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{{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]]==
*[[Raynaud’s phenomenon]]
 
*Swollen fingers or hands
==[[Differentiating Mixed connective tissue disease from other diseases|Differentiating Any Disease from other Diseases]]==
 
==[[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]]


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{{WikiDoc Sources}}
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Latest revision as of 15:36, 8 May 2018

Mixed connective tissue disease Microchapters

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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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