Overview
Myopathies
Name that Antibody
What if I got it?
Potpourri
100
How do inflammatory myopathies typically present?
The inflammatory myopathies commonly present with symmetric proximal muscle weakness without substantial muscle pain or tenderness; serum creatine kinase is almost universally elevated and is a marker of muscle damage.
100
What is this specific physical exam finding called and which disease process is it a hallmark for?
Gottron papules or Gottron sign, typically are erythematous to violaceous areas over the metacarpophalangeal and proximal interphalangeal joints.
Dermatomyositis
100
Dermatomyositis and Polymyositis are associated with which antibody?
Anti-Jo-1
100
There is an association between risk of malignancy and DM (3- to 12-fold that of age-matched populations) as well as, to a lesser extent, PM. The risk of malignancy is greatest in the 3 years following the diagnosis of DM or PM. Can you name 2 cancers that are commonly associated with DM or PM?
Common associated cancers are ovarian, lung, pancreas, stomach, colon, and lymphoma.
100
What is the name of the room Harry uses to teach Dumbledore’s Army?
The Room of Requirement
200
Which genetic association has been shown to have the clearest association to increased risk for inflammatory myopathies?
The clearest association is with HLA DRB1*0301, but alleles vary in populations throughout the world.
200
Anti Mi 2 are strongly associated with which disease process?
Dermatomyositis
200
Anti-histone antibodies are associated with..? Can you name 3 drugs that can cause this?
Drug-induced lupus
Hydralazine, Methyldopa, Procainamide, Isoniazid, Chlorpromozine
200
What is the mainstay of treatment for the majority of inflammatory myopathies?
Initial treatment for most inflammatory myopathies consists of glucocorticoids, with additional immunosuppressives (most commonly methotrexate and azathioprine) often required to control inflammation or serve as glucocorticoid-sparing agents. DM and PM are most responsive to immunosuppression, although DM is more responsive than PM.
200
How many times did Ross Geller get divorced on Friends?
Three times (Carol, Emily, Rachel).
300
The presence of pain or tenderness, however, should prompt considerations of other causes of myopathy. Muscle pain is more common with overexertion, muscle cramps, and injury. Muscle tenderness should prompt consideration of infectious, thyroid, or drug-induced myopathies. Name three medications that can cause drug-induced myopathies?
Statins
Antimalarials
Alcohol
Cocaine
Colchicine
Glucocorticoids
Zidovudine
300
MNM occurs in a small subset of patients with inflammatory myopathy who demonstrate prominent myonecrosis on biopsy without the inflammatory changes typical of the other inflammatory myopathies. An immune-mediated mechanism is suggested by the presence of antibodies to
anti HMG-CoA reductase antibody
300
Mixed connective tissue disease is associated with which antibody?
MCTD is usually involve a constellation of syndromes that overlap with features of multiple connective tissue disorders. Which three CTD shares their features with MCTD?
Anti-U1 ribonucleoprotein
SLE, Polymyositis, Systemic Sclerosis
300
Sjogren syndrome typically presents with sicca symptoms, including dryness of the eyes (keratoconjunctivitis sicca) and mouth (xerostomia). Patients with Sjögren syndrome are at increased risk for which type of cancer?
Non-Hodgkin lymphoma
300
The Triple Crown award is given to a horse that wins which three races?
The Kentucky Derby, Preakness Stakes, and Belmont Stakes.
400
What is the classic and dominant feature/symptom that defines polymyositis?
The symptoms of PM usually progress slowly over months, although a more rapid onset may occur. Progressive, symmetric, proximal muscle weakness is the classic and dominant feature. Activities such as arising from a chair, stair climbing, and lifting objects above shoulder height are typically affected. Neck flexors are also commonly involved. Muscle pain and tenderness, if present, are mild. Muscle atrophy is a late manifestation.
400
Inclusion body myositis is a uniformly insidious condition and is usually easily distinguishable from PM, DM, and IMNM. Can you name certain features of IBM that help distinguish this from the other myopathies?
1. Typically have a very slow onset. IMNM is typically very rapid in onset.
2. IBM usually involves the proximal and distal muscles, unlike PM or DM
3. Serum creatine kinase levels are elevated but are lower than levels seen in PM and DM.
400
Which disease process is associated with c-ANCA?
Wegener's granulomatosis
400
Kawasaki disease (KD) is a medium-vessel vasculitis that affects children and is very rare in adults. KD presents as fever, rash, cervical lymphadenopathy, conjunctival congestion, and mucositis. Coronary vessel vasculitis, aneurysm formation, and other cardiac complications (heart failure, pericarditis, arrhythmias) may develop. Treatment is with
IVIg and Aspirin
400
Which Avenger other than Captain America was able to pick up Thor’s Mjolnir in the Marvel movies?
The Vision
500
In addition to interstitial lung disease and myositis, anti-synthetase syndrome is characterized by the presence of......?
Raynaud phenomenon, nonerosive arthritis, and mechanic's hands, a dermatologic manifestation characterized by rough, cracked, scaly skin along the lateral aspects of the digits and palms with horizontal lines resembling the weathered hands of a laborer.
500
What is this clinical sign called and which disease process it is associated with?
Shawl sign, seen with dermatomyositis
500
Subacute Cutaneous SLE is associated with which antibody?
Anti-SSA/Ro
500
Which antibodies are associated with Inclusion body myositis?
Anti-cytoplasmic 5’-nucleotidase 1A
500
Can you name 3 out of the 4 US presidents that have been assassinated in US history?
Abraham Lincoln, James A. Garfield, William McKinley, John F. Kennedy