Class 5 (2/4/21) Flashcards
1
Q
Most rheumatic conditions involve pathological process involving …
A
Immune system.
2
Q
Rheumatoid arthritis (RA) is…
A
- Chronic systemic inflammatory disease.
- Polyarticular disease that most commonly involves the peripheral non-weight bearing joints.
- Progressive and results in pain, stiffness, and swelling of joints.
- Women are more affected.
- Age of onset is between 35 to 45.
3
Q
Etiology of RA?
A
- Exact cause of RA is unknown.
2. A blend of environmental and genetic factors is responsible.
4
Q
Pathophysiology of RA?
A
- Hypertrophy of synovial membrane.
2. Pannus formation results in destruction of articular cartilage and subchondral bone (bone erosion).
5
Q
Presentation of RA?
A
- RA usually has an insidious, slow onset over weeks to months.
- Joints most commonly involved first are metacarpophalangeal (MCP) joints of the hands, proximal interphalangeal (PIP) joints, and wrists.
- Rheumatoid nodules are found most often on extensor surfaces such as olecranon process.
- Boutonniere’s deformity (flexed PIP, extended DIP)
- Swan neck deformity
- Ulnar deviation of wrist
- Extra-articular complications (Cardiac, pulmonary, hepatic, ocular, vascular)
6
Q
Lab studies in RA
A
- Positive serum RF (serum rheumatoid factor), about 70-85% of patients with RA.
- Erythrocyte sedimentation rate (ESR) is elevated with 90% of patients with RA.
- C-reactive protein (CRP) is elevated in inflammatory disease.
- Complete blood count (CBC): presence of anemia.
7
Q
Imaging studies in RA?
A
X-ray:
- Bone erosion and cycts
- Osteopenia
- Joint space swelling
- Calcification
- Narrowed joint space
- Deformities, separations, and fractures.
8
Q
Diagnostic criteria for RA:
A
A patient must exhibit 4 of the 7 criteria:
- Morning stiffness lasting at least 1 hour and present for at least 6 weeks.
- Swelling in three or more joints for at least 6 weeks.
- Swelling in hand joints (MCP, PIP, wrist) for at least 6 weeks.
- Symmetrical joint swelling for at least 6 weeks.
- Erosions or decalcification (osteopenia) on x-ray of the hands.
- Presence of rheumatoid nodules.
- Elevated level of serum rheumatoid factor (RF)
9
Q
Treatment of RA
A
- Moist heat (Paraffin bath)
Hubbard bath - hydrotherapy - Physical therapy
- Pain relief (NSAIDS/COX-2 inhibitor), NSAIDS reduces pain and inflammation.
- DMARDS - disease modifying antirheumatic drugs
- Glococorticoids/corticosteroids
10
Q
Nonsteroidal anti-inflammatory drugs (NSAIDs) &
Cyclooxygenase-2 (COX-2) inhibitors [Celecoxib]
A
Nonsteroidal anti-inflammatory drugs (NSAIDs):
- NSAIDs have been the cornerstone of therapy for RA.
- NSAIDs reduce pain and inflammation and allow for improvements in mobility and function.
Cyclooxygenase-2 (COX-2) inhibitors [Celecoxib]:
- Has anti-inflammatory, analgesic, and antipyretic activity. Blocks prostaglandin synthesis.
- Unlike other NSAIDs, COX-2 inhibitors suppress only cyclooxygenase-2, the enzyme involved in inflammation. COX-2 inhibitors also have fewer GI Side effects.
11
Q
DMARDS - Disease-Modifying Antirheumatic Drugs
A
- First-line therapy (in addition to NSAIDS).
- These drugs most frequently are used in various combination therapy regimens.
- Limit the amount of joint damage that occurs in rheumatoid arthritis.
- Slow the disease and save the joints and other tissues from permanent damage.
- They are often used to try and control synovial inflammation, decrease erosions and reduce the necessity for corticosteroids.
- Associated with better long-term disability index.
Methotrexate - MTX (Rheumatrex)
- Gold standard of care in patients with RA.
- Has potent immunosuppressive effects.
Side effects: nausea, GI discomfort, rash, diarrhea, and headaches.
Rare SE: hepatotoxicity and bone marrow suppression. Therefore, a CBC, urinalysis, and comprehensive metabolic panel should be monitored every 4 to 6 weeks.
Sulfasalazine, antimalarials (hydroxychloroquine), gold
Immunomodulators: medications used to help regulate or normalize the immune system. Examples include one class of immunomodulator which is used as an adjunctive therapy to treat asthma.
12
Q
Surgical therapy for RA
A
- Synovectomy - local destruction or removal of inflamed synovium from individual joints.
- Joint Replacement
- Joint Fusion - wrist, thumb, C-spine
- Reconstruction - tendon repair
13
Q
Systemic Lupus Erythematosus (SLE)
A
- Development of autoantibodies.
- Affects multiple organ systems.
- Characterized by peripheral polyarthritis with symmetric involvement of small and large joints WITHOUT joint erosion.
- The disease is quite variable from patient to patient
and even within a given patient, the disease can
manifest as a highly variable course. - A chronic, relapsing, inflammatory, and often febrile
multi-systemic disorder of connective tissue,
characterized principally by involvement of skin, joint, and kidneys.
14
Q
Epidemiology of SLE
A
- Greater incidence in women (due to estrogen, etc.)
- The onset of SLE is highest between the ages 15-
40 years old. (= SLE is the most common cause of systemic illness in young females between the
ages of 15 and 40 years). - It is three times more common in African American women in Caucasian women (also more common in women of Hispanic, Asian, and Native American descent
than in Caucasian women).
15
Q
Pathogenesis of SLE
A
- It is a classic autoimmune disease.
- Target tissue damage is caused primarily by pathogenic
autoantibodies, and immune complex formation that
induces a vasculitis in many organ systems
16
Q
Genetics on SLE
A
- Susceptibility to SLE depends on multiple genes.
- Multiple genetic defects appear to contribute to the
development of pathogenetic autoantibodies.
17
Q
Environmental factors of SLE
A
- Ultraviolet light promotes apoptosis in dermal cells
which results in the proliferation of autoantibodies
including: antinuclear antibodies (ANA) and antiphospholipid antibodies (APLA), Anti-dsDNA (anti-double-stranded DNA).
18
Q
Hormonal influences of SLE
A
- Estrogen: prepubertal and postmenopausal women have similar incidence to men. Men who develop lupus have a higher concentration of estrogenic metabolites.
19
Q
Medications capable producing lupus
A
- Anticonvulsants (dilantin, phenobarbital).
- Hydralazine ( antihypertensive – lower BP).
- Procainamide ( anti arrhythmic).
- isoniazid (anti-TB)…
20
Q
Clinical findings of SLE
A
- Unexplained fever
- Extreme fatigue
- Malar flush on the face (SLE)/Discoid lesions on the skin
- Painful or swollen joints and muscle pain
- Pale or purple fingers or toes from cold or stress (Raynaud’s phenomenon)
- Sensitivity to the sun / Photosensitive dermatitis
21
Q
Skin involvement in SLE
A
- Malar rash: (aka “butterfly rash”) the classic skin finding of SLE is the malar rash sparing the nasolabial folds.
- Discoid Lesion: Chronic discoid lesions with central atrophy, depigmentation.
- Raynaud’s phenomenon
22
Q
Joint involvement of SLE
A
- Arthritis is common and affects both small and
large joints in a symmetric pattern. - The axial spine is not involved.
- Nonerosive: no bony erosions.
23
Q
Renal involvement in SLE
A
- In patients younger than the age of 20 years,
the most common presentation is renal disease
manifesting with proteinuria. - ## Develop glomerulonephritis.
24
Q
The best screening test for SLE is…
A
- Antinuclear antibody (ANA) test
- The presence of ANA antibodies is a hallmark of connective tissue disease and occurs in the majority of
lupus patients. - Anti-phospholipid antibodies: These antibodies interfere with the normal function of blood vessels and can lead to narrowing of the blood vessels or blood clots. These
complications can lead to stroke, heart attack, and miscarriage. - Anti-dsDNA (anti-double-stranded DNA), anti-Sm (anti-
Smith antibodies), and anti-RNP antibodies:
The antidsDNA and anti-RNP tests confirm whether there are antibodies being produced to the genetic material in the cell. The anti-Sm test measures if there are antibodies against a certain protein found in the nucleus of cells.
25
Flares
- Like many autoimmune diseases, SLE can have a
clinical course that includes periods where the disease
is well controlled and periods of symptom
exacerbation.
- The breakthrough of symptoms while on treatment is
known as flares.
26
Common triggers of SLE
- Overwork, stress, pregnancy, exposure to sunlight or other source of UV light.
27
Conservative therapy in SLE
- UV protection
- NSAIDs / COX-2 inhibitors
- Antimalarials - hydroxychloroquine (Plaquenil)
28
Aggressive therapy in SLE
- Institution of aggressive therapy, beginning with highdose glucocorticoids, is used whenever a patient has life-threatening SLE that is likely to respond to steroids.
- Cortisteroids
- Immunosuppresives
- Plasmaphoresis
29
Sjogren's syndrome
- A chronic inflammatory disease that primarily affects the lacrimal and saliva glands.
- Exists in both a primary and secondary form.
- In the absence of other autoimmune disorders, it is
classified as primary Sjögren’s syndrome.
- When occurring with a well defined connective tissue
disease, usually rheumatoid arthritis or systemic lupus
erythematosus it is known as secondary.
Clinical manifestation:
- Dry eye (damage to the eye surface)
- Dry mouth—difficulty swallowing food without drinking & rapidly progressive cavities (secondary to decreased saliva volume).
30
Epidemiology of Sjogren's syndrome
- Gender: The disease affects predominantly middle-aged women, in the peri- or post-menopausal period.
- Secondary Sjogren's syndrome = Sicca syndrome
- Age: 20-40
31
Diagnosis of Sjogren's syndrome
Schirmer test: A test strip of number 41 Whatman
filter paper is placed near the lower conjunctival sac to
measure tear formation.
- Autoantibodies (anti-Ro and –La): the presence of
anti-Ro/SSA and anti-La/SSB antibodies in the tear
fluid and serum of patients with Sjögren’s syndrome.
32
Treatment/medication for Sjogren's syndrome
- Moisture replenishment: Lubricants for dry eyes.
- Pilocarpine (Salagen): a cholinergic agonist. May increase salivary and lacrimal flow rates.
- Cyclosporine ophthalmic drops: Immunosuppressive agent
- Antimalarials, Hydroxychloroquine (Plaquinel):
an antimalarial drug used in lupus and rheumatoid
arthritis, may be helpful in some patients with
Sjögren's syndrome.
33
Vasculitis
- Inflammatory disorders involving blood
vessel walls.
- Most of these disorders are considered to be
autoimmune.
34
Classification of Vasculitis
- Large-vessel vasculitis= Giant cell (temporal) arteritis = GCA
35
Epidemiology of temporal arteritis
- Gender: Women are more affected.
- Age: GCA occurs in individuals older than 50 years of age.
- Racial Factors: GCA is most common among people of North European descent.
36
Clinical features of temporal arteritis
- headache, which may be unilateral
- scalp tenderness
- jaw and/or tongue claudication (pain in the jaw associated with chewing.)
- changes in vision, including blindness.
- temporal artery tenderness or decreased temporal
arterial pulse
37
Lab tests in temporal arteritis
- Erythrocyte sedimentation rate (ESR) and/or C-reactive protein (CRP) are increased
38
Diagnosis for GCA
- Temporal artery biopsy: Temporal artery biopsy is the
diagnostic procedure of choice and the standard for the
diagnosis of GCA.
- Angiography can be used when biopsy results are negative, or it can be used to help guide biopsy by demonstrating areas of abnormality.
- Magnetic resonance angiography (MRA) has results
comparable to those of angiography in evaluating medium to large vessels.
39
Treatment of GCA
- Corticosteroids: The primary treatment for the disease is oral corticosteroids (prednisone) to reduce the inflammatory process.
40
Ankylosing spondylitis (AS)
- Tendency toward fibrosis and secondary ossification and ankylosis (stiffening) of involved joints.
- A classic feature of AS is the progressive and ascending bony fusion of the spine.
41
Epidemiology of AS
- Family history of ankylosing spondylitis is a strong risk factor for the disease.
- The risk for development of AS in an HLA-B27–positive relative of a case is approximately 20%.
- Racial distribution: the highest distribution is among whites, especially those of Scandinavian and Ugro - Finnish ancestry.
- Gender: common among men.
- Age: young men, onset is 15-35.
42
Clinical presentation of AS
- Young man between 15 and 40 years old who experiences the insidious onset of intermittent or persistent low back pain and stiffness.
- The pain is typically relieved by physical activity. It is usually centered in the lumbosacral spine.
- The cervical spine is ankylosed (fused) late in the course of the disease, leading to restriction in neck movement and head rotation.
- Eventually, the spine is completely rigid, with loss of the normal curvatures and movement.
43
Lab studies of AS
- HLA – B27
- Increased ESR
- Mild Anemia
44
Physical examination of AS
- Sacroiliac (SI) joints: Early signs include local tenderness over the SI joints and tenderness with paraspinal muscle spasm at lumbosacral vertebral levels.
- SI joint involvement may be elicited by special maneuvers to stress the joint.
- Spine: Loss of spinal motion (lateral motion, flexion, and extension) occurs early in most cases.
- With progression of disease, there is typically: loss of the normal lordosis, progressive kyphosis of the thoracic spine, fixed flexion of the neck, and ultimately a stooped posture with fixed flexion contractures of the hips and knees
45
Spine of AS
- AS is aptly named bamboo spine.
46
Treatment of AS
- Physical therapy (All patients should be enrolled in a PT program. Maintenance of erect posture is critical in all activities, including sitting, standing, and walking. The patient should sleep in a prone position or supine on a firm mattress with one small or no pillow. Walking and swimming are excellent ways to maintain joint mobility.)
- Medication
Indomethacin - most commonly prescribed drug.
NSAIDS -Other nonsteroidal antiinflammatory drugs, such as naproxen are reported to offer comparable pain relief.
Sulfasalazine - is a sulfa containing drug and is a strong anti-inflammatory.
=>Side effects: Neutropenia
DMARDS = methotrexate
Systemic steroids = helpful to treat acute flares but has side effects in long term.
Intraarticular corticosteroids = may occasionally be useful for acutely inflamed joints.
47
Surgery of AS
- It is usually reserved for patients with far-advanced disease causing painful deformities or loss of function.
- Total hip replacement is the most commonly performed procedure.
48
Prognosis of AS
- The course of AS varies. In some patients, the disease progresses relentlessly (often despite therapy), with fusion of axial and peripheral joints.
- In others, bony ankylosis may develop gradually with little pain or discomfort. In still others, skeletal involvement may be limited to only mild sacroiliitis and never progress to serious spondylitis or ankylosing disease.
