Sandbox: Shamila: Difference between revisions

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	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
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Natural History, Complications and Prognosis
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Revision as of 20:47, 26 March 2018

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2], Jesus Rosario Hernandez, M.D. [3], Mahshid Mir, M.D. [4]

Synonyms and keywords: MCTD; mixed connective tissue disorder; Sharp's syndrome

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 joint pain/swelling, Raynaud phenomenon, muscle inflammation, and scarring of the skin of the hand. It does not typically cause kidney disease or seizures. Distinguishing laboratory characteristics are a positive, speckled anti-nuclear antibody and an anti-U1-RNP antibody.^[1]

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.

Fibrous Pericarditis in MCTD
Cellular aggregates in perivascular space and adjacent myocardium
Cross Section of Intramural Coronary Artery
Small pulmonary artery in patient with MCTD and pulmnonary hypertension

Historical Perspective

Classification

Pathophysiology

Associated Conditions:
- Systemic lupus erythematosus–like findings
  - Polyarthritis
  - Lymphadenopathy
  - Facial erythema
  - Pericarditis or pleuritis
  - Leukopenia (<4,000/mm3) or thrombocytopenia (<100,000/mm3)
- 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)
- Polymyositis-like findings

Causes

Differentiating Mixed Connective Tissue Disease from other Diseases

Epidemiology and Demographics

Age

Gender

Race

Risk Factors

Natural History, Complications and Prognosis

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)^[2]

A teenager with MCTD presented as ACS before PCI
A teenager with MCTD presented as ACS post PCI

Diagnosis

Diagnostic Criteria

Symptoms

Raynaud’s phenomenon
Swollen fingers or hands
Muscle weakness

Physical examination

Gallery

Trunk

url = http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=294>

Extremities

Laboratory Findings

Elevated serum level of muscle enzymes (creatine kinase)
MCTD is characterized by anti–U1 small nuclear RNP positivity.

Imaging Findings

- Myogenic pattern on electromyogram

Other Diagnostic Studies

- Hypomotility or dilatation of esophagus

References

↑ Venables PJ. Mixed connective tissue disease. Lupus. 2006;15(3):132-7. PMID 1663436
↑ 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

[1] Venables PJ. Mixed connective tissue disease. Lupus. 2006;15(3):132-7. PMID 1663436

[2] 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

[1]

[2]

@@ Line 1: / Line 1: @@
-{{familytree/start |summary=Lack of CFTR normal activity}}
+__NOTOC__
-{{familytree | | | | | | | A02 | | | |A02=Lack of CFTR normal activity}}
+{{Mixed connective tissue disease}}
-{{familytree | | |,|-|-|-|-|^|-|-|-|-|-|.| | | | | | }}
+{{SI}}
-{{familytree | | B01 | | | | | | | | | B02 | | |B01=Less chloride secretion|B02=More sodium absorption}}
+{{CMG}}; {{AE}} {{CZ}}, {{JH}}, {{MIR}}
-{{familytree | | |!| | | | | | | | | | |!| | | | }}
-{{familytree | | C01 | | | | | | | | | C02 | | |C01=Less water transport into the epithelial surface layer|C02=Excessive sodium and water absorption through the epithelial channel}}
+{{SK}} MCTD; mixed connective tissue disorder; Sharp's syndrome
+==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">
+<gallery heights="225" widths="225">
+image:Fibrous Pericarditis in MCTD.jpg|Fibrous Pericarditis in MCTD
+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
+image:Small pulmonary artery in patient with MCTD and pulmonary hypertension.jpg|Small pulmonary artery in patient with MCTD and pulmnonary hypertension
+</gallery>
+</div>
+==Historical Perspective==
+==Classification==
+==Pathophysiology==
+*Associated Conditions:
+**[[Systemic lupus erythematosus]]–like findings
+***[[Polyarthritis]]
+***[[Lymphadenopathy]]
+***Facial [[erythema]]
+***[[Pericarditis]] or [[pleuritis]]
+***[[Leukopenia]] (<4,000/mm3) or thrombocytopenia (<100,000/mm3)
+**[[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)
+**[[Polymyositis]]-like findings
+==Causes==
+==Differentiating Mixed Connective Tissue Disease from other Diseases==
+==Epidemiology and Demographics==
+===Age===
+===Gender===
+===Race===
+==Risk Factors==
+==Natural History, Complications and Prognosis==
+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">
+<gallery heights="225" widths="225">
+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>
+==Diagnosis==
+===Diagnostic Criteria===
+===Symptoms===
+*[[Raynaud’s phenomenon]]
+*Swollen fingers or hands
+*Muscle weakness
+===Physical examination===
+====Gallery====
+=====Trunk=====
+<gallery>
+Image: Mixed_connective_tissue_disease_01.jpg|Mixed connective tissue disease. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=12  With permission from Dermatology Atlas.]''<ref name="www.atlasdermatologico.com.br">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=294>
+</gallery>
+=====Extremities=====
+<gallery>
+Image: Mixed_connective_tissue_disease_02.jpg|Mixed connective tissue disease. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=12  With permission from Dermatology Atlas.]''<ref name="www.atlasdermatologico.com.br">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=294>
+Image: Mixed_connective_tissue_disease_03.jpg|Mixed connective tissue disease. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=12  With permission from Dermatology Atlas.]''<ref name="www.atlasdermatologico.com.br">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=294>
+</gallery>
+===Laboratory Findings===
+*Elevated serum level of muscle enzymes (creatine kinase)
+*MCTD is characterized by anti–U1 small nuclear RNP positivity.
+===Imaging Findings===
+**Myogenic pattern on electromyogram
+===Other Diagnostic Studies===
+**Hypomotility or dilatation of esophagus
+== References ==
+{{reflist|2}}

Sandbox: Shamila: Difference between revisions

Revision as of 20:47, 26 March 2018

Overview

Pathophysiology

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Mixed Connective Tissue Disease from other Diseases

Epidemiology and Demographics

Age

Gender

Race

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Criteria

Symptoms

Physical examination

Gallery

Trunk

Extremities

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

References

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