Systemic scleroderma: Difference between revisions
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{{SI}} | |||
{{Infobox medical condition | |||
| name = Systemic scleroderma | |||
| image = [[File:Riehl_Zumbusch_Tafel_LXIX_(1).jpg]] | |||
| caption = Skin changes in systemic scleroderma | |||
| synonyms = [[Systemic sclerosis]] | |||
| pronounce = | |||
| specialty = [[Rheumatology]] | |||
| symptoms = [[Skin thickening]], [[Raynaud's phenomenon]], [[joint pain]], [[esophageal dysmotility]], [[pulmonary fibrosis]] | |||
| complications = [[Pulmonary hypertension]], [[renal crisis]], [[heart failure]] | |||
| onset = Typically between ages 30 and 50 | |||
| duration = Chronic | |||
| causes = [[Autoimmune disease]] | |||
| risks = [[Genetic predisposition]], [[environmental factors]] | |||
| diagnosis = [[Clinical examination]], [[autoantibody testing]], [[skin biopsy]] | |||
| differential = [[Localized scleroderma]], [[lupus erythematosus]], [[dermatomyositis]] | |||
| treatment = [[Immunosuppressive therapy]], [[physical therapy]], [[antifibrotic agents]] | |||
| medication = [[Methotrexate]], [[mycophenolate mofetil]], [[cyclophosphamide]] | |||
| prognosis = Variable; depends on organ involvement | |||
| frequency = 1 in 10,000 people | |||
| deaths = | |||
}} | |||
{{Short description|A chronic connective tissue disease}} | {{Short description|A chronic connective tissue disease}} | ||
[[File:Left Arm Scleroderma Patient.jpg|left|thumb |500px|Left Arm Scleroderma Patient]] | |||
[[File:NIH 3T3.jpg|left|thumb|Fibroblasts]] | |||
[[Image:Peptic stricture.png|right|left|thumb|200px|[[Gastroscopy|Endoscopic]] image of peptic stricture, or narrowing of the [[esophagus]] near the junction with the [[stomach]] due to chronic [[gastroesophageal reflux]]. This is the most common cause of [[dysphagia]], or difficulty swallowing, in scleroderma.]] | |||
[[Image:Thrombotic microangiopathy - very high mag.jpg|left|thumb|[[Micrograph]] showing [[thrombotic microangiopathy]], the [[histomorphology|histomorphologic]] finding seen in scleroderma renal crisis. [[Kidney biopsy]]. [[PAS stain]].]] | |||
[[File:Systemic sclerosis finger.jpg|left|thumb|Clinical appearance of acrosclerotic piece-meal [[necrosis]] of the thumb in a patient with systemic sclerosis.]] | |||
'''Systemic scleroderma''', also known as '''systemic sclerosis''', is a chronic autoimmune disease characterized by [[fibrosis]] (thickening and hardening) of the skin and internal organs. It is a complex condition that affects the [[connective tissue]] and can lead to significant morbidity and mortality. | '''Systemic scleroderma''', also known as '''systemic sclerosis''', is a chronic autoimmune disease characterized by [[fibrosis]] (thickening and hardening) of the skin and internal organs. It is a complex condition that affects the [[connective tissue]] and can lead to significant morbidity and mortality. | ||
==Pathophysiology== | ==Pathophysiology== | ||
Systemic scleroderma is primarily characterized by excessive [[collagen]] deposition in the skin and other organs. The exact cause of the disease is unknown, but it is believed to involve a combination of genetic, environmental, and immunological factors. The disease process involves [[autoimmunity]], [[vascular dysfunction]], and [[fibrosis]]. | Systemic scleroderma is primarily characterized by excessive [[collagen]] deposition in the skin and other organs. The exact cause of the disease is unknown, but it is believed to involve a combination of genetic, environmental, and immunological factors. The disease process involves [[autoimmunity]], [[vascular dysfunction]], and [[fibrosis]]. | ||
===Autoimmunity=== | ===Autoimmunity=== | ||
In systemic scleroderma, the immune system mistakenly attacks the body's own tissues, leading to inflammation and fibrosis. [[Autoantibodies]] such as anti-centromere and anti-Scl-70 are often present in patients and can be used as markers for diagnosis. | In systemic scleroderma, the immune system mistakenly attacks the body's own tissues, leading to inflammation and fibrosis. [[Autoantibodies]] such as anti-centromere and anti-Scl-70 are often present in patients and can be used as markers for diagnosis. | ||
===Vascular Dysfunction=== | ===Vascular Dysfunction=== | ||
Vascular abnormalities are a hallmark of systemic scleroderma. Patients often experience [[Raynaud's phenomenon]], which is characterized by episodic vasospasm of the fingers and toes. Over time, persistent vascular damage can lead to [[digital ulcers]] and [[pulmonary arterial hypertension]]. | Vascular abnormalities are a hallmark of systemic scleroderma. Patients often experience [[Raynaud's phenomenon]], which is characterized by episodic vasospasm of the fingers and toes. Over time, persistent vascular damage can lead to [[digital ulcers]] and [[pulmonary arterial hypertension]]. | ||
===Fibrosis=== | ===Fibrosis=== | ||
Fibrosis in systemic scleroderma results from the overproduction of collagen by [[fibroblasts]]. This leads to thickening and hardening of the skin and can affect internal organs such as the [[lungs]], [[heart]], [[kidneys]], and [[gastrointestinal tract]]. | Fibrosis in systemic scleroderma results from the overproduction of collagen by [[fibroblasts]]. This leads to thickening and hardening of the skin and can affect internal organs such as the [[lungs]], [[heart]], [[kidneys]], and [[gastrointestinal tract]]. | ||
==Clinical Features== | ==Clinical Features== | ||
The clinical presentation of systemic scleroderma is highly variable and can range from mild skin involvement to severe multi-organ disease. | The clinical presentation of systemic scleroderma is highly variable and can range from mild skin involvement to severe multi-organ disease. | ||
===Skin Involvement=== | ===Skin Involvement=== | ||
The skin changes in systemic scleroderma typically begin with [[edema]] and progress to [[sclerosis]]. The skin becomes tight, shiny, and may restrict movement. [[Telangiectasia]] and [[calcinosis]] can also occur. | The skin changes in systemic scleroderma typically begin with [[edema]] and progress to [[sclerosis]]. The skin becomes tight, shiny, and may restrict movement. [[Telangiectasia]] and [[calcinosis]] can also occur. | ||
===Musculoskeletal System=== | ===Musculoskeletal System=== | ||
Patients may experience joint pain, stiffness, and muscle weakness. [[Arthralgia]] and [[myopathy]] are common symptoms. | Patients may experience joint pain, stiffness, and muscle weakness. [[Arthralgia]] and [[myopathy]] are common symptoms. | ||
===Pulmonary Involvement=== | ===Pulmonary Involvement=== | ||
Lung involvement is a major cause of morbidity and mortality in systemic scleroderma. [[Interstitial lung disease]] and pulmonary arterial hypertension are common complications. | Lung involvement is a major cause of morbidity and mortality in systemic scleroderma. [[Interstitial lung disease]] and pulmonary arterial hypertension are common complications. | ||
===Gastrointestinal Tract=== | ===Gastrointestinal Tract=== | ||
The gastrointestinal tract can be affected at any level, leading to symptoms such as [[dysphagia]], [[gastroesophageal reflux disease]], and [[malabsorption]]. | The gastrointestinal tract can be affected at any level, leading to symptoms such as [[dysphagia]], [[gastroesophageal reflux disease]], and [[malabsorption]]. | ||
===Renal Involvement=== | ===Renal Involvement=== | ||
Renal crisis, characterized by sudden onset of [[hypertension]] and renal failure, is a serious complication of systemic scleroderma. | Renal crisis, characterized by sudden onset of [[hypertension]] and renal failure, is a serious complication of systemic scleroderma. | ||
==Diagnosis== | ==Diagnosis== | ||
Diagnosis of systemic scleroderma is based on clinical features, laboratory tests, and imaging studies. The presence of specific autoantibodies can aid in diagnosis. [[Skin biopsy]] may be performed to confirm the diagnosis. | Diagnosis of systemic scleroderma is based on clinical features, laboratory tests, and imaging studies. The presence of specific autoantibodies can aid in diagnosis. [[Skin biopsy]] may be performed to confirm the diagnosis. | ||
==Treatment== | ==Treatment== | ||
There is no cure for systemic scleroderma, but treatment focuses on managing symptoms and preventing complications. [[Immunosuppressive therapy]], [[vasodilators]], and [[antifibrotic agents]] are commonly used. Physical therapy and occupational therapy can help maintain mobility and function. | There is no cure for systemic scleroderma, but treatment focuses on managing symptoms and preventing complications. [[Immunosuppressive therapy]], [[vasodilators]], and [[antifibrotic agents]] are commonly used. Physical therapy and occupational therapy can help maintain mobility and function. | ||
==Prognosis== | ==Prognosis== | ||
The prognosis of systemic scleroderma varies depending on the extent of organ involvement and the presence of complications. Early diagnosis and treatment can improve outcomes. | The prognosis of systemic scleroderma varies depending on the extent of organ involvement and the presence of complications. Early diagnosis and treatment can improve outcomes. | ||
==See also== | |||
== | |||
* [[Autoimmune disease]] | * [[Autoimmune disease]] | ||
* [[Connective tissue disease]] | * [[Connective tissue disease]] | ||
* [[Raynaud's phenomenon]] | * [[Raynaud's phenomenon]] | ||
* [[Pulmonary arterial hypertension]] | * [[Pulmonary arterial hypertension]] | ||
{{Medical resources | |||
| ICD11 = {{ICD11|4A42}} | |||
| ICD10 = {{ICD10|M34}} | |||
| ICD9 = {{ICD9|710.1}} | |||
| ICDO = | |||
| OMIM = 181750 | |||
| MedlinePlus = 000429 | |||
| eMedicineSubj = derm | |||
| eMedicineTopic = 677 | |||
| eMedicine_mult = {{eMedicine2|ped|2197}} | |||
| DiseasesDB = 12845 | |||
| MeshID = D012595 | |||
}} | |||
{{Systemic connective tissue disorders}} | |||
{{Medicine}} | |||
{{DEFAULTSORT:Systemic Scleroderma}} | |||
[[Category:Ailments of unknown cause]] | |||
[[Category:Mucinoses]] | |||
[[Category:Connective tissue diseases]] | |||
[[Category:Autoimmune diseases]] | |||
[[Category:Disorders of fascia]] | |||
[[Category:Systemic connective tissue disorders]] | |||
[[Category:Autoimmune diseases]] | [[Category:Autoimmune diseases]] | ||
[[Category:Connective tissue diseases]] | [[Category:Connective tissue diseases]] | ||
[[Category:Dermatology]] | [[Category:Dermatology]] | ||
Latest revision as of 15:31, 12 April 2025
Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD's medical weight loss NYC, sleep center NYC
Philadelphia medical weight loss and Philadelphia sleep clinics
| Systemic scleroderma | |
|---|---|
| File:Riehl Zumbusch Tafel LXIX (1).jpg | |
| Synonyms | Systemic sclerosis |
| Pronounce | |
| Specialty | Rheumatology |
| Symptoms | Skin thickening, Raynaud's phenomenon, joint pain, esophageal dysmotility, pulmonary fibrosis |
| Complications | Pulmonary hypertension, renal crisis, heart failure |
| Onset | Typically between ages 30 and 50 |
| Duration | Chronic |
| Types | N/A |
| Causes | Autoimmune disease |
| Risks | Genetic predisposition, environmental factors |
| Diagnosis | Clinical examination, autoantibody testing, skin biopsy |
| Differential diagnosis | Localized scleroderma, lupus erythematosus, dermatomyositis |
| Prevention | N/A |
| Treatment | Immunosuppressive therapy, physical therapy, antifibrotic agents |
| Medication | Methotrexate, mycophenolate mofetil, cyclophosphamide |
| Prognosis | Variable; depends on organ involvement |
| Frequency | 1 in 10,000 people |
| Deaths | |
A chronic connective tissue disease
Systemic scleroderma, also known as systemic sclerosis, is a chronic autoimmune disease characterized by fibrosis (thickening and hardening) of the skin and internal organs. It is a complex condition that affects the connective tissue and can lead to significant morbidity and mortality.
Pathophysiology[edit]
Systemic scleroderma is primarily characterized by excessive collagen deposition in the skin and other organs. The exact cause of the disease is unknown, but it is believed to involve a combination of genetic, environmental, and immunological factors. The disease process involves autoimmunity, vascular dysfunction, and fibrosis.
Autoimmunity[edit]
In systemic scleroderma, the immune system mistakenly attacks the body's own tissues, leading to inflammation and fibrosis. Autoantibodies such as anti-centromere and anti-Scl-70 are often present in patients and can be used as markers for diagnosis.
Vascular Dysfunction[edit]
Vascular abnormalities are a hallmark of systemic scleroderma. Patients often experience Raynaud's phenomenon, which is characterized by episodic vasospasm of the fingers and toes. Over time, persistent vascular damage can lead to digital ulcers and pulmonary arterial hypertension.
Fibrosis[edit]
Fibrosis in systemic scleroderma results from the overproduction of collagen by fibroblasts. This leads to thickening and hardening of the skin and can affect internal organs such as the lungs, heart, kidneys, and gastrointestinal tract.
Clinical Features[edit]
The clinical presentation of systemic scleroderma is highly variable and can range from mild skin involvement to severe multi-organ disease.
Skin Involvement[edit]
The skin changes in systemic scleroderma typically begin with edema and progress to sclerosis. The skin becomes tight, shiny, and may restrict movement. Telangiectasia and calcinosis can also occur.
Musculoskeletal System[edit]
Patients may experience joint pain, stiffness, and muscle weakness. Arthralgia and myopathy are common symptoms.
Pulmonary Involvement[edit]
Lung involvement is a major cause of morbidity and mortality in systemic scleroderma. Interstitial lung disease and pulmonary arterial hypertension are common complications.
Gastrointestinal Tract[edit]
The gastrointestinal tract can be affected at any level, leading to symptoms such as dysphagia, gastroesophageal reflux disease, and malabsorption.
Renal Involvement[edit]
Renal crisis, characterized by sudden onset of hypertension and renal failure, is a serious complication of systemic scleroderma.
Diagnosis[edit]
Diagnosis of systemic scleroderma is based on clinical features, laboratory tests, and imaging studies. The presence of specific autoantibodies can aid in diagnosis. Skin biopsy may be performed to confirm the diagnosis.
Treatment[edit]
There is no cure for systemic scleroderma, but treatment focuses on managing symptoms and preventing complications. Immunosuppressive therapy, vasodilators, and antifibrotic agents are commonly used. Physical therapy and occupational therapy can help maintain mobility and function.
Prognosis[edit]
The prognosis of systemic scleroderma varies depending on the extent of organ involvement and the presence of complications. Early diagnosis and treatment can improve outcomes.
See also[edit]
| Systemic connective tissue disorders | ||||||
|---|---|---|---|---|---|---|
|
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