MCID: SYS005
MIFTS: 67

Systemic Scleroderma

Categories: Rare diseases, Skin diseases, Bone diseases, Respiratory diseases, Nephrological diseases, Neuronal diseases, Genetic diseases, Immune diseases

Aliases & Classifications for Systemic Scleroderma

MalaCards integrated aliases for Systemic Scleroderma:

Name: Systemic Scleroderma 37 12 49 24 14 69
Scleroderma 12 72 49 40 14 69
Progressive Systemic Sclerosis 12 72 49
Systemic Sclerosis 12 49 24
Scleroderma, Systemic 49 41
Scleroderma, Familial Progressive 69
Familial Progressive Scleroderma 24
Progressive Scleroderma 24
Scleroderma, Localized 41
Scleroderma Syndrome 12
Diffuse Scleroderma 69
Scleroderma Disease 51
Dermatosclerosis 12
Pss 12

Classifications:



Summaries for Systemic Scleroderma

NIH Rare Diseases : 49 Scleroderma is an autoimmune disorder that may involve changes in the skin, blood vessels, muscles, and internal organs. There are two main types: localized scleroderma, which affects only the skin; and systemic scleroderma, which affects the blood vessels and internal organs, as well as the skin. These two main types also have sub-types. Localized scleroderma subtypes include:Linear scleroderma Morphea Systemic scleroderma subtypes include:Diffuse cutaneous systemic sclerosis Limited cutaneous systemic sclerosis (which includes CREST syndrome) Limited Systemic Sclerosis The underlying cause of scleroderma is currently unknown; however, some scientists suspect it may be related to a buildup of collagen in the skin and other organs due to an abnormal immune system response. Some cases of scleroderma are induced by environmental factors or occur in association with other underlying disorders such as rheumatoid arthritis, lupus or Sjogren syndrome. There is no cure, but various treatments may relieve symptoms. Last updated: 9/12/2016

MalaCards based summary : Systemic Scleroderma, also known as scleroderma, is related to localized scleroderma and scleroderma, familial progressive, and has symptoms including recurrent urinary tract infections, renal insufficiency and nephropathy. An important gene associated with Systemic Scleroderma is CTGF (Connective Tissue Growth Factor), and among its related pathways/superpathways are Th17 Differentiation Pathway and Dendritic Cells Developmental Lineage Pathway. The drugs Bosentan and Silver sulfadiazine have been mentioned in the context of this disorder. Affiliated tissues include skin, lung and heart, and related phenotypes are homeostasis/metabolism and cardiovascular system

Disease Ontology : 12 A scleroderma that is characterized by fibrosis (or hardening) of the skin and major organs, as well as vascular alterations, and autoantibodies.

Genetics Home Reference : 24 Systemic scleroderma is an autoimmune disorder that affects the skin and internal organs. Autoimmune disorders occur when the immune system malfunctions and attacks the body's own tissues and organs. The word "scleroderma" means hard skin in Greek, and the condition is characterized by the buildup of scar tissue (fibrosis) in the skin and other organs. The condition is also called systemic sclerosis because the fibrosis can affect organs other than the skin. Fibrosis is due to the excess production of a tough protein called collagen, which normally strengthens and supports connective tissues throughout the body.

MedlinePlus : 40 Scleroderma means hard skin. It is a group of diseases that cause abnormal growth of connective tissue. Connective tissue is the material inside your body that gives your tissues their shape and helps keep them strong. In scleroderma, the tissue gets hard or thick. It can cause swelling or pain in your muscles and joints. Symptoms of scleroderma include Calcium deposits in connective tissues Raynaud's phenomenon, a narrowing of blood vessels in the hands or feet Swelling of the esophagus, the tube between your throat and stomach Thick, tight skin on your fingers Red spots on your hands and face No one knows what causes scleroderma. It is more common in women. It can be mild or severe. Doctors diagnose scleroderma using your medical history, a physical exam, lab tests, and a skin biopsy. There is no cure, but various treatments can control symptoms and complications. NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

Wikipedia : 72 Systemic scleroderma, also called diffuse scleroderma or systemic sclerosis, is an autoimmune disease of... more...

Related Diseases for Systemic Scleroderma

Diseases in the Systemic Scleroderma family:

Scleroderma, Familial Progressive Localized Scleroderma

Diseases related to Systemic Scleroderma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 171)
# Related Disease Score Top Affiliating Genes
1 localized scleroderma 34.7 CTGF SELE TGFB1
2 scleroderma, familial progressive 33.5 CTGF FBN1 TOP1
3 stiff skin syndrome 32.3 CTGF FBN1 TGFB1
4 crest syndrome 31.5 CENPB TOP1
5 limited scleroderma 30.8 CENPB CTGF EXOSC10 SNRNP70 TOP1 TSSK1B
6 silicosis 30.0 ACE SFTPD TGFB1
7 interstitial lung disease 29.7 IFNG SFTPD TGFB1
8 facial hemiatrophy 29.7 CD40LG EXOSC10 SNRNP70
9 arteries, anomalies of 29.5 ACE CRP SELE SELP TLR9
10 systemic lupus erythematosus 29.4 CD40LG CRP IFNG SELE SNRNP70 TGFB1
11 diffuse cutaneous systemic sclerosis 11.8
12 peeling skin syndrome 1 11.7
13 potocki-shaffer syndrome 11.7
14 peeling skin syndrome 2 11.7
15 peeling skin syndrome 3 11.6
16 peeling skin syndrome 5 11.1
17 adrenoleukodystrophy 11.0
18 reynolds syndrome 11.0
19 diffuse scleroderma 11.0
20 generalized peeling skin syndrome 11.0
21 psychosocial short stature 10.9
22 loeffler syndrome 10.7 ACE CD40LG
23 heterophyiasis 10.6 CD40LG SELE
24 familial vesicoureteral reflux 10.6 ACE TGFB1
25 microvascular complications of diabetes 3 10.6 ACE CTGF TGFB1
26 ureteral disease 10.5 ACE CTGF TGFB1
27 hypersensitivity reaction type iii disease 10.5 CD40LG CRP SELE
28 nephrosclerosis 10.5 ACE CTGF TGFB1
29 pustulosis palmaris et plantaris 10.5 CD40LG SELE TGFB1
30 african tick-bite fever 10.5 CD40LG IFNG SELE
31 granulomatous hepatitis 10.5 ACE CD40LG CRP
32 multiple cranial nerve palsy 10.5 ACE CD40LG CRP
33 generalized atherosclerosis 10.5 ACE CRP SELP
34 heparin-induced thrombocytopenia 10.5 CD40LG SELP
35 legg-calve-perthes disease 10.5 CD40LG SELE SELP
36 chronic active epstein-barr virus infection 10.5 IFNG SELE TGFB1
37 plasmodium vivax malaria 10.5 CD40LG IFNG SELE
38 intermediate coronary syndrome 10.5 ACE CRP SELP
39 neuroretinitis 10.5 ACE CD40LG MLN
40 acalculous cholecystitis 10.5 CD40LG CRP
41 carotid artery disease 10.5 ACE CRP SELE
42 diabetic foot ulcers 10.5 CRP TGFB1
43 brucellosis 10.5 CD40LG CRP IFNG
44 takayasu arteritis 10.5 CD40LG CRP SELE
45 peripheral artery disease 10.5 ACE CRP SELP
46 temporal arteritis 10.5 CD40LG CRP IFNG
47 intermittent claudication 10.5 ACE CRP SELP
48 aortic aneurysm, familial abdominal, 1 10.5 ACE CRP FBN1
49 mononeuritis multiplex 10.5 CD40LG CRP
50 antiphospholipid syndrome 10.5 CD40LG SELE SELP

Comorbidity relations with Systemic Scleroderma via Phenotypic Disease Network (PDN): (show all 13)


Active Peptic Ulcer Disease Acute Cystitis
Deficiency Anemia Esophagitis
Heart Disease Hypothyroidism
Localized Scleroderma Peripheral Vascular Disease
Postinflammatory Pulmonary Fibrosis Protein-Energy Malnutrition
Pulmonary Hypertension, Primary, 1 Raynaud Disease
Systemic Lupus Erythematosus

Graphical network of the top 20 diseases related to Systemic Scleroderma:



Diseases related to Systemic Scleroderma

Symptoms & Phenotypes for Systemic Scleroderma

Human phenotypes related to Systemic Scleroderma:

31 (show top 50) (show all 67)
# Description HPO Frequency HPO Source Accession
1 recurrent urinary tract infections 31 frequent (33%) HP:0000010
2 renal insufficiency 31 occasional (7.5%) HP:0000083
3 nephropathy 31 frequent (33%) HP:0000112
4 narrow mouth 31 occasional (7.5%) HP:0000160
5 xerostomia 31 hallmark (90%) HP:0000217
6 gingival bleeding 31 occasional (7.5%) HP:0000225
7 gingivitis 31 hallmark (90%) HP:0000230
8 behavioral abnormality 31 occasional (7.5%) HP:0000708
9 decreased nerve conduction velocity 31 frequent (33%) HP:0000762
10 hematuria 31 occasional (7.5%) HP:0000790
11 chondrocalcinosis 31 frequent (33%) HP:0000934
12 dry skin 31 hallmark (90%) HP:0000958
13 hyperkeratosis 31 frequent (33%) HP:0000962
14 urticaria 31 frequent (33%) HP:0001025
15 hypopigmented skin patches 31 frequent (33%) HP:0001053
16 acrocyanosis 31 hallmark (90%) HP:0001063
17 seizures 31 occasional (7.5%) HP:0001250
18 muscle weakness 31 hallmark (90%) HP:0001324
19 arthritis 31 hallmark (90%) HP:0001369
20 joint dislocation 31 occasional (7.5%) HP:0001373
21 cirrhosis 31 occasional (7.5%) HP:0001394
22 subcutaneous nodule 31 hallmark (90%) HP:0001482
23 congestive heart failure 31 occasional (7.5%) HP:0001635
24 hypertrophic cardiomyopathy 31 occasional (7.5%) HP:0001639
25 myocardial infarction 31 frequent (33%) HP:0001658
26 pericarditis 31 frequent (33%) HP:0001701
27 nausea and vomiting 31 hallmark (90%) HP:0002017
28 gastroesophageal reflux 31 hallmark (90%) HP:0002020
29 malabsorption 31 frequent (33%) HP:0002024
30 abdominal pain 31 frequent (33%) HP:0002027
31 abnormality of the rectum 31 occasional (7.5%) HP:0002034
32 restrictive ventilatory defect 31 hallmark (90%) HP:0002091
33 pulmonary arterial hypertension 31 occasional (7.5%) HP:0002092
34 pulmonary infiltrates 31 occasional (7.5%) HP:0002113
35 pulmonary fibrosis 31 frequent (33%) HP:0002206
36 gastrointestinal hemorrhage 31 occasional (7.5%) HP:0002239
37 memory impairment 31 occasional (7.5%) HP:0002354
38 tracheoesophageal fistula 31 occasional (7.5%) HP:0002575
39 bowel incontinence 31 frequent (33%) HP:0002607
40 osteomyelitis 31 occasional (7.5%) HP:0002754
41 abnormal pattern of respiration 31 frequent (33%) HP:0002793
42 osteolysis 31 occasional (7.5%) HP:0002797
43 arthralgia 31 hallmark (90%) HP:0002829
44 autoimmunity 31 hallmark (90%) HP:0002960
45 skeletal muscle atrophy 31 occasional (7.5%) HP:0003202
46 myalgia 31 hallmark (90%) HP:0003326
47 cachexia 31 occasional (7.5%) HP:0004326
48 abnormality of the anus 31 occasional (7.5%) HP:0004378
49 cranial nerve paralysis 31 frequent (33%) HP:0006824
50 feeding difficulties in infancy 31 frequent (33%) HP:0008872

UMLS symptoms related to Systemic Scleroderma:


hidebound skin, pruritus, exanthema

MGI Mouse Phenotypes related to Systemic Scleroderma:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.03 SCHIP1 ACE SELE CD40LG SELP CRP
2 cardiovascular system MP:0005385 10.02 SCHIP1 ACE SELE CD40LG SELP CRP
3 mortality/aging MP:0010768 10 SCHIP1 ACE SELE CD40LG SELP CENPB
4 renal/urinary system MP:0005367 9.56 SCHIP1 ACE CD40LG SELP TGFB1 FBN1
5 respiratory system MP:0005388 9.17 SELE SELP SFTPD CTGF TGFB1 FBN1

Drugs & Therapeutics for Systemic Scleroderma

Drugs for Systemic Scleroderma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 256)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bosentan Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1 147536-97-8 104865
2
Silver sulfadiazine Approved, Vet_approved Phase 4 22199-08-2 441244
3
Tadalafil Approved, Investigational Phase 4,Phase 3,Phase 2 171596-29-5 110635
4
Cilostazol Approved, Investigational Phase 4 73963-72-1 2754
5
Citric Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 3,Phase 2,Early Phase 1 77-92-9 311
6 Vaccines Phase 4
7 Antihypertensive Agents Phase 4,Phase 3,Phase 2,Phase 1
8 Endothelin Receptor Antagonists Phase 4,Phase 3,Phase 2,Phase 1
9 Phosphodiesterase 5 Inhibitors Phase 4,Phase 3,Phase 2,Early Phase 1
10 Phosphodiesterase Inhibitors Phase 4,Phase 3,Phase 2,Early Phase 1
11 Vasodilator Agents Phase 4,Phase 2,Phase 3,Early Phase 1
12 Autonomic Agents Phase 4,Phase 2,Phase 3,Early Phase 1
13 Neuroprotective Agents Phase 4,Phase 2,Phase 3,Early Phase 1
14 Peripheral Nervous System Agents Phase 4,Phase 2,Phase 3,Phase 1,Early Phase 1
15 Platelet Aggregation Inhibitors Phase 4,Phase 2,Phase 3
16 Protective Agents Phase 4,Phase 2,Phase 3,Phase 1,Early Phase 1
17 Respiratory System Agents Phase 4,Phase 2,Phase 3
18 Sildenafil Citrate Phase 4,Phase 3,Phase 2,Early Phase 1 171599-83-0
19 Anti-Asthmatic Agents Phase 4
20 Bronchodilator Agents Phase 4
21 Fibrinolytic Agents Phase 4,Phase 2,Phase 3
22 Phosphodiesterase 3 Inhibitors Phase 4
23 Citrate Nutraceutical Phase 4,Phase 3,Phase 2,Early Phase 1
24 Acidophilus Nutraceutical Phase 4,Phase 3
25 Omega 3 Fatty Acid Nutraceutical Phase 4
26
Acetylcysteine Approved, Investigational Phase 2, Phase 3 616-91-1 12035
27
Iloprost Approved, Investigational Phase 2, Phase 3 78919-13-8 6443959
28
Nitroglycerin Approved, Investigational Phase 3,Phase 2 55-63-0 4510
29
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3, Early Phase 1 83-43-2 6741
30
Prednisolone Approved, Vet_approved Phase 2, Phase 3, Early Phase 1 50-24-8 5755
31
rituximab Approved Phase 2, Phase 3 174722-31-7 10201696
32
Cyclophosphamide Approved, Investigational Phase 3,Phase 2,Phase 1 50-18-0, 6055-19-2 2907
33
Mycophenolate mofetil Approved, Investigational Phase 3,Phase 2,Phase 1 128794-94-5 5281078
34
Mycophenolic acid Approved Phase 3,Phase 2,Phase 1 24280-93-1 446541
35
Lenograstim Approved, Investigational Phase 3,Phase 2,Phase 1 135968-09-1
36
Lidocaine Approved, Vet_approved Phase 2, Phase 3 137-58-6 3676
37
Macitentan Approved Phase 3 441798-33-0
38
Alginic acid Approved, Experimental, Investigational Phase 3 9005-32-7
39
Domperidone Approved, Investigational, Vet_approved Phase 3 57808-66-9 3151
40
Dopamine Approved Phase 3 51-61-6, 62-31-7 681
41
Omeprazole Approved, Investigational, Vet_approved Phase 3 73590-58-6 4594
42
Acetylcholine Approved Phase 3,Phase 2 51-84-3 187
43
Methocarbamol Approved, Vet_approved Phase 3,Phase 2 532-03-6 4107
44
Mesna Approved, Investigational Phase 2, Phase 3, Phase 1 3375-50-6 598
45
Imiquimod Approved, Investigational Phase 3 99011-02-6 57469
46
Pirfenidone Approved, Investigational Phase 2, Phase 3,Phase 1 53179-13-8 40632
47
Fludarabine Approved Phase 3,Phase 1,Phase 2 21679-14-1, 75607-67-9 30751
48
Vidarabine Approved, Investigational Phase 3,Phase 1,Phase 2 24356-66-9 21704 32326
49
Lactulose Approved Phase 3 4618-18-2 11333
50
Prednisone Approved, Vet_approved Phase 3,Phase 1 53-03-2 5865

Interventional clinical trials:

(show top 50) (show all 350)

# Name Status NCT ID Phase Drugs
1 Influenza Vaccination in Patients With Scleroderma Unknown status NCT01002508 Phase 4
2 The Clinical And Subclinical Effects on Arterial Stiffness of Bosentan in Patients With Systemic Sclerosis Unknown status NCT02480335 Phase 4 bosentan
3 Exercise Induced Pulmonary Hypertension in Systemic Sclerosis and Treatment With Ambrisentan Unknown status NCT01051960 Phase 4 Ambrisentan
4 Safety and Efficacy of Anti-Pandemic H1N1 Vaccination in Rheumatic Diseases Unknown status NCT01151644 Phase 4
5 Bosentan in Systemic Sclerosis Completed NCT01395732 Phase 4 Bosentan
6 A Clinical Trial of Ambrisentan and Tadalafil in Pulmonary Arterial Hypertension Associated With Systemic Sclerosis Completed NCT01042158 Phase 4 tadalafil and ambrisentan upfront combination therapy
7 Pulmonary Artery Remodelling With Bosentan Completed NCT00595049 Phase 4 bosentan
8 Safety and Efficacy of Pletal (Cilostazol) for the Treatment of Juvenile Primary and Secondary Raynaud’s Phenomenon Completed NCT00048776 Phase 4 Pletal
9 Efficacy and Safety of PLACENTEX ® i.m. in Patients With Scleroderma Diseases Recruiting NCT03388255 Phase 4 Polydeoxyribonucleotides
10 To Compare the Efficacy of Combination Therapy vs Monotherapy for Pulmonary Arterial Hypertension in Systemic Sclerosis Recruiting NCT03053739 Phase 4 Sildenafil 20mg and Bosentan 62.5mg;Sildenafil 20mg and Placebo
11 Trial to Study the Effects of Supplementary Omega-3 on Serum C-Reactive Protein Levels Terminated NCT00578578 Phase 4
12 Comparison of Esophageal and Anorectal Manometry Catheters Terminated NCT00204763 Phase 4
13 Probiotics in Patients With Moderate-to-severe Distention/ Bloating From Systemic Sclerosis Withdrawn NCT01497743 Phase 4 Lactobacillus
14 Intraoperative ICG for Systemic Sclerosis Withdrawn NCT03155464 Phase 4 Indocyanine Green
15 Effects of Probiotics on Gastrointestinal Symptoms and on the Immune System in Patients With Systemic Sclerosis Unknown status NCT02302352 Phase 3
16 High Dose Intravenous N-Acetylcysteine Versus Iloprost for Early, Rapidly Progressive Diffuse Systemic Sclerosis Unknown status NCT00428883 Phase 2, Phase 3 N-acetylcysteine (NAC)
17 Serial Night Time Position Splint on Systemic Sclerosis Unknown status NCT01586663 Phase 3 Drug treatment
18 Platelet Gel in Systemic Sclerosis Unknown status NCT00463125 Phase 2, Phase 3 Platelet Gel
19 Early Diagnosis of Pulmonary Hypertension in Patients With Inflammatory Rheumatic Connective Tissue Diseases Unknown status NCT01387035 Phase 3
20 A Study of a Topical Form of Nitroglycerin and Placebo in the Treatment and Prevention of Raynaud's Phenomenon Unknown status NCT00577304 Phase 3 Nitroglycerin;Topical AmphiMatrix
21 Study of a Topical Formulation for the Treatment and Prevention of Raynaud's Phenomenon Symptoms Unknown status NCT00934427 Phase 3 0.9% nitroglycerin in TAM cream;vehicle cream
22 Sildenafil Effect on Digital Ulcer Healing in sClerodErma SEDUCE STUDY Completed NCT01295736 Phase 3 Sildenafil;placebo
23 Rituximab in Systemic Sclerosis Completed NCT01748084 Phase 2, Phase 3 Rituximab;Placebo (NaCl)
24 Efficacy and Safety Study of GB-0998 for Treatment of Systemic Sclerosis Completed NCT00348296 Phase 3 High-dose intravenous immunoglobulin (Venoglobulin-IH)
25 High Dose Cyclophosphamide for Treatment of Scleroderma Completed NCT00501995 Phase 3 IV Cyclophosphamide
26 Effectiveness and Safety of Lidocaine for Scleroderma Completed NCT00740285 Phase 2, Phase 3 Lidocaine 2% without vessel constrictor
27 Macitentan for the Treatment of Digital Ulcers in Systemic Sclerosis Patients Completed NCT01474109 Phase 3 macitentan 3mg;macitentan 10mg;placebo
28 Gastroesophageal Reflux Treatment in Scleroderma Completed NCT01878526 Phase 3 Alginic acid;placebo (for domperidone);Domperidone;placebo (of alginic acid)
29 Efficacy of Botulinum Toxin In Scleroderma-Associated Raynaud's Syndrome Completed NCT02165111 Phase 3 Onabotulinumtoxin A;sterile saline solution
30 Recombinant Human Relaxin in the Treatment of Diffuse Scleroderma Completed NCT00704665 Phase 3 Relaxin;Relaxin
31 Scleroderma Lung Disease Completed NCT00004563 Phase 3 Cyclophosphamide;Placebo
32 Scleroderma: Cyclophosphamide or Transplantation (SCOT) Completed NCT00114530 Phase 2, Phase 3 cyclophosphamide
33 Effect of Sildenafil on the Microcirculatory Blood Flow and Endothelial Progenitor Cells in Systemic Sclerosis Completed NCT01347008 Phase 3 Sildenafil citrate;Placebo (Sugar pill)
34 Efficacy and Safety of Oral Bosentan on Healing/Prevention of Digital (Finger) Ulcers in Patients With Scleroderma Completed NCT00077584 Phase 3 Bosentan 62.5 mg;Bosentan 125 mg;Placebo
35 Effect of Rosuvastatin on Systemic Sclerosis-related Pulmonary Hypertension Completed NCT00984932 Phase 3 Rosuvastatin
36 Efficacy and Safety of Oral Bosentan in Pulmonary Fibrosis Associated With Scleroderma Completed NCT00070590 Phase 2, Phase 3 Bosentan
37 A Randomized Control Trial to Assess the Efficacy of Tadalafil in Raynaud's Phenomenon in Scleroderma Completed NCT01117298 Phase 3 Tadalafil;Placebo
38 A Trial of Tadalafil in Interstitial Lung Disease of Scleroderma Completed NCT01553981 Phase 3 Tadalafil;Placebo
39 Bosentan in Digital Ulcers Completed NCT00319696 Phase 3 bosentan
40 A Study of RoActemra/Actemra (Tocilizumab) Versus Placebo in Patients With Systemic Sclerosis Completed NCT01532869 Phase 3 Placebo;tocilizumab [RoActemra/Actemra];tocilizumab [RoActemra/Actemra]
41 Randomised Control Trial to Assess the Efficacy of Tadalafil in Raynaud's Phenomenon in Scleroderma Completed NCT00626665 Phase 3 Tadalafil
42 Phase III Randomized, Double-Blind, Placebo-Controlled Study of Oral Iloprost for Raynaud's Phenomenon Secondary to Systemic Sclerosis Completed NCT00004786 Phase 3 iloprost
43 Open-label Study With Bosentan in Interstitial Lung Disease Completed NCT00319033 Phase 2, Phase 3 bosentan
44 Imiquimod in Children With Plaque Morphea Completed NCT00147771 Phase 3 Imiquimod 5% cream
45 A Rho-kinase Inhibitor (Fasudil) in the Treatment of Raynaud's Phenomenon Completed NCT00498615 Phase 3 Fasudil
46 Phase III Study of Topical Gel for Treatment and Prevention of Raynaud's Phenomenon Completed NCT00266669 Phase 3 Topical organogel with nitroglycerin
47 Lab Study of MQX-503 in Treatment of Raynaud's Completed NCT00253331 Phase 2, Phase 3 topical organogel with nitroglycerin
48 Phase III Study of a Topical Gel Formulation for Treatment and Prevention of Raynaud's Phenomenon Completed NCT00419419 Phase 3 Topical AmphiMatrix with nitroglycerin (MQX-503)
49 Six Month Clinical Research Study for Patients With Moderate or Severe Dry Eye Syndrome Completed NCT00025818 Phase 3 Ophthalmic Emulsion
50 "As Required" Oral Sildenafil in Raynaud's Phenomenon Completed NCT02050360 Phase 2, Phase 3 Sildenafil 40 mg;Sildenafil 80 mg;Placebo

Search NIH Clinical Center for Systemic Scleroderma

Inferred drug relations via UMLS 69 / NDF-RT 47 :


Cell-based therapeutics:


LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database
Read about Systemic Scleroderma cell therapies at LifeMap Discovery.
Stem-cell-based therapeutic approaches for Systemic Scleroderma:
Hematopoietic stem cells for systemic sclerosis

Cochrane evidence based reviews: scleroderma, localized

Genetic Tests for Systemic Scleroderma

Anatomical Context for Systemic Scleroderma

MalaCards organs/tissues related to Systemic Scleroderma:

38
Skin, Lung, Heart, Testes, Endothelial, Kidney, Eye

Publications for Systemic Scleroderma

Articles related to Systemic Scleroderma:

(show top 50) (show all 225)
# Title Authors Year
1
Importance of alternative-site blood glucose testing in the diagnosis of artifactual hypoglycaemia in systemic scleroderma. ( 28389064 )
2017
2
Pulmonary metastasis from uterine leiomyosarcoma in a patient with limited cutaneous systemic scleroderma. ( 29264076 )
2017
3
Pharmacokinetic optimitzation of CCG-203971: Novel inhibitors of the Rho/MRTF/SRF transcriptional pathway as potential antifibrotic therapeutics for systemic scleroderma. ( 28285914 )
2017
4
Nephrotic syndrome as the first manifestation of juvenile systemic scleroderma. ( 29173697 )
2017
5
The Antiretroviral nelfinavir mesylate, a potential therapy for systemic scleroderma. ( 28940894 )
2017
6
Platelet-rich gel for the adjuvant treatment of wound healing of transposed flap for arteriovenous fistula in systemic scleroderma. ( 28058708 )
2017
7
Rheopheresis as a causal therapy option for systemic scleroderma (SSc). ( 29036804 )
2017
8
Orofacial Manifestations and Temporomandibular Disorders of Systemic Scleroderma: An Observational Study. ( 27455250 )
2016
9
Fixed Implant Supported Rehabilitation of Partially Edentulous Posterior Maxilla in a Patient With Systemic Scleroderma: A Case Report. ( 26671839 )
2016
10
A neoteric multidrug combination: novel approach to limited cutaneous systemic scleroderma involving the face. ( 27033280 )
2016
11
Caring for the Patient With Limited Systemic Scleroderma. ( 26814000 )
2016
12
Distinguishing between limited systemic scleroderma-associated pseudo-obstruction and peritoneal dissemination. ( 26943389 )
2015
13
Expression of Let-7 family microRNAs in skin correlates negatively with severity of pulmonary hypertension in patients with systemic scleroderma. ( 28785688 )
2015
14
Interstitial Lung Disease in Systemic Scleroderma, Complicated with Bilateral Pulmonary Aspergilloma: An Unusual Association. ( 26816937 )
2015
15
Unilateral acquired Brown's syndrome in systemic scleroderma: An unusual cause for diplopia. ( 26669341 )
2015
16
Pro- and antiangiogenic markers in patients with pulmonary complications of systemic scleroderma. ( 25447676 )
2015
17
Comparison of a new, modified lung ultrasonography technique with high-resolution CT in the diagnosis of the alveolo-interstitial syndrome of systemic scleroderma. ( 24567921 )
2014
18
An unusual cause of acute kidney injury due to oxalate nephropathy in systemic scleroderma. ( 25500295 )
2014
19
Fat embolism syndrome: an autopsy-proven case involving a patient on dialysis and systemic scleroderma. ( 24526839 )
2014
20
Oesophageal stenosis dilatation through retrograde trans-gastrostomal approach in a patient with systemic scleroderma. ( 24956348 )
2014
21
Magnetic resonance imaging findings in patients with systemic scleroderma and musculoskeletal symptoms. ( 22843057 )
2013
22
Systemic scleroderma associated with ulnar nerve entrapment at the elbow. ( 24343497 )
2013
23
Clinicopathological study of 81 cases of localized and systemic scleroderma. ( 22620486 )
2013
24
Antiphospholipid antibodies and systemic scleroderma. ( 24385841 )
2013
25
Antiphospholipid antibodies and systemic scleroderma. ( 24385750 )
2013
26
Functional impairment of systemic scleroderma patients with digital ulcerations: results from the DUO Registry. ( 23910613 )
2013
27
Case of lung carcinoma revealed by vulvar metastasis associated with systemic scleroderma and literature review. ( 24416551 )
2013
28
Pulmonary artery hypertension associated with systemic scleroderma: correlation among lung pulmonary blood volume, lung perfusion single photon emission computed tomography, and chest computed tomography images. ( 23283858 )
2013
29
Bone density in Moroccan women with systemic scleroderma and its relationships with disease-related parameters and vitamin D status. ( 21947376 )
2012
30
The optimal management of patients with systemic scleroderma and coronary artery disease. ( 22774391 )
2012
31
Drug compliance in patients with systemic scleroderma. ( 22875701 )
2012
32
Determinants of disability, quality of life and depression in dermatological patients with systemic scleroderma. ( 21916888 )
2012
33
Cardiac arrhythmia as a side effect of ketanserin therapy in a patient with systemic scleroderma. ( 22507478 )
2012
34
MicroRNA array analysis of microRNAs related to systemic scleroderma. ( 21052672 )
2012
35
Cardiovascular assessment of asymptomatic patients with juvenile-onset localized and systemic scleroderma: 10 years prospective observation. ( 22103465 )
2012
36
Frequency of disease-associated and other nuclear autoantibodies in patients of the German Network for Systemic Scleroderma: correlation with characteristic clinical features. ( 22018289 )
2011
37
Reduction of regulatory T cells in skin lesions but not in peripheral blood of patients with systemic scleroderma. ( 21097800 )
2011
38
Hearing loss in diffuse cutaneous systemic scleroderma. ( 21916804 )
2011
39
Fingertip calcinosis cutis in an incomplete limited-type systemic scleroderma. ( 21708515 )
2011
40
Study about the association between anticardiolipin antibodies and peripheral vascular phenomena in patients suffering from systemic scleroderma. ( 20676482 )
2010
41
Type 1 diabetes mellitus, celiac disease, systemic lupus erythematosus and systemic scleroderma in a 15-year-old girl. ( 19506880 )
2010
42
Vitamin D deficiency in a cohort of patients with systemic scleroderma from the south of Spain. ( 20516041 )
2010
43
[Antibodies to enzymes of purine metabolism as a factor of gastrointestinal tract lesions in systemic scleroderma]. ( 19551958 )
2009
44
Electrocardiographic evaluation in patients with systemic scleroderma and without clinically evident heart disease. ( 19614636 )
2009
45
Surgical result of critical limb ischemia due to tibial arterial occlusion in patients with systemic scleroderma. ( 19223137 )
2009
46
Gastrointestinal symptoms and motility disorders in patients with systemic scleroderma. ( 18304354 )
2008
47
Management of cutaneous vascular complications in systemic scleroderma: experience from the German network. ( 18320192 )
2008
48
Development of systemic scleroderma in a patient with myelodysplastic syndrome. ( 18779108 )
2008
49
The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement. ( 18515867 )
2008
50
Chronic toxicity risk after radiotherapy for patients with systemic sclerosis (systemic scleroderma) or systemic lupus erythematosus: association with connective tissue disorder severity. ( 18158195 )
2008

Variations for Systemic Scleroderma

Expression for Systemic Scleroderma

Search GEO for disease gene expression data for Systemic Scleroderma.

Pathways for Systemic Scleroderma

GO Terms for Systemic Scleroderma

Cellular components related to Systemic Scleroderma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.65 ACE CD40LG CRP CTGF FBN1 IFNG
2 proteinaceous extracellular matrix GO:0005578 9.55 CTGF FBN1 SFTPD TGFB1 TLR9
3 extracellular space GO:0005615 9.36 ACE CD40LG CRP CTGF FBN1 IFNG

Biological processes related to Systemic Scleroderma according to GeneCards Suite gene sharing:

(show all 12)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 9.77 CRP CTGF IFNG TGFB1 TLR9
2 regulation of receptor activity GO:0010469 9.73 CD40LG CTGF FBN1 IFNG MLN TGFB1
3 cellular response to transforming growth factor beta stimulus GO:0071560 9.58 FBN1 SNRNP70 TGFB1
4 leukocyte tethering or rolling GO:0050901 9.51 SELE SELP
5 leukocyte cell-cell adhesion GO:0007159 9.5 CD40LG SELE SELP
6 regulation of regulatory T cell differentiation GO:0045589 9.49 IFNG TGFB1
7 positive regulation of superoxide anion generation GO:0032930 9.48 CRP TGFB1
8 negative regulation of interleukin-17 production GO:0032700 9.46 IFNG TGFB1
9 cellular response to insulin-like growth factor stimulus GO:1990314 9.43 FBN1 TGFB1
10 mononuclear cell proliferation GO:0032943 9.16 ACE TGFB1
11 positive regulation of interleukin-12 production GO:0032735 9.13 CD40LG IFNG TLR9
12 inflammatory response GO:0006954 9.1 CD40LG CRP SELE SELP TGFB1 TLR9

Molecular functions related to Systemic Scleroderma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 sialic acid binding GO:0033691 8.96 SELE SELP
2 oligosaccharide binding GO:0070492 8.62 SELE SELP

Sources for Systemic Scleroderma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
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47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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