Mixed connective tissue disease pathophysiology: Difference between revisions
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*Trigeminal neuralgia | *Trigeminal neuralgia | ||
==Gross Pathology== | ==Gross Pathology== | ||
* | *In MCTD, gross pathology of skin may include:<ref name="pmid9185904">{{cite journal |vauthors=Magro CM, Crowson AN, Regauer S |title=Mixed connective tissue disease. A clinical, histologic, and immunofluorescence study of eight cases |journal=Am J Dermatopathol |volume=19 |issue=3 |pages=206–13 |date=June 1997 |pmid=9185904 |doi= |url=}}</ref> | ||
**Photo-distributed erythematosus annular | |||
**Papulosquamous lesions | |||
==Microscopic Pathology== | ==Microscopic Pathology== | ||
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**Alveolar septal infiltration by lymphocytes and plasma cells | **Alveolar septal infiltration by lymphocytes and plasma cells | ||
**Deposition of type III collagen | **Deposition of type III collagen | ||
*In MCTD, histopathological abnormalities of skin lesions are similar to that of subacute cutaneous lupus erythematosus (SCLE), include: | |||
**Poor and lichenoid interface dermatitis | |||
**Suprabasilar exocytosis around necrotic keratinocytes (in the absence of deep periadnexal or perivascular extension or conspicuous follicular plugging) | |||
==References== | ==References== | ||
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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
Pathophysiology
Pathogenesis
The pathogenesis of mixed connective tissue disease is as follows:[1][2][3][4]
- MCTD is a systemic autoimmune disease that characterized by overlapping features between two or more systemic autoimmune diseases and the presence of antibodies against the U1 small nuclear ribonucleoprotein autoantigen (U1snRNP).
- MCTD is characterized by clinical features seen in systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), dermatomyositis (DM), polymyositis, and scleroderma.
- Main pathogenetic mechanisms in mixed connective tissue disease include:
- Vasculopathy which leads to tissue ischemia
- Immunological and inflammatory processes deriving from autoimmunity
- Excessive fibrosis caused by redundant synthesis of collagen and other matrix proteins
- In MCTD, components of U1-snRNP are important for triggering immune responses. Anti-RNP has a central pathogenic role and may contribute to disease manifestations.
- Development of pulmonary hypertension and interstitial lung disease are the most frequent cause of death.
Genetics
- In MCTD, the frequency of HLA-DR4 is increased compared with healthy controls in worldwide population-based studies.[4]
- A significant association of U1RNP disease with HLA-DR4 and DR154-61 is noted.
Associated Conditions
In MCTD associated conditions include:[3]
- Secondary Sjogren’s syndrome
- Trigeminal neuralgia
Gross Pathology
- In MCTD, gross pathology of skin may include:[5]
- Photo-distributed erythematosus annular
- Papulosquamous lesions
Microscopic Pathology
- In MCTD, histopathological abnormalities of interestital lung disease are similar to those seen in idiopathic pulmonary fibrosis (IPF), including:[6]
- Alveolar septal infiltration by lymphocytes and plasma cells
- Deposition of type III collagen
- In MCTD, histopathological abnormalities of skin lesions are similar to that of subacute cutaneous lupus erythematosus (SCLE), include:
- Poor and lichenoid interface dermatitis
- Suprabasilar exocytosis around necrotic keratinocytes (in the absence of deep periadnexal or perivascular extension or conspicuous follicular plugging)
References
- ↑ Tani C, Carli L, Vagnani S, Talarico R, Baldini C, Mosca M, Bombardieri S (2014). "The diagnosis and classification of mixed connective tissue disease". J. Autoimmun. 48-49: 46–9. doi:10.1016/j.jaut.2014.01.008. PMID 24461387.
- ↑ Thongpooswan S, Tushabe R, Song J, Kim P, Abrudescu A (August 2015). "Mixed Connective Tissue Disease and Papillary Thyroid Cancer: A Case Report". Am J Case Rep. 16: 517–9. doi:10.12659/AJCR.894176. PMC 4530986. PMID 26245523.
- ↑ 3.0 3.1 Latuśkiewicz-Potemska J, Zygmunt A, Biernacka-Zielińska M, Stańczyk J, Smolewska E (October 2013). "Mixed connective tissue disease presenting with progressive scleroderma symptoms in a 10-year-old girl". Postepy Dermatol Alergol. 30 (5): 329–36. doi:10.5114/pdia.2013.38365. PMC 3858664. PMID 24353496.
- ↑ 4.0 4.1 Ciang NC, Pereira N, Isenberg DA (March 2017). "Mixed connective tissue disease-enigma variations?". Rheumatology (Oxford). 56 (3): 326–333. doi:10.1093/rheumatology/kew265. PMID 27436003.
- ↑ Magro CM, Crowson AN, Regauer S (June 1997). "Mixed connective tissue disease. A clinical, histologic, and immunofluorescence study of eight cases". Am J Dermatopathol. 19 (3): 206–13. PMID 9185904.
- ↑ Bodolay E, Szekanecz Z, Dévényi K, Galuska L, Csípo I, Vègh J, Garai I, Szegedi G (May 2005). "Evaluation of interstitial lung disease in mixed connective tissue disease (MCTD)". Rheumatology (Oxford). 44 (5): 656–61. doi:10.1093/rheumatology/keh575. PMID 15716315.