Scleroderma pathophysiology: Difference between revisions
| Line 39: | Line 39: | ||
**Production of autoantibodies | **Production of autoantibodies | ||
**Small vessel vasculopathy | **Small vessel vasculopathy | ||
**Fibrosis | **Fibrosis of the skin and internal organs | ||
**Excess of collagen deposition in the skin and internal organs | **Excess of collagen deposition in the skin and internal organs | ||
*Features of Scleroderma include: | *Features of Scleroderma include: | ||
Revision as of 19:19, 28 March 2018
|
Scleroderma Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Scleroderma pathophysiology On the Web |
|
American Roentgen Ray Society Images of Scleroderma pathophysiology |
|
Risk calculators and risk factors for Scleroderma pathophysiology |
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]
- Important characteristics of scleroderma include:[2]
- Production of autoantibodies
- 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:[3]
- 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:[1]
- Nephrogenic sclerosing fibrosis
- Scleroderma diabeticorum
- Scleromyxedema
- Erythromyalgia
- Porphyria
- Lichen sclerosis
- Graft versus host disease
- Diabetic cheiroarthropathy
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
- ↑ 1.0 1.1 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".