Systemic Sclerosis.

Pathways for Diffuse Cutaneous Systemic Sclerosis

GO Terms for Diffuse Cutaneous Systemic Sclerosis

Cellular components related to Diffuse Cutaneous Systemic Sclerosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.81 CCN2 COL22A1 COMP FBN1 ICOS MIF
2 external side of plasma membrane GO:0009897 9.35 CCR6 HLA-DRB1 ICOS ICOSLG THBS1
3 extracellular matrix GO:0031012 9.02 CCN2 COL22A1 COMP FBN1 THBS1

Biological processes related to Diffuse Cutaneous Systemic Sclerosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 immune system process GO:0002376 9.65 HLA-DRB1 ICOSLG IFI16 IRF5 MIF
2 inflammatory response GO:0006954 9.62 IFI16 MIF SIGLEC1 THBS1
3 B cell activation GO:0042113 9.4 BANK1 ICOSLG
4 positive regulation of phosphorylation GO:0042327 9.37 MIF THBS1
5 extracellular matrix organization GO:0030198 9.26 COL22A1 COMP FBN1 THBS1
6 growth plate cartilage development GO:0003417 9.16 COMP THBS1
7 T cell costimulation GO:0031295 8.8 CAV1 ICOS ICOSLG

Molecular functions related to Diffuse Cutaneous Systemic Sclerosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.8 CAV1 FBN1 ICOSLG IRF5 MIF STAT4
2 signaling receptor binding GO:0005102 9.67 BANK1 CAV1 ICOSLG MIF
3 integrin binding GO:0005178 9.5 CCN2 FBN1 THBS1
4 heparin binding GO:0008201 9.46 CCN2 COMP FBN1 THBS1
5 extracellular matrix structural constituent GO:0005201 9.26 COL22A1 COMP FBN1 THBS1
6 fibronectin binding GO:0001968 8.8 CCN2 COMP THBS1

Sources for Diffuse Cutaneous Systemic Sclerosis

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