Scleroderma pathophysiology: Difference between revisions
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===Pathogenesis=== | ===Pathogenesis=== | ||
*Scleroderma, also known as systemic sclerosis (SSc) is an autoimmune connective tissue disease.<ref name="pmid26210125">{{cite journal |vauthors=Pope JE, Johnson SR |title=New Classification Criteria for Systemic Sclerosis (Scleroderma) |journal=Rheum. Dis. Clin. North Am. |volume=41 |issue=3 |pages=383–98 |date=August 2015 |pmid=26210125 |doi=10.1016/j.rdc.2015.04.003 |url=}}</ref> | *Scleroderma, also known as systemic sclerosis (SSc) is an autoimmune connective tissue disease.<ref name="pmid19420368">{{cite journal |vauthors=Gabrielli A, Avvedimento EV, Krieg T |title=Scleroderma |journal=N. Engl. J. Med. |volume=360 |issue=19 |pages=1989–2003 |date=May 2009 |pmid=19420368 |doi=10.1056/NEJMra0806188 |url=}}</ref><ref name="pmid26210125">{{cite journal |vauthors=Pope JE, Johnson SR |title=New Classification Criteria for Systemic Sclerosis (Scleroderma) |journal=Rheum. Dis. Clin. North Am. |volume=41 |issue=3 |pages=383–98 |date=August 2015 |pmid=26210125 |doi=10.1016/j.rdc.2015.04.003 |url=}}</ref> | ||
*Important features of scleroderma include:<ref name="pmid22269658">{{cite journal |vauthors=Barnes J, Mayes MD |title=Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers |journal=Curr Opin Rheumatol |volume=24 |issue=2 |pages=165–70 |date=March 2012 |pmid=22269658 |doi=10.1097/BOR.0b013e32834ff2e8 |url=}}</ref> | *Important features of scleroderma include:<ref name="pmid22269658">{{cite journal |vauthors=Barnes J, Mayes MD |title=Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers |journal=Curr Opin Rheumatol |volume=24 |issue=2 |pages=165–70 |date=March 2012 |pmid=22269658 |doi=10.1097/BOR.0b013e32834ff2e8 |url=}}</ref> | ||
**Production of autoantibodies against various cellular antigens | **Production of autoantibodies against various cellular antigens | ||
Revision as of 15:59, 29 March 2018
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: M. Khurram Afzal, MD [2]
Overview
The exact pathogenesis of [disease name] is not fully understood.
OR
It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
OR
[Pathogen name] is usually transmitted via the [transmission route] route to the human host.
OR
Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
OR
[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
OR
The progression to [disease name] usually involves the [molecular pathway].
OR
The pathophysiology of [disease/malignancy] depends on the histological subtype.
Pathophysiology
Pathogenesis
- Scleroderma, also known as systemic sclerosis (SSc) is an autoimmune connective tissue disease.[1][2]
- Important features of scleroderma include:[3]
- Production of autoantibodies against various cellular antigens
- Small vessel vasculopathy
- Fibrosis of the skin and internal organs
- Excess of collagen deposition in the skin and internal organs
- Features of Scleroderma include:
- Sclerodactyly (thickened skin of the fingers) is common
- Extensive skin fibrosis may be present
- Raynaud phenomenon
- Esophageal dysmotility
- Pulmonary arterial hypertension
- Cardiac involvement
- Interstitial lung disease
- Inflamatory arthritis
- Digital ulcers
Genetics
Genetics associated with the development of scleroderma include:[4]
- Scleroderma occurs in a sporadic pattern in the general population.
- Variations in human leukocyte antigen (HLA) genes can predispose an individual to developing scleroderma.
- Other genes that increase the risk of developing scleroderma include:
- IRF5
- STAT4
- Genetic variation in the IRF5 gene predisposes an individual to developing diffuse cutaneous systemic scleroderma.
- Genetic variation in the STAT4 gene predisposes an individual to developing limited cutaneous systemic scleroderma.
Associated Conditions
Conditions that are associated with scleroderma include:[2]
- Nephrogenic sclerosing fibrosis
- Scleroderma diabeticorum
- Scleromyxedema
- Erythromyalgia
- Porphyria
- Lichen sclerosis
- Graft versus host disease
- Diabetic cheiroarthropathy
- Systemic lupus erythematosus (SLE)
- Rheumatoid arthritis
- Polymyositis
- Sjögren's syndrome
Gross Pathology
- On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
Microscopic Pathology
- On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
References
- ↑ Gabrielli A, Avvedimento EV, Krieg T (May 2009). "Scleroderma". N. Engl. J. Med. 360 (19): 1989–2003. doi:10.1056/NEJMra0806188. PMID 19420368.
- ↑ 2.0 2.1 Pope JE, Johnson SR (August 2015). "New Classification Criteria for Systemic Sclerosis (Scleroderma)". Rheum. Dis. Clin. North Am. 41 (3): 383–98. doi:10.1016/j.rdc.2015.04.003. PMID 26210125.
- ↑ Barnes J, Mayes MD (March 2012). "Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers". Curr Opin Rheumatol. 24 (2): 165–70. doi:10.1097/BOR.0b013e32834ff2e8. PMID 22269658.
- ↑ "Systemic scleroderma - Genetics Home Reference".