Antibodies
What antibodies lead to a painful myopathy
INIM - HMGCR and SRP
Name the flower that lent its name to a pathognomonic rash in DM
Heliotrope (purple color)
True or False: Patients with PM do not get cancer-associated myositis
False: Account for 20% of cases while DM accounts for the other 80% of cases
Describe muscle weakness
Insidious, painless weakness affecting distal muscle groups eventually, but can involve proximal later
autoantibody that is most associated with polymyositis, interstitial lung disease, Raynaud's, fevers and mechanic's hands
anti-synthetase autoantibodies or JO-1
Name all the pathognomonic rashes of DM
Heliotrope, Gottron's papules, V-sign, Shael-sign, holster-sign
Others:
calcinosis cutis in JDM, poikiloderma, mechanics hands
True or False: About 30% of patients with PM/DM have cancer-associated myositis
False - about 10-20%
True or False: CK is usually 10x the upper limit of normal in IBM
False: It is almost always <1000 and only high in 20-30% of cases
What is the autoantibody most associated with juvenile DM, contractures and calcinosis?
anti-NXP-2/anti-MJ?
True or False: Some patients with DM have negative MSA panels
True: Likely more MSAs that have not been discovered yet.
What two types of cancer are most often found. Think broad.
Adenocarcinomas and hematologic
What other autoimmune CTD has IBM been associated with
Sjogrens
What is the antibody is associated with more severe disease, especially for the heart?
signal recognition particle (anti-SNP)
True or False: DM causes dysphagia through impaired peristalsis and can be seen on esophageal manometry.
False, it is due to weakness in voluntary muscles of the mouth, pharynx, and upper esophageal sphincter. Leads to oropharyngeal dysphagia.
Of patient with cancer-associated myositis, what is the percent of having cancer or developing it in the first year of diagnosis
68%
True or False: EMG findings will show both myopathic and neuropathic changes
True
What are the four clinical features of having positive anti-MDA5/antiCAMD-140 antibodies associated with
dermatomyositis, amyopathic disease, mucocutaneous lesions, severe lung disease
What are the two peaks of age of onset for dermatomyositis
5-10 years and 50-60 years
Name the two MSAs that are negatively associated with cancer and name the two that are most associated
TIF-1gamma and NXP2 are highly associated and Jo1 and Mi-2 are not.
Name the MSA associated with IBM
anti-cN1a or anti-NT5C1A and is found in 33-60% of patients
These are the HLA haplotypes most associated with caucasian dermatomyositis patients.
HLA-DRB1*0301 and HLA-DQA1*0501