MCID: DFF035
MIFTS: 52

Diffuse Cutaneous Systemic Sclerosis

Categories: Rare diseases, Nephrological diseases, Respiratory diseases, Bone 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 scleroderma, familial progressive and arthritis. 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 tongue, and related phenotypes are renal insufficiency and xerostomia

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 renal insufficiency 59 32 occasional (7.5%) Occasional (29-5%) HP:0000083
2 xerostomia 59 32 frequent (33%) Frequent (79-30%) HP:0000217
3 carious teeth 59 32 frequent (33%) Frequent (79-30%) HP:0000670
4 muscle weakness 59 32 frequent (33%) Frequent (79-30%) HP:0001324
5 arthritis 59 32 frequent (33%) Frequent (79-30%) HP:0001369
6 flexion contracture 59 32 frequent (33%) Frequent (79-30%) HP:0001371
7 congestive heart failure 59 32 occasional (7.5%) Occasional (29-5%) HP:0001635
8 dysphagia 59 32 frequent (33%) Frequent (79-30%) HP:0002015
9 nausea and vomiting 59 32 occasional (7.5%) Occasional (29-5%) HP:0002017
10 gastroesophageal reflux 59 32 hallmark (90%) Very frequent (99-80%) HP:0002020
11 malabsorption 59 32 frequent (33%) Frequent (79-30%) HP:0002024
12 pulmonary arterial hypertension 59 32 occasional (7.5%) Occasional (29-5%) HP:0002092
13 dyspnea 59 32 hallmark (90%) Very frequent (99-80%) HP:0002094
14 pulmonary infiltrates 59 32 hallmark (90%) Very frequent (99-80%) HP:0002113
15 pulmonary fibrosis 59 32 frequent (33%) Frequent (79-30%) HP:0002206
16 osteolysis 59 32 frequent (33%) Frequent (79-30%) HP:0002797
17 arthralgia 59 32 frequent (33%) Frequent (79-30%) HP:0002829
18 autoimmunity 59 32 hallmark (90%) Very frequent (99-80%) HP:0002960
19 dyspareunia 59 32 frequent (33%) Frequent (79-30%) HP:0030016
20 abnormal bowel sounds 59 32 occasional (7.5%) Occasional (29-5%) HP:0030142
21 oliguria 59 32 hallmark (90%) Very frequent (99-80%) HP:0100520
22 telangiectasia of the skin 59 32 frequent (33%) Frequent (79-30%) HP:0100585
23 hypertensive crisis 59 32 occasional (7.5%) Occasional (29-5%) HP:0100735
24 narrow foramen obturatorium 59 32 hallmark (90%) Very frequent (99-80%) HP:0100958
25 skin ulcer 59 32 frequent (33%) Frequent (79-30%) HP:0200042
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 IRF5 MIF SIGLEC1 STAT4 BANK1 THBS1
2 homeostasis/metabolism MP:0005376 9.73 IRF5 MIF SIGLEC1 STAT4 THBS1 CAV1
3 immune system MP:0005387 9.4 MIF SIGLEC1 STAT4 BANK1 THBS1 CAV1

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 85)
# 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
Lenograstim Approved, Investigational Phase 3 135968-09-1
4
Cyclophosphamide Approved, Investigational Phase 3,Phase 2,Phase 1 50-18-0, 6055-19-2 2907
5
Methocarbamol Approved, Vet_approved Phase 3,Phase 2 532-03-6 4107
6 Respiratory System Agents Phase 2, Phase 3
7 N-monoacetylcystine Phase 2, Phase 3
8 Vasodilator Agents Phase 2, Phase 3
9 Platelet Aggregation Inhibitors Phase 2, Phase 3
10 Expectorants Phase 2, Phase 3
11 Antidotes Phase 2, Phase 3
12 Anti-Infective Agents Phase 2, Phase 3,Phase 1
13 Antioxidants Phase 2, Phase 3
14 Protective Agents Phase 2, Phase 3
15 Antiviral Agents Phase 2, Phase 3
16 Alkylating Agents Phase 3,Phase 2,Phase 1
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 Antirheumatic Agents Phase 3,Phase 2,Phase 1
21 Antineoplastic Agents, Alkylating Phase 3,Phase 2,Phase 1
22
Abatacept Approved Phase 1, Phase 2,Phase 2 332348-12-6 10237
23
Methotrexate Approved Phase 1, Phase 2,Phase 2 1959-05-2, 59-05-2 126941
24
Miconazole Approved, Investigational, Vet_approved Phase 1, Phase 2 22916-47-8 4189
25
Everolimus Approved Phase 1, Phase 2 159351-69-6 6442177
26
Mycophenolic acid Approved Phase 2,Phase 1 24280-93-1 446541
27
Mycophenolate mofetil Approved, Investigational Phase 2,Phase 1 128794-94-5 5281078
28
belimumab Approved Phase 2 356547-88-1 5957 10451420
29
Sirolimus Approved, Investigational Phase 1, Phase 2 53123-88-9 5284616 6436030 46835353
30
Thiotepa Approved, Investigational Phase 2 52-24-4 5453
31
Tofacitinib Approved, Investigational Phase 1, Phase 2 477600-75-2
32
Azathioprine Approved Phase 2 446-86-6 2265
33
leucovorin Approved, Nutraceutical Phase 1, Phase 2 58-05-9 143 6006
34
Folic Acid Approved, Nutraceutical, Vet_approved Phase 1, Phase 2 59-30-3 6037
35 Ifetroban Investigational Phase 2 143443-90-7
36 Folic Acid Antagonists Phase 1, Phase 2
37 Mitogens Phase 1, Phase 2
38 Dermatologic Agents Phase 1, Phase 2
39 Nucleic Acid Synthesis Inhibitors Phase 1, Phase 2
40 Anti-Bacterial Agents Phase 2,Phase 1
41 Vitamin B Complex Phase 1, Phase 2
42 Antibiotics, Antitubercular Phase 2,Phase 1
43 Antibodies Phase 2,Phase 1,Not Applicable
44 Immunoglobulins Phase 2,Phase 1,Not Applicable
45 Antibodies, Monoclonal Phase 2,Phase 1
46 Antifungal Agents Phase 1, Phase 2
47 Antilymphocyte Serum Phase 2
48 Antitubercular Agents Phase 2,Phase 1
49 Antimetabolites Phase 1, Phase 2,Phase 2
50 Antimetabolites, Antineoplastic Phase 1, Phase 2

Interventional clinical trials:

(show all 47)
# 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 Belimumab for the Treatment of Diffuse Cutaneous Systemic Sclerosis Completed NCT01670565 Phase 2 Belimumab
8 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
9 Proof of Biological Activity of SAR100842 in Systemic Sclerosis Completed NCT01651143 Phase 2 SAR100842;Placebo (for SAR100842)
10 Phase II Study of Recombinant Relaxin for Progressive Systemic Sclerosis Completed NCT00004380 Phase 2 relaxin
11 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
12 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
13 Oral Type I Collagen for Relieving Scleroderma Completed NCT00005675 Phase 2 Oral bovine type I collagen;Placebo
14 IL1-TRAP, Rilonacept, in Systemic Sclerosis Completed NCT01538719 Phase 1, Phase 2 Rilonacept
15 Rapamycin vs Methotrexate in Diffuse SSc Completed NCT00241189 Phase 1, Phase 2 rapamycin;methotrexate
16 Evaluation of Tofacitinib in Early Diffuse Cutaneous Systemic Sclerosis (dcSSc) Recruiting NCT03274076 Phase 1, Phase 2 Tofacitinib;Placebo Oral Tablet
17 Brentuximab Vedotin for Systemic Sclerosis Recruiting NCT03222492 Phase 1, Phase 2
18 Atorvastatin for Microvascular Endothelial Function and Raynaud in Early Diffuse Scleroderma Recruiting NCT02370784 Phase 2 atorvastatin;Placebo
19 Proof of Mechanism Study of GSK2330811 in Diffuse Cutaneous Systemic Sclerosis Recruiting NCT03041025 Phase 2 GSK2330811;Placebo
20 Oral Ifetroban to Treat Diffuse Cutaneous Systemic Sclerosis (SSc) or SSc-associated Pulmonary Arterial Hypertension Recruiting NCT02682511 Phase 2 Oral Ifetroban;Oral Placebo
21 Effectiveness and Safety of SAR156597 in Treating Diffuse Systemic Sclerosis Recruiting NCT02921971 Phase 2 SAR156597 (ACT14604);Placebo
22 Autologous Stem Cell Transplantation for Progressive Systemic Sclerosis Recruiting NCT01895244 Phase 2 Autologous stemcell transplantation with CD (cluster of differentiation) 34 selected stem cells
23 A Study of Subcutaneous Abatacept to Treat Diffuse Cutaneous Systemic Sclerosis Active, not recruiting NCT02161406 Phase 2 Abatacept;Placebo
24 Proof-of-concept Trial of IVA337 in Diffuse Cutaneous Systemic Sclerosis Active, not recruiting NCT02503644 Phase 2 IVA337;Placebo
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 Efficacy and Safety of Riociguat in Patients With Systemic Sclerosis Active, not recruiting NCT02283762 Phase 2 Riociguat (Adempas, BAY63-2521);Placebo
27 Brentuximab Vedotin in Early Diffuse Cutaneous Systemic Sclerosis Not yet recruiting NCT03198689 Phase 2 Brentuximab Vedotin
28 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)
29 Proof of Concept Trial of Gleevec (Imatinib) in Active Diffuse Scleroderma Terminated NCT01545427 Phase 2 Imatinib mesylate
30 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
31 Mycophenolate Mofetil in Systemic Sclerosis Completed NCT00433186 Phase 1 Mycophenolate Mofetil
32 Fresolimumab In Systemic Sclerosis Completed NCT01284322 Phase 1 Fresolimumab
33 Thalidomide for Decreasing Collagen Biosynthesis in People With Progressive Systemic Sclerosis Terminated NCT00418132 Phase 1 Thalidomide;Placebo thalidomide
34 Nonmyeloablative Allo Stem Cell Transplant for Severe Autoimmune Diseases Terminated NCT00849745 Phase 1
35 Autologous SCT for Progressive Systemic Sclerosis: a Prospective NIS Study Unknown status NCT02264405
36 Treatment Outcome in Early Diffuse Cutaneous Systemic Sclerosis Completed NCT02339441 Methotrexate;Mycophenolate mofetil;Cyclophosphamide
37 Study of Ambrisentan With Antifibrotic Agent Combination Therapy in Diffuse Systemic Sclerosis Completed NCT01093885 Not Applicable Ambrisentan
38 EBMT ADWP Prospective Non Interventional Study : AutoHSCT in SSc Patients Completed NCT02516124
39 A Non-Interventional Pilot Study Assessing Whether Lysyl Oxidase-like 2 (LOXL2) is Present in Subjects With Scleroderma Completed NCT01881529
40 Skin Biopsy Specimens as Biomarkers of Systemic Sclerosis and Response to Mycophenolate Mofetil Completed NCT00853788
41 Idiopathic Pulmonary Fibrosis--Pathogenesis and Staging - SCOR in Occupational and Immunological Lung Diseases Completed NCT00005317
42 Angiogenic/Angiostatic Mediators in Patients With Systemic Sclerosis Completed NCT00668473
43 Stimulatory Autoantibodies to the Platelet-Derived Growth Factor Receptor (PDGFR) in Patients With Systemic Sclerosis Completed NCT00667134
44 Itch and Clinacal Caracteristics in Systemic Sclerosis Completed NCT03297580
45 IVIG Treatment in Systemic Sclerosis Active, not recruiting NCT01785056 Not Applicable
46 The Effects of Exercise in Patients With Systemic Sclerosis Active, not recruiting NCT03058887 Not Applicable
47 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, Tongue, Kidney, Endothelial, Heart, Bone

Publications for Diffuse Cutaneous Systemic Sclerosis

Articles related to Diffuse Cutaneous Systemic Sclerosis:

(show top 50) (show all 62)
# 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
Double-blind, Randomized, 8-week Placebo-controlled followed by a 16-week open label extension study, with the LPA1 receptor antagonist SAR100842 for Patients With Diffuse Cutaneous Systemic Sclerosis. ( 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
Watermelon stomach and colon in a patient with diffuse cutaneous systemic sclerosis. ( 27414211 )
2016
17
The American College of Rheumatology Provisional Composite Response Index for Clinical Trials in Early Diffuse Cutaneous Systemic Sclerosis. ( 26808827 )
2016
18
Whole Exome Sequencing for Identification of Potential Causal Variants for Diffuse Cutaneous Systemic Sclerosis. ( 27111861 )
2016
19
The American College of Rheumatology Provisional Composite Response Index for Clinical Trials in Early Diffuse Cutaneous Systemic Sclerosis. ( 26806474 )
2016
20
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
21
Classical case of diffuse cutaneous systemic sclerosis. ( 27728683 )
2016
22
Perivascular Cells in Diffuse Cutaneous Systemic Sclerosis Overexpress Activated ADAM12 and Are Involved in Myofibroblast Transdifferentiation and Development of Fibrosis. ( 27252423 )
2016
23
Trigeminal neuropathy responding to systemic corticosteroids and methotrexate in a patient with diffuse cutaneous systemic sclerosis. ( 27748258 )
2016
24
RISE-SSc: Riociguat in diffuse cutaneous systemic sclerosis. ( 27746061 )
2016
25
Prediction of improvement in skin fibrosis in diffuse cutaneous systemic sclerosis: a EUSTAR analysis. ( 27016052 )
2016
26
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
27
Prediction of worsening of skin fibrosis in patients with diffuse cutaneous systemic sclerosis using the EUSTAR database. ( 24981642 )
2015
28
Rituximab in diffuse cutaneous systemic sclerosis: should we be using it today? ( 25573841 )
2015
29
A Longitudinal Biomarker for the Extent of Skin Disease in Patients With Diffuse Cutaneous Systemic Sclerosis. ( 26240058 )
2015
30
Intravenous immunoglobulin may be an effective therapy for refractory, active diffuse cutaneous systemic sclerosis. ( 25433527 )
2015
31
Disease progression in systemic sclerosis-overlap syndrome is significantly different from limited and diffuse cutaneous systemic sclerosis. ( 24389298 )
2015
32
A preliminary study of acoustic radiation force impulse quantification for the assessment of skin in diffuse cutaneous systemic sclerosis. ( 25593239 )
2015
33
Reliability, validity and responsiveness to change of the Saint George's Respiratory Questionnaire in early diffuse cutaneous systemic sclerosis. ( 25667436 )
2015
34
Gene expression changes reflect clinical response in a placebo-controlled randomized trial of abatacept in patients with diffuse cutaneous systemic sclerosis. ( 26071192 )
2015
35
Pityriasis rubra pilaris-like eruption with dermatomyositis and diffuse cutaneous systemic sclerosis overlap. ( 25758527 )
2015
36
High reactive oxygen species in fibrotic and nonfibrotic skin of patients with diffuse cutaneous systemic sclerosis. ( 26143738 )
2015
37
Derivation and external validation of a 5-year mortality prediction rule for patients with early diffuse cutaneous systemic sclerosis. ( 26554637 )
2015
38
Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. ( 25058083 )
2014
39
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
40
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
41
Derivation and validation of a prediction rule for two-year mortality in early diffuse cutaneous systemic sclerosis. ( 24470430 )
2014
42
A patient with diffuse cutaneous systemic sclerosis complicated by antineutrophil-cytoplasmic antibody-associated vasculitis exhibiting honeycomb lung without volume loss. ( 24694501 )
2014
43
Increased expression of S100A8 and S100A9 in patients with diffuse cutaneous systemic sclerosis. A correlation with organ involvement and immunological abnormalities. ( 23754244 )
2013
44
Increased production of soluble inducible costimulator in patients with diffuse cutaneous systemic sclerosis. ( 23053221 )
2013
45
A randomized, double-blind, placebo-controlled trial: intravenous immunoglobulin treatment in patients with diffuse cutaneous systemic sclerosis. ( 23910617 )
2013
46
Case of diffuse cutaneous systemic sclerosis with anti-Ku and anti-centromere antibodies. ( 23663134 )
2013
47
Augmented ICOS expression in patients with early diffuse cutaneous systemic sclerosis. ( 23024058 )
2013
48
Antineutrophil cytoplasm antibody-positive pulmonary-renal syndrome in a patient with diffuse cutaneous systemic sclerosis. ( 23771961 )
2013
49
Association of UBE2L3 polymorphisms with diffuse cutaneous systemic sclerosis in a Japanese population. ( 22294623 )
2012
50
Patterns and predictors of change in outcome measures in clinical trials in scleroderma: an individual patient meta-analysis of 629 subjects with diffuse cutaneous systemic sclerosis. ( 22328195 )
2012

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 9.61 COL22A1 COMP CTGF FBN1 ICOS MIF
2 external side of plasma membrane GO:0009897 9.02 CCR6 HLA-DRB1 ICOS ICOSLG THBS1

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

# Name GO ID Score Top Affiliating Genes
1 inflammatory response GO:0006954 9.46 IFI16 MIF SIGLEC1 THBS1
2 immune system process GO:0002376 9.35 HLA-DRB1 ICOSLG IFI16 IRF5 MIF
3 T cell costimulation GO:0031295 8.92 CAV1 HLA-DRB1 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 9.43 CAV1 FBN1 IRF5 MIF STAT4 THBS1
2 integrin binding GO:0005178 9.33 CTGF FBN1 THBS1
3 fibronectin binding GO:0001968 9.26 CTGF THBS1
4 heparin binding GO:0008201 8.92 COMP CTGF FBN1 THBS1

Sources for Diffuse Cutaneous Systemic Sclerosis

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19 FMA
28 GO
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31 HMDB
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33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
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45 MESH via Orphanet
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58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
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74 UMLS via Orphanet
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