Rheum Test: Packet 3

5 Written Questions

1. hematologic malignancies, aplastic anemia
2. Giant Cell Arteritis (blindness if untreated)
3. Wegener's Granulomatosis
4. Giant Cell Arteritis
5. autoimmune do where IgG antibody formed that binds to platelets; destruction in spleen; isolated thrombocytopenia (other cell lines normal)

5 Matching Questions

1. How do you dx myasthenia gravis?
2. 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?
3. What is the tx for ITP?
4. A pt comes in with purpura, petechiae and hemorrhagic bullae in their mouth. What do they have?
5. 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.

1. a 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
2. b 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)
3. c Myasthenia Gravis (chronic autoimmune neuromuscular disorder with varying degrees of weakness of skeletal (voluntary) muscles of body)/ mya - muscles, anticholinesterase, acetylcholine receptor antibodies
4. d Systemic Vasculitis
5. e idiopathic thrombocytopenia purpura (ITP); chief complaint - mucosal or skin bleeding

5 Multiple Choice Questions

1. Polyarteritis Nodosa; poor prognosis
   1. 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.
   2. What is a rare, systemic necrotizing inflammation of medium and small arteries that is mc in males? It is characterized by abdominal pain, systemic htn, jaundice, peripheral neuropathy and gangrene as well as livedo reticularis.
   3. What is a rare disorder with vasculitis of small arteries and symptoms of nasal congestion, sinusitis and otitis media as well as fever, cough, dyspnea, wt loss and malaise? It is in males or females in their 4th-5th decades
   4. What has an ESR > 50 or often 100 mm/h and an elevated AP?
2. PoG -> Wegman's for Japanese + German food = Polyarteritis Nodosa, Giant Cell Arteritis, Wegener's Granulomatosis, Takayasu's arteritis, Churg-Strauss Syndrome
   1. What is the tx for ITP?
   2. What are the different types of systemic vasculitis?
   3. What is in the differential diagnosis for ITP?
   4. What is tx for Takayasu's arteritis?
3. congestion, crusting, bleeding around nasal mucosa, inflamed gums/ otitis media, conjunctivitis, dvt and pe
   1. How do you dx Takayasu's arteritis?
   2. What are PE findings for Wegener's Granulomatosis?
   3. How do you tx Wegener's Granulomatosis?
   4. What is tx for Takayasu's arteritis?
4. Polyarteritis Nodosa
   1. What has an ESR > 50 or often 100 mm/h and an elevated AP?
   2. What has an ESR >50, is ANCA-negative, has low titers of ANA or RF?
   3. What is in the differential diagnosis for ITP?
   4. What is the hallmark in lab findings for ITP?
5. Wegener's Granulomatosis (fatal if not treated)
   1. What is a rare disorder with vasculitis of small arteries and symptoms of nasal congestion, sinusitis and otitis media as well as fever, cough, dyspnea, wt loss and malaise? It is in males or females in their 4th-5th decades
   2. What is a rare, systemic necrotizing inflammation of medium and small arteries that is mc in males? It is characterized by abdominal pain, systemic htn, jaundice, peripheral neuropathy and gangrene as well as livedo reticularis.
   3. What Ig is involved with ITP? Which cell lines are affected?
   4. What are the different types of systemic vasculitis?

5 True/False Questions

1. What is tx for Churg-Strauss Syndrome? → 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

   True        False

2. What has asthma as its cardinal feature and the pt also has nasal polyps and allergic rhinitis. This affects small to medium sized vessels and can also be associated with tingling in extremities, can affect the lungs, etc... → Polyarteritis Nodosa

   True        False

3. How do you dx Takayasu's arteritis? → 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

   True        False

4. What should you screen for in pt's with 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

   True        False

5. How do you tx myasthenia gravis? → 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

   True        False