5 Written questions
5 Matching questions
- What Ig is involved with ITP? Which cell lines are affected?
- 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.
- What has lab findings of anemia, mild leukocytosis, elevated ESR, hilar adenopathy, C-ANCA (limited to kidney) and P-ANCA (localized to kidney)?
- What has an ESR >50, is ANCA-negative, has low titers of ANA or RF?
- How do you tx Wegener's Granulomatosis?
- a crucial to prevent end-organ damage/ tightly control htn
CAP: Cyclophosphamide and prednisone, ASA 325 mg for TIAs/ AM: Azathioprine 2 mg/kg/d for remission and methotrexate 20 mg/wk if not sign/renal dysfunction
- b Systemic Vasculitis
- c Polyarteritis Nodosa
- d autoimmune do where IgG antibody formed that binds to platelets; destruction in spleen; isolated thrombocytopenia (other cell lines normal)
- e Wegener's Granulomatosis
5 Multiple choice questions
- 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)
- NAP: (since it improves with rest)/ otherwise CT
Anticholinesterase drugs give symptomatic relief
i.e. Neostigmine 15 mg qid and Pyridostigmine 60 mg qid; thymectomy; failure of above - corticosteroids
- thrombocytopenia with platelet counts less than 10,000/mcL (counts less than 5000/mcL can lead to CNS bleeding); peripheral smear, bone marrow and coagulation studies are normal
- PoG -> Wegman's for Japanese + German food = Polyarteritis Nodosa, Giant Cell Arteritis, Wegener's Granulomatosis, Takayasu's arteritis, Churg-Strauss Syndrome
- Myasthenia Gravis (chronic autoimmune neuromuscular disorder with varying degrees of weakness of skeletal (voluntary) muscles of body)/ mya - muscles, anticholinesterase, acetylcholine receptor antibodies
5 True/False questions
What is a vasculitis that presents in pts over 50 with a warning headache, visual symptoms, jaw claudication, throat pain, dry cough, and a fever? It affects medium and large arteries. There is an asymmetry of pulses in arms, murmur of aortic regurgitation and bruits near the clavicle. → Giant Cell Arteritis (blindness if untreated)
How do you treat polyarteritis nodosa? → high dose corticosteroids (60 mg Prednisone daily), Methylprednisolone 1 g IV daily for 3 days if critically ill, immunosuppressive agents like Cyclophosphamide; Hep B responds to short course of prednisone + lamivudine 100 mg/d and plasmapheresis 3 wk for 6 weeks
A pt comes in with purpura, petechiae and hemorrhagic bullae in their mouth. What do they have? → Myasthenia Gravis (chronic autoimmune neuromuscular disorder with varying degrees of weakness of skeletal (voluntary) muscles of body)/ mya - muscles, anticholinesterase, acetylcholine receptor antibodies
What should you screen for in pt's with polyarteritis nodosa? → Hep screening since 10% associated with Hep B
In myasthenia gravis what do you see with the thymus gland? → it is abnormal (thymoma = thymic hyperplasia)/ it has clusters of immune cells indicative of lymphoid hyperplasia/ autoimmunity and production of the acetylcholine receptor antibodies setting the stage for attack on neuromuscular transmission -> skel muscle weakness