Aut oimmune My ositis and Ov erlap Syndromes

BlueDot P olymy ositis/Scleroderma8

For the differential diagnosis of aut oimmune my ositis and B l u e D o t k i t f o r o v erlap syndrome. t h e d e t e ct i o n o f m y o s i t i s / s c l e r o d e rm a - r e la t e d a u t o a n t i b o d i es i n h u m a n se ru m .

>8 diff er ent my ositis / Scl er oderma t ar get antigens c o v er ed in one singl e t es t

• Jo-1 • PL-7 • PL-12 • SRP • Mi-2 • Ku • PM-Scl • Scl-70

D-t ek sa Rue Bris sel ot 11 - B7000 Mons - BELGIUM Tel. +32-65 84 18 88 Fax. +32-65 84 26 63 www.d-t ek.be

11 / 2008

prognosisMyositis. with patients all of respond poorly to immunosuppressive therapy. They have the poorest the have They therapy. immunosuppressive to poorly respond

namto ad rqet ada ivleet Ptet generally Patients involvement. cardiac frequent and inflammation

SRP syndrome » is a severe form of with acute myositic myositic acute with polymyositis of form severe a is » syndrome SRP

not exhibit involvement of the joints, lungs or skin. The classic « anti- « classic The skin. or lungs joints, the of involvement exhibit not

with aminoacyl-tRNA synthetase , SRP positive patients do do patients positive SRP antibodies, synthetase aminoacyl-tRNA with

the subgroup of Jo-1 negative patients. In contrast to patients patients Myositis to contrast In patients. negative Jo-1 of subgroup the

Myositis patients are positive for anti-SRP antibodies, rising to 18 % in in % 18 to rising antibodies, anti-SRP for positive are patients Myositis SRP

than myositis associated with other antibodies (e.g. PM-Scl). (e.g. antibodies other with associated myositis than

PL-7 or PL-12 associated myositis appears to be more difficult to treat to difficult more be to appears myositis PL-12PL-7 associated or differential diagnosis and therapy of myositis of unclear origin. Indeed Indeed origin. unclear of myositis of therapy and diagnosis differential

idiopathic myositis (around 2-3 %), are important markers for the the for markers important are %), 2-3 (around myositis idiopathic

PL-7

episodes and a poor prognosis. poor a and episodes

useful useful prognostic marker for more severe clinical course, frequent active

of myositis and fibrosing alveolitis. Furthermore a as consideredalveolitis. fibrosing is and Jo-1 myositis of Jo-1

myositis and myositis in overlap syndromes. overlap in myositis and myositis

Antibodies to Jo-1, PL-7 and PL-12 are highly specific for idiopathic idiopathic for specific highly are PL-12 and PL-7 Jo-1, to Antibodies (Threonyl-tRNA-synthetase) and PL-12 and (Threonyl-tRNA-synthetase) synthetase). (Alanyl-tRNA

tRNA-synthetases. These include Jo-1 (Histidyl-tRNA-synthetase), PL-7 (Histidyl-tRNA-synthetase), Jo-1 include These tRNA-synthetases.

important important antigens for the serological diagnosis of PM/DM are aminoacyl-

occurrence of specific antibodies to different antigens. The most most The antigens. different to antibodies specific of occurrence A striking feature of PM/DM, including overlap syndromes, is the the is syndromes, overlap including PM/DM, of feature striking A

examination, electromyography, examination, serological and biopsy findings.

Diagnosis Diagnosis of PM/DM is based on a combination of medical history, physical

Diagnosis Diagnosis

cytotoxic substances or even plasmapheresis. even or substances cytotoxic

by application of corticoïds but severe cases may require application of of application require may cases severe but corticoïds of application by

case case for most autoimmune diseases. Skin irritations are easily managed

cases. Standard therapy of PM/DM is immunosuppression as it is the the is it as immunosuppression is PM/DM of therapy Standard cases.

slow the progression of the disease and reduce the symptoms in most most in symptoms the reduce and disease the of progression the slow Although Although therapy is rather unspecific, an early treatment can significantly

of an interstitial pulmonary disease. pulmonary interstitial an of

lead to various complications with poor prognosis, like the development development the prognosis, poor like with complications various to lead The course of the PM/DM can vary from The forms mild course of to the severe which PM/DM may can vary

5 to 15 (juvenile form) and between ages form) 55. – 35 between and (juvenile 15 to 5

women are more affected than men. The age of predilection is from agefrom is predilection of age The men. affected than moreare women

strong radiations. Hormonal status may also be of relevance since since relevance of be also may status Hormonal radiations. strong

genetic predisposition and exposure to external factors like drugs or or drugs like factors external to exposure and predisposition genetic

diseases – are of an uncertain etiology. Probable causes include a a include causes Probable etiology. uncertain an of are – diseases

PM/DM are connective tissue diseases which – like most autoimmune autoimmune most like – which diseases tissue connective are PM/DM

Etiology and Pathology and Etiology

scleroderma

since since

threatening state. The diagnosis of PM/DM however is difficult difficult is however PM/DM of diagnosis The state. threatening

igoe ad rae rpdy pors oe tm t a life- a to time over progress rapidly, treated and diagnosed

The illnesses severely reduce the quality of life and may, if not not if may, and life of quality the reduce severely illnesses The skin, although other organs may also be involved (lung, heart...). heart...). (lung, involved be also may organs other although skin,

mune diseases which primarily affect the muscles and/or the the and/or muscles the affect primarily which diseases mune

oyysts n Draoysts (PM/DM) and Polymyositis

antibodies are highly specific for Polymyositis. About 5 % of of % 5 About Polymyositis. for specific highly are antibodies

and and

antibodies are found in about 60 % of patients with a combination combination a with patients of % 60 about foundare in antibodies

overlap

PL-12

namely,are frequent.

syndromes with features of other diseases, diseases, other of features with syndromes

antibodies, although less frequently detected in in detected frequently less although antibodies,

r autoim are

-

24 tests 24

PMS8D

oe Product Code

Availableproducts a

single test. single

antibodies Jo-1, PL-7, PL-12, SRP, Mi-2, Ku, PM-Scl and Scl-70 in a a in Scl-70 and PM-Scl Ku, SRP, Mi-2, PL-12,PL-7, Jo-1, antibodies

cost effectiveness for the reliable detection of the eight most relevant relevant most eight the of detection reliable the for effectiveness cost

of unclear origin, in one easy test run. It offers easy handling and and handling easy offers It run. test easy one in origin, unclear of

from PM/DM to scleroderma, through overlap syndrome and myositis myositis and syndrome overlap through scleroderma, to PM/DM from

leo Polymyositis/Scleroderma BlueDot

BlueDot Polymyositis/S BlueDot

r ascae wt a eee ytmc ore n a or prognosis. poor a and course systemic severe a with associated are (scleroderma). They are essentially prevalent in the diffuse forms and and forms diffuse the in prevalent essentially are They (scleroderma).

Scl-70

of another connective tissue disease (e.g. SLE) has been ruled out. ruled been has SLE) (e.g. disease tissue connective another of

scleroderma overlap is diagnostically relevant only after the possibility possibility the after only relevant diagnostically is overlap scleroderma

eemnto o K atbde i cs o ssiin f polymyositis/ of suspicion of case in antibodies Ku of determination

scleroderma overlap syndrome. However their specificity is low and the the and low is specificity their However syndrome. overlap scleroderma Ku

to that of or scleroderma. or dermatomyositis juvenile of that to

disease of childhood. The clinical course is relatively benign compared benign relatively is course clinical The childhood. of disease in children) which appears to be the most common scleroderma-like common most the be to appears which children) in

juvenile juvenile (overlap syndrome, mild scleroderma and myositis

and and scleroderma ( 1-16 %). Interestingly PM-Scl is a reliable marker for polymyositis/scleroderma overlap polymyositis/scleroderma syndrome overlap (20-25 %), PM/DM (8-12 %)

PM-Scl

glucocorticosteroidstherefore and prognosis. good a have to tend

for Mi-2 generally have a relatively mild clinical course, respond well to to well respond course, clinical mild relatively a generallyhave for Mi-2

aminoacyl-tRNA aminoacyl-tRNA synthetase antibodies (Jo-1, PL-7, PL-12), those positive

dermatomyosotis. Compared to myositis patients who test positive for positive test who patients myositis to Compared dermatomyosotis.

Mi-2

Negative

PositiveControl

antibodies are detectable in 5-25 % of patients with polymyositis/ with patients of % 5-25 in detectable are antibodies

Product

Strip number Strip

antibodies are detected almost exclusively in patients with with patients in exclusively almost detected are antibodies

niois r hgl seic o ssei sclerosis systemic for specific highly are antibodies

Control

-24

antibodies are found almost exclusively in patients with with patients in exclusively almost found are antibodies

Antigens

name

BlueDot Features BlueDot

Polymyositis / Scleroderma BlueDot

Scl-70

PM-Scl

Ku

Mi-2

SRP

PL-12

PL-7 Jo-1

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cleroderma

8

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8

PositivePatient

Ku Jo-1

A

ntigens

•

(Mi-2)

PM-Scl

•

ExemplesInterpretation of

PL-7

www.d-tek.be

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-

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-

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- -

8

•

•

PL-12

Scl-70

ValidTEST

•

PM-Scl

Scl-70

PL-12

Mi-2

Jo-1

PL-7

SRP

SRP

Ku

•

Mi-2

N

- -

-

-

- -

- -

Patient

egative