S2: Ch 6 Connective Tissue 1-9

Front 1

235. Tx of Idiopathic inflammatory myopathies (dermatomyositis, polymyositis, inclusion body myositis)?

Back 1

a. Corticosteroids are the initial treatment.
i. Continue until symptoms improve
ii. Then taper very slowly (up to 2 years may be necessary)
b. immunosuppressive agents (for patients who do not respond steroids)
c. physical therapy

Front 2

236. What 3 immunosuppressive agents are used for the Idiopathic inflammatory myopathies?

Back 2

1. Methotrexate
2. Cyclophosphamide
3. Chlorambucil

Front 3

237. In whom is Inclusion Body Myositis more common?

Back 3

a. Men (elderly)

Front 4

238. Presentation of Inclusion Body Myositis?

Back 4

a. Insidious onset of slowly progressive proximal and distal weakness, often leads to delay in diagnosis.
b. There is early weakness and atrophy of quadriceps, forearm flexors, and tibialis anterior muscles.
c. Involvement is asymmetrical.
d. Facial weakness occurs in one third of patients, and dysphagia in one of patients.

Front 5

239. In terms of DTRs, how is Inclusion Body Myositis different from polymyositis and dermatomyositis?

Back 5

a. Patients can also have loss of deep tendon reflexes (nerves are not involved in polymyositis and dermatomyositis)

Front 6

240. Are there extra-muscular manifestations with Inclusion Body Myositis?

Back 6

a. Extra muscular manifestations are rare

Front 7

241. Diagnosis and prognosis of Inclusion Body Myositis?

Back 7

a. Diagnosis: slight elevation of CK levels (relatively low)
b. There is a poor response to therapy

Front 8

242. Diagnostic criteria for polymyositis?

Back 8

a. If 2 of first four ->Possible polymyositis
b. If 3 of first four -> probable polymyositis
c. if all four -> Definate polymyositis
1. Symmetric proximal muscle weakness.
2. Elevation in serum creatinine phosphokinase.
3. EMG findings of myopathy.
4. Biopsy evidence of myositis.
5. Characteristic rash of dermatomyositis

Front 9

243. In whom is Polymyalgia Rheumatica primarily seen?

Back 9

a. Usually occurs in elderly patients (rare before age 50).
b. The mean age of onset is 70.
c. More common in women.

Front 10

244. Pathophysiology of Polymyalgia Rheumatica?

Back 10

a. An autoimmune process may be responsible.
b. Possible genetic link (Association with HLA –DR4 allele).

Front 11

245. Prognosis of Polymyalgia Rheumatica?

Back 11

a. Self-limited disease -duration of one to two years

Front 12

246. Clinical features of Polymyalgia Rheumatica?

Back 12

a. Hip and shoulder muscle pain (bilateral)
b. constitutional symptoms are usually present: malaise, fever, depression, weight loss, and fatigue.
c. Joint swelling.
d. S/S of temporal arteritis (if present)

Front 13

247. Features of hip and shoulder muscle pain with Polymyalgia Rheumatica?

Back 13

a. Often begins abruptly (may be gradual)
b. Stiffness in shoulder and hip regions after Inactivity the most prominent symptom.
c. Pain occurs on movement
d. muscle strength is normal
e. Profound, morning stiffness is common

Front 14

248. Features of joint swelling in Polymyalgia Rheumatica?

Back 14

a. Up to 20% of patients have Synovitis in knees, wrists, work and joints (can be confused with RA)
b. Synovitis and tenosynovitis around the shoulder may lead to rotator cuff tendinitis or adhesive capsulitis.

Front 15

249. Diagnosis of Polymyalgia Rheumatica?

Back 15

a. Essentially a clinical diagnosis
b. ESR is usually elevated and he in diagnosis
c. Almost always > 50, frequently > 100
i. ESR correlates with disease activity

Front 16

250. Treatment of Polymyalgia Rheumatica?

Back 16

a. Corticosteroids!
b. Response usually occurs within 1 to 7 days.
c. Steroids are not curative, but are effective in suppressing inflammation until the disease resolves itself.
d. After 4 to 6 weeks, begin to taper slowly
e. Most patients (60-70%) can stop steroids within two years.
f. A few patients have symptoms for up to 10 years

Front 17

251. note: about 10% of people with polymyalgia rheumatica develop temporal arteritis. Whereas up to 40 to 50% of people temporary rightists have coexisting polymyalgia rheumatica

Back 17

251. note: about 10% of people with polymyalgia rheumatica develop temporal arteritis. Whereas up to 40 to 50% of people temporary rightists have coexisting polymyalgia rheumatica

Front 18

245. Prognosis of Polymyalgia Rheumatica?

Back 18

a. Self-limited disease -duration of one to two years

Front 19

246. Clinical features of Polymyalgia Rheumatica?

Back 19

a. Hip and shoulder muscle pain (bilateral)
b. constitutional symptoms are usually present: malaise, fever, depression, weight loss, and fatigue.
c. Joint swelling.
d. S/S of temporal arteritis (if present)

Front 20

247. Features of hip and shoulder muscle pain with Polymyalgia Rheumatica?

Back 20

a. Often begins abruptly (may be gradual)
b. Stiffness in shoulder and hip regions after Inactivity the most prominent symptom.
c. Pain occurs on movement
d. muscle strength is normal
e. Profound, morning stiffness is common

Front 21

248. Features of joint swelling in Polymyalgia Rheumatica?

Back 21

a. Up to 20% of patients have Synovitis in knees, wrists, work and joints (can be confused with RA)
b. Synovitis and tenosynovitis around the shoulder may lead to rotator cuff tendinitis or adhesive capsulitis.

Front 22

249. Diagnosis of Polymyalgia Rheumatica?

Back 22

a. Essentially a clinical diagnosis
b. ESR is usually elevated and he in diagnosis
c. Almost always > 50, frequently > 100
i. ESR correlates with disease activity

Front 23

250. Treatment of Polymyalgia Rheumatica?

Back 23

a. Corticosteroids!
b. Response usually occurs within 1 to 7 days.
c. Steroids are not curative, but are effective in suppressing inflammation until the disease resolves itself.
d. After 4 to 6 weeks, begin to taper slowly
e. Most patients (60-70%) can stop steroids within two years.
f. A few patients have symptoms for up to 10 years

Front 24

251. note: about 10% of people with polymyalgia rheumatica develop temporal arteritis. Whereas up to 40 to 50% of people temporary rightists have coexisting polymyalgia rheumatica

Back 24

251. note: about 10% of people with polymyalgia rheumatica develop temporal arteritis. Whereas up to 40 to 50% of people temporary rightists have coexisting polymyalgia rheumatica