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{{SI}}
{{Mixed connective tissue disease}}
{{CMG}}; '''Associate Editor:''' {{CZ}}


{{SK}} MCTD; mixed connective tissue disorder; Sharp's syndrome
{{CMG}}; {{AE}} {{SHH}}
==Overview==
'''Mixed connective tissue disease''' ('''MCTD''') or ''Sharp's syndrome'' is a human [[autoimmune disease]] in which the [[immune system]] attacks the body. MCTD combines features of [[polymyositis]], [[systemic lupus erythematosus]], and [[systemic scleroderma]] and is thus considered an [[overlap syndrome]]. MCTD commonly causes [[arthritis|joint pain/swelling]], [[Raynaud phenomenon]], [[myositis|muscle inflammation]], and [[sclerodactyly|scarring of the skin of the hand]]. It does not typically cause [[kidney]] disease or [[seizure]]s. Distinguishing laboratory characteristics are a positive, speckled [[anti-nuclear antibody]] and an [[anti-U1-RNP antibody]].<ref>Venables PJ. ''Mixed connective tissue disease.'' Lupus. 2006;15(3):132-7. PMID 1663436</ref> 
==Pathophysiology==
There are no specific histologic findings that aid in the diagnosis of [[Mixed Connective Tissue Disorder]] as a separate autoimmune disease. For example, [[nephritis]] in [[MCTD]] is usually indistinguishable from [[lupus]] nephritis.


<div align="left">
==[[Mixed connective tissue disease overview|Overview]]==
<gallery heights="225" widths="225">
 
image:Fibrous Pericarditis in MCTD.jpg|Fibrous Pericarditis in MCTD
==[[Mixed connective tissue disease historical perspective|Historical Perspective]]==
image:Cellular aggregates in perivascular space and adjacent myocardium.jpg|Cellular aggregates in perivascular space and adjacent myocardium
 
image:Cross Section of Intramural Coronary Artery.jpg|Cross Section of Intramural Coronary Artery
==[[Mixed connective tissue disease classification scheme|Classification]]==
image:Small pulmonary artery in patient with MCTD and pulmonary hypertension.jpg|Small pulmonary artery in patient with MCTD and pulmnonary hypertension
 
</gallery>
==[[Mixed connective tissue disease pathophysiology|Pathophysiology]]==
</div>
 
==Diagnosis==
==[[Mixed connective tissue disease causes|Causes]]==
It is characterized by -
 
*[[Raynaud’s phenomenon]]
==[[Differentiating Mixed connective tissue disease from other diseases|Differentiating Any Disease from other Diseases]]==
*Swollen fingers or hands
 
==[[Mixed connective tissue disease epidemiology and demographics|Epidemiology and Demographics]]==


===Mixed Connective Tissue Disorder Findings===
==[[Mixed connective tissue disease risk factors|Risk Factors]]==


A. [[Systemic lupus erythematosus]]–like findings
==[[Mixed connective tissue disease screening|Screening]]==
*[[Polyarthritis]]
*[[Lymphadenopathy]]
*Facial [[erythema]]
*[[Pericarditis]] or [[pleuritis]]
*[[Leukopenia]] (<4,000/mm3) or thrombocytopenia (<100,000/mm3)
B. [[Scleroderma|Progressive Systemic Sclerosis]] –like findings
*[[Sclerodactyly]]
*[[Pulmonary fibrosis]], restrictive changes of the lung (forced vital capacity <80% of predicted), or reduced carbon monoxide diffusing capacity (<70% of predicted)
*Hypomotility or dilatation of esophagus
C. [[Polymyositis]]-like findings
*Muscle weakness
*Elevated serum level of muscle enzymes (creatine kinase)
*Myogenic pattern on electromyogram


MCTD is characterized by anti–U1 small nuclear RNP positivity.
==[[Mixed connective tissue disease natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
===Cardiac Involvement in MCTD===
Cardiovascular abnormalities associated with mixed connective tissue disease are rare. Presence of any of the complications listed below indicates unfavorable prognosis;
*Acute [[pericarditis]] and/or [[pericardial effusion]],
*[[Mitral valve prolapse]],  
*Intimal hyperplasia of coronary arteries,
*Perivascular and myocardial leukocytic infiltrates,
*[[Pulmonary hypertension]].
*Coronary Artery Disease (although it is rarer, ACS may occur secondary to vascular changes and underlying CAD. The youngest patient with MCTD and ACS in literature was 18 years old)<ref>Jang J J, Olin J W, Fuster V. A teenager with mixed connective tissue disease presenting with an acute coronary syndrome. Vascular Medicine; 2004, Vol. 9 Issue 1, 31-34</ref>


<div align="left">
==Diagnosis==
<gallery heights="225" widths="225">
[[Mixed connective tissue disease history and symptoms|History and Symptoms]] | [[Mixed connective tissue disease physical examination|Physical Examination]] | [[Mixed connective tissue disease laboratory tests|Laboratory Findings]] | [[Mixed connective tissue disease electrocardiogram|Electrocardiogram]] | [[Mixed connective tissue disease chest x ray|Chest X Ray]] | [[Mixed connective tissue disease CT|CT]] | [[Mixed connective tissue disease MRI|MRI]] | [[Mixed connective tissue disease ultrasound or echocardiography|ultrasound or echocardiography]] | [[Mixed connective tissue disease other imaging findings|Other Imaging Findings]] | [[Mixed connective tissue disease other diagnostic studies|Other Diagnostic Studies]]
image:Teenager before PCI.jpg|A teenager with MCTD presented as ACS before PCI
image:Teenager post PCI.jpg|A teenager with MCTD presented as ACS post PCI
</gallery>
</div>


== References ==
==Treatment==
{{reflist|2}}
[[Mixed connective tissue disease medical therapy|Medical Therapy]] | [[Mixed connective tissue disease surgery|Surgery]] | [[Mixed connective tissue disease primary prevention|Primary Prevention]] | [[Mixed connective tissue disease secondary prevention|Secondary Prevention]] | [[Mixed connective tissue disease Future or Investigational Therapies|Future or Investigational Therapies]]


[[Category:Diseases involving the fasciae]]
==Case Studies==
[[Type page name here case study one|Case #1]]
 
 
[[Category:Medicine]]
[[Category:Up-To-Date]]
[[Category:Rheumatology]]
[[Category:Rheumatology]]
[[Category:Cardiology]]
[[fr:Syndrome de Sharp]]
[[nl:Mixed connective tissue disease]]
[[ja:混合性結合組織病]]
[[sv:Mixed Connective Tissue Disease]]


{{WH}}
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{{WikiDoc Sources}}
{{WS}}

Latest revision as of 15:36, 8 May 2018

Mixed connective tissue disease Microchapters

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Mixed connective tissue disease from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shaghayegh Habibi, M.D.[2]

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Any Disease from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | CT | MRI | ultrasound or echocardiography | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Future or Investigational Therapies

Case Studies

Case #1

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