Step Up - Connective Tissue and Joint Disease

Question 1

What is the most common antibody found in patients with SLE? What 2 antibodies are considered diagnostic for SLE? What antibody is present in drug-induced lupus?

Answer 1

1) ANA
2) Anti-ds DNA, Anti-Sm Ab
3) Antihistone Abs

Question 2

The diagnosis of SLE requires 4 of 11 criteria are met. Provide the 11 criteria.

Answer 2

1) Butterfly rash
2) Photosensitivity
3) Oral or nasopharyngeal ulcers
4) Discoid rash
5) Arthritis
6) Pericarditis/pleuritis
7) Hematologic disease - reticulocytosis, thrombo/leuko/lympho-penia
8) Renal disease - protein >0.5g/day
9) CNS - seizures, psychosis
10) Immunologic - positive anti-ds DNS, ant-Sm Ab
11) ANAs

Question 3

ANAs are typically elevated in what rheumatologic diseases (4)?

Answer 3

1) SLE
2) Scleroderma
3) Sjogren syndrome
4) Polymyositis

Question 4

RF is detectable (typically) in what rheumatologic disease?

Answer 4

RA (70%), 3% in gen pop.

Question 5

C-ANCA is the lab marker for which rheumatologic disease?

Answer 5

Wegener Granulomatosis

Question 6

P-ANCA is the lab marker for which rheumatologic disease?

Answer 6

Polyarteritis nodosa

Question 7

Outline the treatment for SLE, highlighting appropriate therapy for mild vs severe disease and acute vs maintenance therapy.

Answer 7

Mild: use NSAIDs
Severe: Systemic steroids
Acute: local or systemic corticosteroids
Chronic: Hydroxychloroquine (concern is retinal toxicity)
- cyclophosphamide used for GN if present

Question 8

What are some typical findings of antiphospholipid antibody syndrome (4)? What is the appropriate therapy? What investigative test will provide a false-positive?

Answer 8

1) recurrent venous/arterial thrombosis, recurrent fetal loss, thrombocytopenia, livedo reticularis
2) Anti-coagulate
3) Syphilis

Question 9

Discuss the skin involvement, rapidity of onset, visceral involvement level, associated antibody, and prognosis of diffuse scleroderma.

Answer 9

1) Skin - widespread involvement
2) Onset - rapid (Raynaud’s first)
3) Visceral involvement - significant (resp, GI, CV, renal)
4) Antibody - Antitopoisomerase 1
5) poor prognosis

Question 10

Discuss the skin involvement, rapidity of onset, visceral involvement level, associated antibody, and prognosis of limited scleroderma.

Answer 10

1) Skin - spares trunk
2) Onset - delayed (Raynaud’s first)
3) Visceral involvement - late (resp, CV)
4) Antibody - Anticentromere Ab
5) better prognosis

Question 11

What is the treatment for scleroderma (4)?

Answer 11

Treatment aimed at symptoms:

1) NSAIDs for MSK pain
2) H2 blocker or PPI for GERD
3) Avoid cold for Raynaud’s
4) ACEi for renal HTN

Question 12

What are the clinical features of Sjogren syndrome (4)?

Answer 12

1) Dry eyes
2) Dry mouth - tooth decay
3) Arthralgias/arthritis
4) Interstitial nephritis and vasculitis

Question 13

What is a clinical test that can be performed for Sjogren syndrome?

Answer 13

Schirmer test - filter paper in eye to determine tear production

Question 14

How is Sjogren syndrome treated (4)?

Answer 14

1) Pilocarpine for increased secretions
2) Artificial tears
3) Good oral hygiene
4) NSAIDs for arthralgias
- if secondary Sjogren, treated associated rheumatoid disease.

Question 15

Describe the clinical features of rheumatoid arthritis. What joints are most likely to be involved (2)? What are some characterisitc deformities (4)? What is a risk when intubating these patients?

Answer 15

1) Joints: typically symmetric distribution. Often wrists and hands first
2) Deformities: ulnar deviation at MCP, boutonniere’s deformity, swan-neck deformity, subcutaneous rheumatoid nodules
3) C1-C2 instability

Question 16

What is required for the Dx of rheumatoid arthritis (4)?

Answer 16

1) Inflammatory arthritis in 3+ joints for 6+ weeks
2) High CRP and ESR
3) Positive RF or ACPA
4) Xray shows changes of RA

Question 17

What are the radiographic findings consistent with RA (2)?

Answer 17

1) Periarticular osteoporosis

2) Narrowing of joint space

Question 18

Outline the medical management for RA (3)

Answer 18

1) Symptom control - NSAIDs, corticosteroids
2) DMARDs - methotrexate > hydroxychloroquine > sulfalazine
3) Second-line: Anti-TNF inhibiting agents (etanercept, infliximab).

Question 19

Describe the clinical features of an acute gouty arthritis (5)?

Answer 19

1) Men 40-60
2) Exquisite pain (wakes from sleep). Often 1st MTP
3) Cellulitic changes - erythema, swelling, tender
4) +/- fever
5) desquamation of overlying skin on resolution

Question 20

Describe the clinical features of chronic tophaceous gout (2)

Answer 20

1) those with poorly controlled gout for 10-20 years

2) Common locations: extensors of forearms, elbows, knees, achilles, pinna of ear

Question 21

What is seen on joint aspiration of a gouty joint? What is seen on xray?

Answer 21

1) Needle-shaped, negatively birefringent urate crystals

2) Punched out erosions with rim of cortical bone

Question 22

How is gout treated (3)? What are the prophylactic medications that can be used (2)?

Answer 22

• Acute:
  1) NSAIDs
  2) Colchicine - lots of SE
  3) Corticosteroids - prednisone 7-10 days, or intra-articular injection
• Prophylactic:
  1) Uricosuric agent if urine uric acid excretion less than 800mg/day
  2) Allupurinol if urine uric acid excretion >800mg/day

Question 23

What patient population is affected by pseudogout?

Answer 23

elderly with degenerative joint disease

Question 24

What are the most common joints affected by pseudogout (2)? What is seen on aspiration of the joint? What is seen on xray?

Answer 24

1) knees and wrists (monoarthropathy)
2) positively birefringent rhomboid crystals (calcium pyrophosphate)
3) chondrocalcinosis

Question 25

What is the appropriate treatment for pseudogout (3)?

Answer 25

1) NSAIDs 2) Colchicine for prophylaxis 3) Intra-articular steroid injection

Question 26

What are the clinical features of polymyalgia rheumatica (4)?

Answer 26

1) hip and shoulder pain bilaterally - bad morning stiffness 2) Constitutional Sx - fever, depression, fatigue 3) Joint swelling/synovitis 4) SSx of temporal arteritis

Question 27

How is polymalgia rheumatica diagnosed? How is it treated?

Answer 27

1) diagnosis is clinical (ESR usually high) | 2) Corticosteroids for 2+ years.

Question 28

What are the clinical features of fibromyalgia (3)?

Answer 28

1) Stiffness 2) Disrupted sleep 3) Depression and anxiety

Question 29

How is fibromyalgia diagnosed (3)?

Answer 29

1) pain includes axial pain for >3 months 2) Pain in 11 of 18 trigger spots 3) r/o other causes

Question 30

Outline the treatment for fibromyalgia (4)

Answer 30

1) Increase activity level 2) Amitriptyline or SNRI 3) Local anesthetic at trigger points 4) CBT, psych

Question 31

What are the clinical features of ankylosing spondilitis (4) What antigen is it associated with?

Answer 31

1) Associated with HLA-B27 antigen 2) Male predominance 3) Bilateral sacroiliitis 4) Enthesitis (tendon inflammation) - achilles, supraspinatous 5) Extra-articular - anterior uveitis, AV heart block, aortic insufficiency

Question 32

What is seen on xray in ankylosing spondylitis?

Answer 32

Bamboo spine - fusion of lumbar spine

Question 33

How is ankylosing spondylitis treated (4)?

Answer 33

1) NSAIDs 2) Anti-TNF agents (etanercept, infliximab) 3) Mobility exercises 4) Surgery if severe

Question 34

Describe the clinical picture of a reactive arthritis (2). What are the common organisms that cause it?

Answer 34

1) Recent enteric or urogenital infection (1-4 weeks prior) 2) Assymetric oligoarthritis affecting lower extremities 3) Salmonella, shigella, campylobacter, chlamydia, yersinia

Question 35

How is reactive arthritis diagnosed? How is it treated (3)?

Answer 35

1) Clinical diagnosis, tap joint effusion to rule out crystals 2) NSAIDs > Sulfasalazine > Azathioprine

Question 36

What are two clinical findings of psoriatic arthritis? What joints are typically affected? What is the treatment (3)?

Answer 36

1) Sausage digits (dactylitis), nail pitting 2) Small joints 3) NSAIDs > methotrexate > anti-TNF

Question 37

What are some clinical features of temporal/giant cell arteritis (5)?

Answer 37

1) Constitutional symptoms 2) Headache (severe) 3) Blindness - ophthalmic artery involvement 4) Claudication of tongue/jaw 5) Lack of temporal pulse

Question 38

Provide three serious sequelae of giant cell arteritis.

Answer 38

1) Aortic aneurysm 2) Aortic dissection 3) Blindness

Question 39

How is temporal arteritis confirmed?

Answer 39

Biopsy of temporal artery

Question 40

How is temporal/giant cell arteritis treated (3)?

Answer 40

1) If vision loss present - high-dose IV steroids 2) If no vision loss - oral prednisone 3) Follow ESR, treat for 2-3 years - treat presumptively, don't wait for biopsy results

Question 41

What vessels are affected in Takayasu arteritis? What is the treatment?

Answer 41

1) Aortic arch and branching vessels. | 2) Steroids - prednisone

Question 42

What are two findings in Churg-Strauss syndrome? What does it affect?

Answer 42

1) eosinophilia, p-ANCA | 2) Vasculitis involving multiple organ systems

Question 43

What two systems are commonly affected in Wegener's granulomatosis? How is it diagnosed (4)? How is it treated (2)?

Answer 43

1) Lung and kidney 2) High ESR, c-ANCA, CXR nodules, lung biopsy confirms 3) Cyclophosphamide and corticosteroids

Question 44

What systems are affected by polyarteritis nodosa (2)? How is it diagnosed (3)? How is it treated (2)?

Answer 44

1) Medium sized vessels of NS and GI 2) ESR high, p-ANCA, biopsy 3) corticosteroids, then cyclophosphamide if severe

Question 45

What is seen in Buerger Disease (Thromboangiitis obliterans) (4)?

Answer 45

1) Affects young male smokers 2) Segmental inflammation of small/medial vessels of extremities 3) May lead to autoamputation and gangrene 4) Raynaud's phenomena may be seen - Tx: stop smoking