Yes , mixed connective tissue paper disease ( MCTD ) is a rare auto immune disease the relative incidence is roughly 2/100,000 annually commonly experience in cleaning lady ( M : F – 1:9 ) and the mean age of onset is 10 - 29 long time .
The exact aetiology is not clear however , the pathophysiology is believed to be due to an autoimmune response . The body raise increase turn of antibody probably due to the hyperreactive B and T lymph cell . These antibodies bind to different cells and damage the cells which gives hike to the clinical feature film . The antibody that is unremarkably tie in with mixed connective tissue disease is uroporphyrin isomerase ribonucleoprotein ( UI - RNP ) .
A universe - based written report done in Norway had a total of 147 patients with mixed connective tissue disease and 113 were female person , the female to male ratio is 3.3 . The full stop prevalence rate of interracial connective tissue disease in Norway is 3.8 cases per 100,000 grownup universe and the one-year incidence rate is about 2.1 per million . The common clinical manifestation report wereRaynaud ’s phenomenon(99 % ) , swollen hand ( 93 % ) , arthritis ( 79 % ) , and esophageal dysmotility ( 50 % ) . Fifteen patient role in the survey were name before 18 years of age , there were 4 patients who were aged 9 long time when they were diagnosed with mixed connective tissue disease . The rest 132 patients had adult - onrush sundry connective tissue disease and the meanspirited years of diagnosis was 37.9 twelvemonth . Most of the adult patient role had their initial symptom at a mean age of 31.5 years and they were diagnosed with mixed connective tissue disease at a mean eld of 35.1 years .
A universe - based study in Olmsted County , Minnesota carried out on 50 patients with incident mixed connective tissue disease from 1985 to 2014 show that assorted connective tissue disease occurred in about 2 person per 100,000 per year . 84 % of the affected role with sundry connective tissue disease were female . The standard mortality ratio was 1.1 and it was not much of a difference when compared to the general universe . The most common initial symptoms were Raynaud ’s phenomenon ( 50 % ) , thenarthralgia(30 % ) and third swollen hand ( 16 % ) . The diagnosis was check about 3.6 years after the induction of the first symptom . Evolution into other connective tissue disorders occurred infrequently with 10 - year rate of phylogeny of 8.5 % in systemic lupus erythematosus and 6.3 % in systemic genus Scleroderma
A cogitation done on American Indian and Alaska Native adults showed the preponderance is 6.4 per 100,000 . An epidemiologic survey carried in Japan revealed the mixed connective tissue disease prevalence is 2.7 case per 100,000 populations .
The preponderance in child is abut 0.3 % of pediatrics rheumatology patients , its very rare in tiddler compare to the adults . Median age of onset is about 11 year it can range from 2 - 16 year . Even in small fry it ’s more unwashed in female person the ratio is about 1:6 .
The prognosis of assorted connective tissue disease has a favorable resultant . Most of the causa with typical clinical feature and serologic investigations occasionally transfer into scleroderma , systemic lupus erythematosus or creaky disease . interracial connective tissue disease related death hap most commonly due to pulmonic high blood pressure . Anticardiolipin antibodies and lupus anticoagulant are markers of pulmonic hypertension . Pericarditis is also another reason of dying ( 13 - 65 % ) . Other cardiac abnormalities such as conductivity abnormalities , pericardiac effusion , mitral valve descensus , diastolic dysfunction , and speed up atherosclerosis also can cause death . In three prospective study with MCT patient role followed - up for 13 - 15 years had an overall mortality pace of 10.4 % and 20 % of these deaths were due to cardiac problems
Conclusion
Yes , sundry connective tissue paper disease ( MCTD ) is a rarified car immune disease the incidence is approximately 2/100,000 annually usually come across in woman ( M : F – 1:9 ) and the mean age of onset is 10 - 29 years . A population - based study done in Norway had a total of 147 patients with assorted connective tissue disease and 113 were females , the female to male proportion is 3.3 . The point preponderance charge per unit of MCTD in Norway is 3.8 casing per 100,000 adult population and the yearly relative incidence rate is about 2.1 per million . A population - free-base field in Olmsted County , Minnesota pack out on 50 patients with incident mixed connective tissue paper disease from 1985 to 2014 show up that MCTD pass in about 2 person per 100,000 per year . A study done on American Indian and Alaska Native adult show the preponderance is 6.4 per 100,000 . An epidemiological sight carried in Japan expose the mixed connective tissue paper disease prevalence is 2.7 cases per 100,000 populations .
Also study :
