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29 Cards in this Set

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  • Back
31. What drug should be used to treat constitutional, cutaneous, and articular manifestations of SLE?
a. Hydroxychloroquine
32. When should cytotoxic agents such as cyclophosphamide be used for SLE?
a. For active glomerulonephritis
33. What produces the most morbidity in SLE?
a. Renal disease
34. What two organ systems are NOT affected by drug-induced lupus?!?!?
a. Kidneys and CNS!
b. Intrarenal, or CNS involvement is present, it is NOT drug-induced lupus!
c. Additionally, the classic butterfly rash, alopecia, and ulcers are typically not seen in drug-induced lupus.
35. Prognosis of drug-induced lupus?
a. Most patients improved after withdrawal of the offending drug. Therefore, the prognosis is obviously more favourable.
36. What are commonly implicated agents for drug-induced lupus (6)?
1. Hydralazine
2. Procainamide
3. INH
4. Chlorpromazine
5. Methyldopa
6. Quinidine
37. Lab findings in drug-induced lupus?
a. Anti-histone antibodies are always present!!!
b. There is an absence of anti-dsDNA and anti-SM Ab.
38. What are the five types of renal involvement with Lupus glomerulonephritis?
1. Type I (5%) minimal lesions-renal failure is very rare
2. Type II (20%)-mesangia lupus GN -renal failure is rare
3. Type III (25%)- focal proliferative GN-renal failure is uncommon
4. Type IV (40%) - Diffuse proliferative GN-renal failure is common!
5. Type V (10%) - membranous lupus GN -renal failure is uncommon
39. Prognosis of SLE?
a. Most pts do not achieve normal life expectancy.
b. With proper treatment, severe organ damage can be prevented and symptoms controlled in many cases.
c. The most common causes of death are opportunistic infections and renal failure.
40. In 7 conditions are ANAs elevated?
1. SLE
2. RA
3. Scleroderma
4. Sjogren's syndrome
5. mixed connective tissue disease
6. polymyositis and dermatomyositis
7. drug-induced lupus
41. Pathophysiology of scleroderma (systemic sclerosis)?
a. Cytokines stimulate fibroblasts, causing abnormal amount of collagen deposition.
b. Is the high quantity of collagen causes the problems associated with this disease (the composition of collagen is normal)!!!
42. In whom is scleroderma more common?
a. In women.
b. Average age of onset is 35 to 50.
43. What systems (or features) are affected by scleroderma?
1. Raynaud's phenomenon
2. Cutaneous Fibrosis
3. GI
4. pulmonary involvement
5. cardiac involvement
6. renal involvement.
44. What is the significance of Raynaud's phenomenon scleroderma?
a. Is present in almost all patients and usually appears before other findings
b. It is caused by vasospasm and thickening of vessel walls in the digits
c. cold occur and stress-free about color changes in fingers – blanching first, then cyanotic, and then red.
45. What is the risk of rain on phenomenon?
a. It can lead to digital ischaemia with ulceration and infarction/gangrene
46. Features of cutaneous fibrosis in scleroderma?
a. Thickening of skin of the face and extremities (sclerodactyly refers to claw-like appearance of the hand).
b. Can lead to contractures, disability, and disfigurement
47. GI complications of scleroderma?
a. Occurs in most patients (both diffuse and limited)
b. findings include:
1. Dysphagia/reflux from the oesophageal immobility (up to 90% of patients)
2. Delayed gastric emptying
3. constipation/diarrhoea
4. abdominal distention
5. Pseudoobstruction
6. prolonged acid reflux and eventually lead to esophageal strictures
48. What is the most common cause of death in scleroderma?
a. Pulmonary complications
49. Pulmonary complications of scleroderma?
a. Most common cause of death in scleroderma.
b. Interstitial fibrosis and/or pulmonary hypertension may be present as well
50. Cardiac involvement in scleroderma?
a. Paracardial effusions
b. myocardial involvement which can lead to congestive heart failure and arrhythmias.
51. Complications of renal involvement in scleroderma?
a. Renal crisis-rapid malignant hypertension occurs in patients with these disease (rare today).
52. Diagnosis of scleroderma?
a. Diagnostic tests or limited utility.
b. Almost all patients have elevated ANAs (high sensitivity, low specificity)
53. What anybody is very specific for scleroderma-diffuse form?
a. Anti-topoisomerase I (anti-SCL-70)
54. What anybody very specific for CREST?
a. Anticentromere antibody.
55. What tests are used to detect complications of scleroderma?
a. Barium swallow (esophageal dysmotility)
b. Pulmonary function test.
56. Skin involvement in Scleroderma vs. CREST (limited Scleroderma)?
a. Diffuse Scleroderma: Widespread skin involvement
b. Limited Scleroderma: Skin involvement limited to distal extremities (and face, neck)-sparing of the trunk.
57. Symptoms onset in Scleroderma vs. CREST (limited Scleroderma)?
a. Diffuse Scleroderma: Rapid onset of symptoms (skin and other complications occur rapidly after onset of Raynaud’s phenomenon)
b. Limited Scleroderma: Delayed onset: skin involvement occurs slowly after the onset of Raynaud’s. Therefore, the pt has a long hx of Raynaud’s phenomenon before other symptoms begin.
58. Visceral involvement with Scleroderma vs. CREST (limited Scleroderma)?
a. Diffuse Scleroderma: Significant visceral involvement- Fibrosis of internal organs- lungs, heart, GI tract, kidneys.
b. Limited Scleroderma: Visceral involvement occurs later- pulmonary HTN and ischaemic vascular disease; minimal constitutional symptoms
59. Prognosis of Scleroderma vs. CREST (limited Scleroderma)?
a. Diffuse Scleroderma: Poorer prognosis- 10-yr survival is 40-65%.
b. Limited Scleroderma: better prognosis than diffuse form. Normal life span is expected in most cases, unless severe pulmonary HTN develops.