Step Up - Connective Tissue and Joint Disease Flashcards
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?
1) ANA
2) Anti-ds DNA, Anti-Sm Ab
3) Antihistone Abs
The diagnosis of SLE requires 4 of 11 criteria are met. Provide the 11 criteria.
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
ANAs are typically elevated in what rheumatologic diseases (4)?
1) SLE
2) Scleroderma
3) Sjogren syndrome
4) Polymyositis
RF is detectable (typically) in what rheumatologic disease?
RA (70%), 3% in gen pop.
C-ANCA is the lab marker for which rheumatologic disease?
Wegener Granulomatosis
P-ANCA is the lab marker for which rheumatologic disease?
Polyarteritis nodosa
Outline the treatment for SLE, highlighting appropriate therapy for mild vs severe disease and acute vs maintenance therapy.
Mild: use NSAIDs
Severe: Systemic steroids
Acute: local or systemic corticosteroids
Chronic: Hydroxychloroquine (concern is retinal toxicity)
- cyclophosphamide used for GN if present
What are some typical findings of antiphospholipid antibody syndrome (4)? What is the appropriate therapy? What investigative test will provide a false-positive?
1) recurrent venous/arterial thrombosis, recurrent fetal loss, thrombocytopenia, livedo reticularis
2) Anti-coagulate
3) Syphilis
Discuss the skin involvement, rapidity of onset, visceral involvement level, associated antibody, and prognosis of diffuse scleroderma.
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
Discuss the skin involvement, rapidity of onset, visceral involvement level, associated antibody, and prognosis of limited scleroderma.
1) Skin - spares trunk
2) Onset - delayed (Raynaud’s first)
3) Visceral involvement - late (resp, CV)
4) Antibody - Anticentromere Ab
5) better prognosis
What is the treatment for scleroderma (4)?
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
What are the clinical features of Sjogren syndrome (4)?
1) Dry eyes
2) Dry mouth - tooth decay
3) Arthralgias/arthritis
4) Interstitial nephritis and vasculitis
What is a clinical test that can be performed for Sjogren syndrome?
Schirmer test - filter paper in eye to determine tear production
How is Sjogren syndrome treated (4)?
1) Pilocarpine for increased secretions
2) Artificial tears
3) Good oral hygiene
4) NSAIDs for arthralgias
- if secondary Sjogren, treated associated rheumatoid disease.
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?
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
What is required for the Dx of rheumatoid arthritis (4)?
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
What are the radiographic findings consistent with RA (2)?
1) Periarticular osteoporosis
2) Narrowing of joint space
Outline the medical management for RA (3)
1) Symptom control - NSAIDs, corticosteroids
2) DMARDs - methotrexate > hydroxychloroquine > sulfalazine
3) Second-line: Anti-TNF inhibiting agents (etanercept, infliximab).
Describe the clinical features of an acute gouty arthritis (5)?
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
Describe the clinical features of chronic tophaceous gout (2)
1) those with poorly controlled gout for 10-20 years
2) Common locations: extensors of forearms, elbows, knees, achilles, pinna of ear
What is seen on joint aspiration of a gouty joint? What is seen on xray?
1) Needle-shaped, negatively birefringent urate crystals
2) Punched out erosions with rim of cortical bone
How is gout treated (3)? What are the prophylactic medications that can be used (2)?
- 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
What patient population is affected by pseudogout?
elderly with degenerative joint disease
What are the most common joints affected by pseudogout (2)? What is seen on aspiration of the joint? What is seen on xray?
1) knees and wrists (monoarthropathy)
2) positively birefringent rhomboid crystals (calcium pyrophosphate)
3) chondrocalcinosis
What is the appropriate treatment for pseudogout (3)?
1) NSAIDs
2) Colchicine for prophylaxis
3) Intra-articular steroid injection
What are the clinical features of polymyalgia rheumatica (4)?
1) hip and shoulder pain bilaterally - bad morning stiffness
2) Constitutional Sx - fever, depression, fatigue
3) Joint swelling/synovitis
4) SSx of temporal arteritis
How is polymalgia rheumatica diagnosed? How is it treated?
1) diagnosis is clinical (ESR usually high)
2) Corticosteroids for 2+ years.
What are the clinical features of fibromyalgia (3)?
1) Stiffness
2) Disrupted sleep
3) Depression and anxiety
How is fibromyalgia diagnosed (3)?
1) pain includes axial pain for >3 months
2) Pain in 11 of 18 trigger spots
3) r/o other causes
Outline the treatment for fibromyalgia (4)
1) Increase activity level
2) Amitriptyline or SNRI
3) Local anesthetic at trigger points
4) CBT, psych
What are the clinical features of ankylosing spondilitis (4) What antigen is it associated with?
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
What is seen on xray in ankylosing spondylitis?
Bamboo spine - fusion of lumbar spine
How is ankylosing spondylitis treated (4)?
1) NSAIDs
2) Anti-TNF agents (etanercept, infliximab)
3) Mobility exercises
4) Surgery if severe
Describe the clinical picture of a reactive arthritis (2). What are the common organisms that cause it?
1) Recent enteric or urogenital infection (1-4 weeks prior)
2) Assymetric oligoarthritis affecting lower extremities
3) Salmonella, shigella, campylobacter, chlamydia, yersinia
How is reactive arthritis diagnosed? How is it treated (3)?
1) Clinical diagnosis, tap joint effusion to rule out crystals
2) NSAIDs > Sulfasalazine > Azathioprine
What are two clinical findings of psoriatic arthritis? What joints are typically affected? What is the treatment (3)?
1) Sausage digits (dactylitis), nail pitting
2) Small joints
3) NSAIDs > methotrexate > anti-TNF
What are some clinical features of temporal/giant cell arteritis (5)?
1) Constitutional symptoms
2) Headache (severe)
3) Blindness - ophthalmic artery involvement
4) Claudication of tongue/jaw
5) Lack of temporal pulse
Provide three serious sequelae of giant cell arteritis.
1) Aortic aneurysm
2) Aortic dissection
3) Blindness
How is temporal arteritis confirmed?
Biopsy of temporal artery
How is temporal/giant cell arteritis treated (3)?
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
What vessels are affected in Takayasu arteritis? What is the treatment?
1) Aortic arch and branching vessels.
2) Steroids - prednisone
What are two findings in Churg-Strauss syndrome? What does it affect?
1) eosinophilia, p-ANCA
2) Vasculitis involving multiple organ systems
What two systems are commonly affected in Wegener’s granulomatosis? How is it diagnosed (4)? How is it treated (2)?
1) Lung and kidney
2) High ESR, c-ANCA, CXR nodules, lung biopsy confirms
3) Cyclophosphamide and corticosteroids
What systems are affected by polyarteritis nodosa (2)? How is it diagnosed (3)? How is it treated (2)?
1) Medium sized vessels of NS and GI
2) ESR high, p-ANCA, biopsy
3) corticosteroids, then cyclophosphamide if severe
What is seen in Buerger Disease (Thromboangiitis obliterans) (4)?
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
