Reynolds syndrome: Difference between revisions

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[[File:Autosomal_dominant_-_en.svg|Autosomal dominant - en|thumb]] '''Reynolds syndrome''' is a rare [[autoimmune disease]] characterized by the combination of [[primary biliary cholangitis]] (PBC) and [[systemic sclerosis]] (SSc). It is named after the American physician [[Telfer B. Reynolds]], who first described the syndrome.
{{SI}}
 
{{Infobox medical condition
| name            = Reynolds syndrome
| image          = [[File:Autosomal_dominant_-_en.svg|200px]]
| caption        = [[Autosomal dominant]] pattern is the mode of inheritance
| field          = [[Rheumatology]], [[Hepatology]]
| symptoms        = [[Scleroderma]], [[Primary biliary cholangitis]]
| complications  = [[Pulmonary hypertension]], [[Liver cirrhosis]]
| onset          = Middle age
| duration        = Chronic
| causes          = Unknown
| risks          = [[Autoimmune disease]]
| diagnosis      = Clinical evaluation, [[Antinuclear antibody]] test
| differential    = [[CREST syndrome]], [[Systemic sclerosis]]
| treatment      = Symptomatic treatment, [[Immunosuppressive drug]]s
| medication      = [[Ursodeoxycholic acid]], [[Calcium channel blocker]]s
| prognosis      = Variable, depends on organ involvement
| frequency      = Rare
}}
'''Reynolds syndrome''' is a rare [[autoimmune disease]] characterized by the combination of [[primary biliary cholangitis]] (PBC) and [[systemic sclerosis]] (SSc). It is named after the American physician [[Telfer B. Reynolds]], who first described the syndrome.
==Presentation==
==Presentation==
Patients with Reynolds syndrome typically present with symptoms of both primary biliary cholangitis and systemic sclerosis. Common symptoms include:
Patients with Reynolds syndrome typically present with symptoms of both primary biliary cholangitis and systemic sclerosis. Common symptoms include:
* [[Fatigue]]
* [[Fatigue]]
* [[Pruritus]] (itching)
* [[Pruritus]] (itching)
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* [[Sclerodactyly]] (thickening and tightening of the skin on the fingers)
* [[Sclerodactyly]] (thickening and tightening of the skin on the fingers)
* [[Telangiectasia]] (small dilated blood vessels near the surface of the skin)
* [[Telangiectasia]] (small dilated blood vessels near the surface of the skin)
==Pathophysiology==
==Pathophysiology==
The exact cause of Reynolds syndrome is unknown, but it is believed to involve an abnormal immune response that targets the body's own tissues. In PBC, the immune system attacks the small bile ducts in the liver, leading to progressive liver damage. In SSc, the immune system targets the connective tissue, leading to fibrosis and vascular abnormalities.
The exact cause of Reynolds syndrome is unknown, but it is believed to involve an abnormal immune response that targets the body's own tissues. In PBC, the immune system attacks the small bile ducts in the liver, leading to progressive liver damage. In SSc, the immune system targets the connective tissue, leading to fibrosis and vascular abnormalities.
==Diagnosis==
==Diagnosis==
Diagnosis of Reynolds syndrome is based on clinical presentation, laboratory tests, and imaging studies. Key diagnostic criteria include:
Diagnosis of Reynolds syndrome is based on clinical presentation, laboratory tests, and imaging studies. Key diagnostic criteria include:
* Positive [[anti-mitochondrial antibodies]] (AMA) for PBC
* Positive [[anti-mitochondrial antibodies]] (AMA) for PBC
* Positive [[anti-centromere antibodies]] (ACA) for SSc
* Positive [[anti-centromere antibodies]] (ACA) for SSc
* Liver biopsy showing features of PBC
* Liver biopsy showing features of PBC
* Skin biopsy showing features of SSc
* Skin biopsy showing features of SSc
==Treatment==
==Treatment==
There is no cure for Reynolds syndrome, and treatment is primarily aimed at managing symptoms and slowing disease progression. Treatment options include:
There is no cure for Reynolds syndrome, and treatment is primarily aimed at managing symptoms and slowing disease progression. Treatment options include:
* [[Ursodeoxycholic acid]] for PBC
* [[Ursodeoxycholic acid]] for PBC
* [[Immunosuppressive drugs]] for SSc
* [[Immunosuppressive drugs]] for SSc
* [[Calcium channel blockers]] for Raynaud's phenomenon
* [[Calcium channel blockers]] for Raynaud's phenomenon
* [[Physical therapy]] for skin and joint involvement
* [[Physical therapy]] for skin and joint involvement
==Prognosis==
==Prognosis==
The prognosis for patients with Reynolds syndrome varies depending on the severity of liver and skin involvement. Early diagnosis and treatment can help manage symptoms and improve quality of life.
The prognosis for patients with Reynolds syndrome varies depending on the severity of liver and skin involvement. Early diagnosis and treatment can help manage symptoms and improve quality of life.
 
==See also==
==Related Pages==
* [[Primary biliary cholangitis]]
* [[Primary biliary cholangitis]]
* [[Systemic sclerosis]]
* [[Systemic sclerosis]]
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* [[Telangiectasia]]
* [[Telangiectasia]]


==Categories==
[[Category:Autoimmune diseases]]
[[Category:Autoimmune diseases]]
[[Category:Hepatology]]
[[Category:Hepatology]]
[[Category:Rheumatology]]
[[Category:Rheumatology]]
[[Category:Rare diseases]]
[[Category:Rare diseases]]
{{Autoimmune-disease-stub}}
{{Autoimmune-disease-stub}}
{{medicine-stub}}
{{medicine-stub}}

Latest revision as of 15:55, 8 April 2025

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC

Reynolds syndrome
Synonyms N/A
Pronounce N/A
Specialty N/A
Symptoms Scleroderma, Primary biliary cholangitis
Complications Pulmonary hypertension, Liver cirrhosis
Onset Middle age
Duration Chronic
Types N/A
Causes Unknown
Risks Autoimmune disease
Diagnosis Clinical evaluation, Antinuclear antibody test
Differential diagnosis CREST syndrome, Systemic sclerosis
Prevention N/A
Treatment Symptomatic treatment, Immunosuppressive drugs
Medication Ursodeoxycholic acid, Calcium channel blockers
Prognosis Variable, depends on organ involvement
Frequency Rare
Deaths N/A


Reynolds syndrome is a rare autoimmune disease characterized by the combination of primary biliary cholangitis (PBC) and systemic sclerosis (SSc). It is named after the American physician Telfer B. Reynolds, who first described the syndrome.

Presentation[edit]

Patients with Reynolds syndrome typically present with symptoms of both primary biliary cholangitis and systemic sclerosis. Common symptoms include:

Pathophysiology[edit]

The exact cause of Reynolds syndrome is unknown, but it is believed to involve an abnormal immune response that targets the body's own tissues. In PBC, the immune system attacks the small bile ducts in the liver, leading to progressive liver damage. In SSc, the immune system targets the connective tissue, leading to fibrosis and vascular abnormalities.

Diagnosis[edit]

Diagnosis of Reynolds syndrome is based on clinical presentation, laboratory tests, and imaging studies. Key diagnostic criteria include:

Treatment[edit]

There is no cure for Reynolds syndrome, and treatment is primarily aimed at managing symptoms and slowing disease progression. Treatment options include:

Prognosis[edit]

The prognosis for patients with Reynolds syndrome varies depending on the severity of liver and skin involvement. Early diagnosis and treatment can help manage symptoms and improve quality of life.

See also[edit]


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