5 Written questions
5 Matching questions
- A pt comes in with ptosis, diplopia, difficulty in chewing and swallowing, respiratory difficulties and limb weakness. The muscle weakness increases with activity and improves after periods of rest. What do they have?
- How do you dx Takayasu's arteritis?
- What should you screen for in pt's with polyarteritis nodosa?
- What Ig is involved with ITP? Which cell lines are affected?
- What is the tx for ITP?
- a Myasthenia Gravis (chronic autoimmune neuromuscular disorder with varying degrees of weakness of skeletal (voluntary) muscles of body)/ mya - muscles, anticholinesterase, acetylcholine receptor antibodies
- b Hep screening since 10% associated with Hep B
- c autoimmune do where IgG antibody formed that binds to platelets; destruction in spleen; isolated thrombocytopenia (other cell lines normal)
- d angiography
- e PISD = initial tx: Prednisone 1-2 mg/kg/day; high dose IV Ig of 1 g/kg for 1-2 days for platelet counts < 5000; Splenectomy is definitive tx; Danazol 600 mg/day if all of above fail
5 Multiple choice questions
- Wegener's Granulomatosis (fatal if not treated)
- lateral and ap x-rays of the chest and CT scans to demonstrate a co-existing thymoma; dx can be confirmed by response to a short-acting anticholinesterase (Phone Eddie)/ Edrophonium IV 10 mg will improve muscle strength, EMG, assay of serum for elevated levels of circulating acetylcholine receptor antibodies (ANA increased but lower titers than SLE)
- Giant Cell Arteritis
- hematologic malignancies, aplastic anemia
- Polyarteritis Nodosa; poor prognosis
5 True/False questions
What is tx for Takayasu's arteritis? → angiography
What is a group of vascular dz's that cause inflammatory injury and necrosis of the blood vessels? They are classified as small, medium or large. → P M & M: Prednisone 1 mg/kg/d tapered to < 20 in 8-20 wks with maintenance at 10 mg; Methotrexate 25 mg/wk and mycophenolate mofetil 1500 mg bid for those who are refractory
How do you treat polyarteritis nodosa? → urgently with prednisone 60 mg/d immediately -> bx to confirm/ then low dose ASA 81 mg/d/ continue prednisone 1 month before tapering
How do you tx Wegener's Granulomatosis? → angiography
What are the different types of systemic vasculitis? → hematologic malignancies, aplastic anemia