Seropositive spondyloarthropathic disorders (obj 22) Flashcards
thickening of skin confined to face, neck, and distal extremities
scleroderma (limited) - 80% of cases
widespread thickening of skin, including truncal involvement, with areas of increased pigmentation & depigmentation
scleroderma (diffuse) - 20% of cases
these 2 things are present in virtually all scleroderma patients
Raynaud phenomenon
positive ANA test
another name for limited scleroderma
CREST syndrome
CREST syndrome is:
Calcinosis cutis Raynaud phenomenon Esophageal motility disorder Sclerodactyly Telangiectasia
dryness of the eyes and dry mouth (sicca components) are the most common features of this disease
Sjögren syndrome
Sjögren syndrome is usually associated with ____ but could occur with SLE, scleroderma, etc.
Rheumatoid arthritis
These two lab tests are probably positive in Sjögren syndrome
RF
ANA
This test measures the quantity of tears secreted, and therefore could be useful in diagnosing Sjögren syndrome
Schirmer test
bilateral gradual, progressive muscle weakness of proximal upper and lower extremities as well as neck
polymyositis, dermatomyositis
How can polymyositis/dermatomyositis be differentiated from myasthenia gravis?
facial/ocular weakness!
polymyositis/dermatomyositis: NO; myasthenia gravis yes
What differentiates the dysphagias of scleroderma and polymyositis/dermatomyositis?
~scleroderma: affects smooth muscle of lower esophagus, causing a “sticking” sensation below sternum
~poly/dermato: affects striated muscles of upper pharynx, making initiation of swallowing difficult
What are THREE characteristic rashes of dermatomyositis?
- dusky red, malar distribution
- heliotrope (violaceous periorbital edema)
- shawl sign (erythema over neck, shoulders, upper chest/back)
Describe the hands of someone with dermatomyositis.
Gottron sign! (scaly red patches over the dorsum of the PIP and MCP joints)
–also periungual erythema and dilations of nailbed capillaries
The only specific diagnostic test for polymyositis/dermatomyositis:
muscle biopsy
The 2 different muscle biopsy findings for poly and dermato myositis
polymyositis: endomysial infiltration
dermatomyositis: perifascicular atrophy
treatment for polymyositis/dermatomyositis
corticosteroids
hypercoagulability with recurrent thromboses
antiphospholipid syndrome
With antiphospholipid syndrome, thromboses occur in (venous, arterial) circulation
EITHER
If your patient has three or more unexplained miscarriages during the first trimester, you may suspect:
antiphospholipid syndrome
treatment for antiphospholipid syndrome
lifelong anticoagulation with warfarin
heparin + aspirin if pregnant
Patients with ____ or _____ have an increased risk of malignancy.
Dermatomyositis
SLE
Many of the clinical manifestations of this disease are due to the trapping of antigen-antibody complexes in capillaries of viscera OR due to autoimmune destruction of host cells
Systemic lupus erythematosus (SLE)
This demographic group tends to get SLE.
Young black women
Before making a diagnosis of SLE, it is important to ascertain that the condition is not due to _____.
DRUGS
This test is sensitive but not specific for SLE.
ANA
positive in virtually all patients with SLE but also positive in other conditions (e.g. scleroderma, Sjögren syndrome)
What is the rash usually thought of as characteristic of SLE?
malar (butterfly) rash on face
Over 90% of patients with SLE have this clinical manifestation, which is often the earliest manifestation.
joint symptoms
What are 3 major sources of disease morbidity in SLE?
- glomerulonephritis
- CNS disease
- antiphospholipid antibodies
SLE patient education: patients with SLE must be cautioned against exposure to what?
SUN
Treatments for SLE:
~NSAIDs (for joint pain)
~corticosteroids (for skin lesions, glomerulonephritis, CNS problems, etc.)
~antimalarials (for lupus rashes, joint symptoms)
~immunosuppressive agents
Symmetric polyarthritis in small joints of hands and feet
Rheumatoid arthritis
What is the most specific blood test for rheumatoid arthritis?
Anti-CCP antibodies
Radiographic changes seen in rheumatoid arthritis are:
- joint space narrowing
2. joint erosion
Compare and contrast rheumatoid arthritis and osteoarthritis. (3)
Osteoarthritis spares the wrist and MCP joints.
Osteoarthritis joint pain is relieved by rest.
Osteoarthritis has minimal articular inflammation.
As soon as you confirm your diagnosis of RA, you should start what treatment?
DMARDs
disease-modifying anti-rheumatic drugs
Name one synthetic DMARD and one biologic DMARD.
synthetic: methotrexate
biologic: TNF inhibitors
What is the role of corticosteroids in the treatment of RA?
Corticosteroids have a PROMPT anti-inflammatory effect, and are often used as a “bridge” to reduce disease activity until the slower-acting DMARDs take effect (or as adjunctive therapy for disease that persists despite treatment with DMARDs).
Why would you give ACE inhibitors for SLE?
renal disease/BP control
Smith autoantibody
SLE
