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Flashcards in Connective Tissue and Joint Disease Deck (20):
1

Scleroderma diagnosis and treatment

Diagnosis: Elevated ANAs highly sensitive
Anti-centromere specific for limited form
Anti-topoismerase I specific for diffuse form
Barium swallow and PFTs for complications

Treatment:
No effective cure
NSAIDs for musculoskeletal pain, H2 blockers or PPIs for esophageal reflux
Raynauds: avoid cold and smoking, keep hands warm, use CCBs if severe

2

Sjogren's Syndrome diagnosis and treatment

Diagnosis: ANAs in 95% can also have RF
Ro (SS-A) and La Abs
increased ESR, normocytic normochromic anemia, leukopenia
Schirmer test: filter paper inserted into eye to measure lacrimal output-high sensitivity and specficity
Salivary gland biopsy (lip or parotid) most accurate but not done

Treatment:
Pilocarpine or Cevimeline (enhances oral and ocular secretions)
Artificial tears
Good oral hygeine
NSAIDs, steroids for arthralgias, arthritis

3

Diagnosis and Treatment of Rheumatoid Arthritis

Diagnosis: High titers of RF (the higher=more severe disease)
Anti-citrullinated peptide/proteins-specific!
elevated ESR, CRP
Normocytic normochromic anemia (anemia of chronic disease)

Radiographs
Loss of juxtaaricular bone mass (periarticular osteoporosis)
narrowing of joint space
Bony erosions

Treatment:
Exercise helps with range of motion and muscle strength
NSAIDs for pain control (corticosteroids if nonresponsive)
Methotrexate (DMARD) takes 6 weeks to take effect
Use with folate
Leflunomide can also be used
Combo with hydroxychloroquine and sulfasalazine higher remission rates
Anti tumor necrosis factor can help as well

Cervical spine radiographs before undergoing surgery

4

Diagnosis and treatment of Gout

Diagnosis:
Synovial fluid: cloudy yellow, >5000 WBCs, 50-70% PMNs, negatively biferingent Crystals
Radiographs: punched out lesions

Treatment:
Avoid thiazide and loop diuretics, obesity, alcohol, red meat and seafood
Bed rest
NSAIDs for acute gout (indomethacin)
Colcichine if can't tolerate NSAIDs
Corticosteroids (oral prednisone 7-10 days) if don't respond to either
Intra articular corticosteroid injections provides dramatic relief

Prophylactic
Must have 2 acute gouty attacks per year
Colcichine and NSAID for 3-6 months
Uricosuric drugs (probenecid, sulfinpyrazone) if 24 hour urine uric acid is less than 800 mg/day
Allopurinol if 24 hr urine uric acid is greater than 800 mg/day

5

Pseduogout diagnosis and treatment

Diagnosis: monoarticular-knees and wrists
weakly positively birefrigent rod shaped and rhomboidal crystals
Radiographs: chondrocalcinosis

Treatment:
underlying disease-OA, hemochromatosis, hyperparathyroidism, hypothyroidism, and Bartters syndrome

6

Inclusion body myositis

male more than female
no autoAbs
proximal and distal muscle Involvement-weakness and atrophy of quads, forearm flexoors and tibialis anterior (assymetrical)
Facial weakness and dysphagia
Loss of deep tendon reflex (not involved in dermatomyositis and polymyositis)
relatively low CK
Prognosis is poor

Endomysial inflammation

7

Dermatomyositis diagnosis and treatment

Diagnosis: CK is significantly elevated
LDH, aldolase, AST, ALT are also elevated
ANA
Antisynthetase Abs (anti-Jo-1)-abrupt onset of fever, cracked hands, Raynauds, interstitial lung disease, arthritis-does not respond well
Antisignal recongition particle-cardiac manifestations, worst prognosis
Anti-Mi-Abs-better prognosis
EMG-abnormal in 90%
Muscle biopsy-perivascular and perimysial inflammation

Treatment: corticosteroids is initial continue until symptoms improve and then taper slowly
Immunosuppressive agents (if unresponsive)-methotrexate, cyclophosphamide, chlorambucil
Physical therapy

Look for underlying lung, breast, ovary, GI and myeloproliferative cancers

8

Polymyositis treatment and diagnosis

Diagnosis: CK is significantly elevated
LDH, aldolase, AST, ALT are also elevated
ANA
Antisynthetase Abs (anti-Jo-1)-abrupt onset of fever, cracked hands, Raynauds, interstitial lung disease, arthritis-does not respond well
Antisignal recongition particle-cardiac manifestations, worst prognosis
Anti-Mi-Abs-better prognosis
EMG-abnormal in 90%
Muscle biopsy-endomysial inflammation

Treatment: corticosteroids is initial continue until symptoms improve and then taper slowly
Immunosuppressive agents (if unresponsive)-methotrexate, cyclophosphamide, chlorambucil
Physical therapy

9

Diagnosis and treatment of Polymyalgia Rheumatica

Diagnosis: elevated ESR greater than 100

Treatment: corticosteroids
response within 1-7 days begin tapering after 4-6 weeks

10

Ankylosing Spondylitis diagnosis and treatment

Diagnosis: Imaging studies of lumbar spine and pelvis (plain film, MRI, CT)-sclerotic changes (vertebral columns fuse producing bamboo spine)
Elevated ESR
Worse in morning, improves with exercise and hot shower and worsens with rest or inactivity

Treatment:
NSAIDS (indomethacin) for symptomatic relief
Anti-TNF medications (etanercept, infliximab)
Physical therapy

11

Severe signs of Back pain and diagnosis requirements

Age >50
history of previous cancer
constitutional symptoms
night time pain-sleep difficulty
pain duration >1 month
no response to previous therapy
neurological symptoms
If positive perform MRI in 24 hours if not severe
if severe emergency MRI plus glucocorticoids then radiation or surgery
NO red flags perform Xray-if positive then MRI, if negative perform bone scan

12

reactive arthritis, causes diagnosis and treatment

Causes: Salmonella, Shigella, campylobacter, chlamydia, yersinia

Diagnosis: synovial fluid: cloudy yellow, >5000 WBC, 50-70% PMNs

Treatment: NSAIDs
Sulfasalizine or azathioprine if unresponsive

13

Diagnosis and treatment of giant cell arteritis

Diagnosis: ESR elevated
Biopsy of temporal artery

Treatment: Treat immediately with oral prednisone
if loss vision treat with IV steroids and admit to hospital
Diagnosis confirmed: treat for 4 weeks then taper gradually but maintain for 2-3 years

14

Takayasus arteritis

Vasculitis of aortic arch leading to stenosis and narrowing

Clinical: constitutional-fever, sweats, malaise, arthralgias, fatigue
Absent carotid, radial or ulnar pulses
Aortic regurgitation may be present
Severe complications: limb ischemia, aortic anuerysms, aortic regurgitation,stroke and HTN due to RAS

Diagnosis: arterogram
Decreased or absent peripheral pulses, discrepancies of BP (arm vs. leg), arterial bruits

Treatment: steroids, surgery or angioplasty to recannulate stenosed vessels

15

Churg Straus syndrome

Clinical: fever, fatigue, weight loss, prominent respiratory tract findings (asthma, dyspnea) and skin lesions (subcutaneous nodules, palpable purpura)

Diagnosis: skin or lung biopsy-prominence of eosinophils
p-ANCA

Poor prognosis even with steroid treatment

16

Wegeners Granulomatosis diagnosis and treatment

Chest radiograph: nodules or infiltrates
marked elevated ESR, anemia (normochromic normocytic), hematuria, positive c-ANCA, thrombocytopenia
Open lung biopys confirms diagnosis

Treatment: prognosis is poor
Cylcophosphamide and corticosteroids
Renal transplantation for ESRD

17

Polyarteritis nodosa

Vasculitis of nervous system and GI tract
Associated with hep B, HIV, and drug reactions

PMN invasion of all layers leading to fibrinoid necrosis-ischemia, infarction and aneurysms

Clinical: fever, weakness, weight loss, myalgias and arthralgias, and abdominal pain (bowel angina)
HTN and livedo reticularis

Diagnosis:
Biopsy of involved tissue or mesenteric angiorgaphy
ESR elevated and p-ANCA
Test for fecal occult blood

Treatment: corticosteroids to begin with
Cyclopohsphamide if severe

18

Buergers Disease

young men who smoke cigarettes

may lead to gangrene

Ischemic claudication: cold, cyanotic, painful distal extremities, paresthesias of distal extremities and ulceration of digits

Smoking cessation is mandatory

19

Hypersensitivty Vasculitis

Small vessel vasculitis in response to a drug-penicillin, sulfa drugs or infection

Skin: palpalble purpura, macules, or vesicles (can be painful)

Diagnosis: biopsy of tissue

Treatment: withdrawal offending agent, steroids

20

Diagnosis and treatment of SLE

Diagnosis:
Positive ANA test: screening-sensitive (do first)
Anti-ds DNA and anti-Sm Ab: specific and diagnostic
Anti ss DNA
Antihistone Abs: drug induced (Hydralazine, Procainmide, Isoniazid, Quinidine, Chlropromazine, methyldopa)
Ro (SS-A) and La (SS-B)
Complement deficiecy
Positive LE prepearation

Treatment:
Avoid the sun
NSAIDs for less severe symptoms
Corticosteroids for acute exacerbations
Systemic steroids for severe manifestations
Long term therapy: hydroxychloroquine (annual eye exam)
Cyclophosphamide for active glomerulonephritis