[Autoantibody profile in myositis].

[inclusion body myositis]

Patients suffering from muscular symptoms or with an increase of creatine kinase levels may present a myopathy . In such situations , clinicians have to confirm the existence of a myopathy and determine if it is an acquired or a genetic muscular disease . In the presence of an acquired myopathy after having ruled out an infectious , a toxic agent or an endocrine cause , physicians must identify which type of idiopathic myopathy the patient is presenting : either a myositis including polymyositis , dermatomyositis , and inclusion body myositis , or an immune-mediated necrotizing myopathy . Histopathology examination of a muscle biopsy is determinant but detection of autoantibody is now also crucial . The myositis -specific antibodies and myositis -associated antibodies lead to a serologic approach complementary to the histological classification , because strong associations of myositis -specific antibodies with clinical features and survival have been documented . The presence of anti-synthetase antibodies is associated with an original histopathologic pattern between polymyositis and dermatomyositis , and defines a syndrome where interstitial lung disease drives the prognosis . Anti-MDA-5 antibody are specifically associated with dermatomyositis , and define a skin -lung syndrome with a frequent severe disease course . Anti-TIF 1 -γ is also associated with dermatomyositis but its presence is frequently predictive of a cancer association whereas anti-MI 2 is associated with the classical dermatomyositis . Two specific antibodies , anti-SRP and anti-HMGCR , are observed in patients with immune-mediated necrotizing myopathies and may be very useful to distinguish acquired myopathies from dystrophic muscular diseases in case of a slow onset and to allow the initiation of effective therapy .

Diseases
Validation

Diseases presenting "cancer" symptom

achondroplasia

acute rheumatic fever

adrenal incidentaloma

alpha-thalassemia

benign recurrent intrahepatic cholestasis

cadasil

canavan disease

carcinoma of the gallbladder

cholangiocarcinoma

coats disease

congenital adrenal hyperplasia

congenital diaphragmatic hernia

cowden syndrome

cushing syndrome

cutaneous mastocytosis

dedifferentiated liposarcoma

dystrophic epidermolysis bullosa

epidermolysis bullosa simplex

erdheim-chester disease

erythropoietic protoporphyria

esophageal adenocarcinoma

esophageal carcinoma

esophageal squamous cell carcinoma

familial hypocalciuric hypercalcemia

familial mediterranean fever

gm1 gangliosidosis

heparin-induced thrombocytopenia

hereditary cerebral hemorrhage with amyloidosis

hirschsprung disease

hodgkin lymphoma, classical

inclusion body myositis

junctional epidermolysis bullosa

kabuki syndrome

kallmann syndrome

kindler syndrome

lamellar ichthyosis

liposarcoma

locked-in syndrome

lymphangioleiomyomatosis

monosomy 21

neuralgic amyotrophy

oculocutaneous albinism

oligodontia

oral submucous fibrosis

papillon-lefèvre syndrome

pendred syndrome

pleomorphic liposarcoma

primary effusion lymphoma

proteus syndrome

pyomyositis

pyruvate dehydrogenase deficiency

severe combined immunodeficiency

sneddon syndrome

systemic capillary leak syndrome

triple a syndrome

von hippel-lindau disease

waldenström macroglobulinemia

well-differentiated liposarcoma

werner syndrome

wiskott-aldrich syndrome

wolf-hirschhorn syndrome

x-linked adrenoleukodystrophy

This symptom has already been validated