Term
| Name 4 rheumatic diseases with autoimmune etiology? |
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Definition
1)Rheumatoid arthritis
2)Systemic lupus erythematosus
3)Sjogren sydrome
4)Scleroderma |
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Term
| Autoimmune rheumatic diseases are characterized by the presence of 1 or more autoantibodies directed against what? |
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Definition
1 or more cellular components:
-Cell surface
-cytoplasm
-nuclear envelope
-nucleus of the cell |
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Term
| Autoantibodies to nuclear antigens is the hallmark of what diseases? |
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Definition
| systemic rheumatic diseases |
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Term
| What is a general definition for rheumatoid arthritis? |
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Definition
| Autoimmune polyarticular disease affecting joints in symmetrical pattern. |
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Term
| Give the general order in which RA starts in the joints |
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Definition
-MCP-PIP-MTP-Wrists-Knees-Elbows-Ankles-Hips-Shoulders
**Advanced cases: cervical spine- temporamandibular and sternociavicular joints |
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Term
| What are the main differences between rheumatoid arthritis and osteoarthritis? |
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Definition
-Osteoarthritis involves the spine
-RA has a larger involvement in the hands and feet |
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Term
| Why is it important to adequately treat RA early in the disease process? |
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Definition
| RA will typically lead to severe, disabling joint deformities w/in a few months. Also extra-articular manifestations |
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Term
| List some of the extraarticular manifestations of rheumatoid arthritis (7) *Include examples |
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Definition
1)Skin: rheumatoid nodules on extensor surfaces and pressue points
2)Bone: risk factor for osteoporosis
3)Blood: anemia of chronic disease
4)Eyes (scleritis, episcleritis, rarely uveitis): scleritis may lead to scleromalacia perforans
5)Lungs: interstitial fibrosis, rheumatoid nodules in the lung, pleural disease
6)Heart: coronary artery disease; risk factor for MI, pericarditis, myocarditis
7) Neurologic: peripheral neuropathy, numbness in hands and feet |
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Term
| What is the age of onset for RA? |
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Definition
Patient may present at any age.
-Highest incidence found among persons aged 40-70.
-Women in late childbearing age are most commonly affected
-Men in the sixth to eighth decade are most commonly affected |
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Term
| What is the lifetime risk of developing RA for women? men? |
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Definition
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Term
| What is the gender preference for RA? |
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Definition
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Term
What is the genetic contribution to RA?
Do most patients have a positive family history?
What 2 genes appear to predispose patients to RA? |
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Definition
1)~65%
2)No
3)HLA-DR4 and HLA-DR1 |
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Term
| Describe the pathophysiology of rheumatoid arthritis? |
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Definition
-Autoimmune in nature w/ antibodies attacking the joint synovia
-Involves both the innate and adaptive immune system with various cell types and cytokines. |
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Term
| What are the two key players in joint destruction? |
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Definition
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Term
| What are the common clinical features of RA? (8) |
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Definition
1)Inflammation of joints, tendons, and/or bursae
2)Early morning joint stiffness lasting >1 hr & easing w/ physical activity
3)Inflammation leads to: joint swelling (synovitis), joint tenderness, decreased range of motion, & morning stiffness.
4)Fatigue
5)Weakness
6)Weight loss
7)Low-grade fevers (less specific)
8)Activities of daily living are affected |
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Term
| What is a very characteristic factor of RA, having to do w/ initial pattern of joint involvement? |
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Definition
| -Generally symmetrical. Most commonly polyarticular. Some may present w/ oligoarticular involvement but then it is at least 3 joints. |
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Term
Clinical features of RA:
What joints are involved first?
Name 3 classical joints involved? |
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Definition
1)Small joints involved first. Large joints come later in the course of the disease.
2)PIP, MCP, and MTP |
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Term
| List the most important positive findings for the diagnosis of RA? (5) |
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Definition
1)Joint swelling and pain
2)Positive antibodies (rheumatoid factor (RF) and cyclic citrullinated peptide (CCP))
3)Erosive changes on radiography
4)Joint symptoms lasting >6 wks, worse in the morning w/ improvement w/ movement
5)Elevated acute-phase reactants (ERS, CRP) |
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Term
According to ACR diagnosis (score based on joints involved), a patient w/ a score of ____ is likely to have RA.
ex: 0= 1 large joint,1= 2-10 large joints,2= 1-3 small joints, 3= 4-10 small joints, 5= >10 joints |
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Definition
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Term
| What are the 3 pharmacologic classes used in the treatment of RA? |
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Definition
1)Adjunctive medication (analgesics)
2)Nonbiologic DMARDs
3)Biologic DMARDs
*want to target the immune system |
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Term
| What is systemic lupus erythematosus (SLE)? |
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Definition
A chronic autoimmune multisystem disease w/ a fluctuating course.
-commonly affects skin & joints
-can involve any body system |
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Term
| Who is most commonly affected by systemic lupus erythematosus? |
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Definition
-childbearing year for women aged 20-45 (80% cases), but does occur in all age groups of both sexes before 8 yrs old
-<15 yrs of age, incidence 0.5/100,000 in US |
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Term
| The diagnosis of SLE is made on what? |
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Definition
| Clinical grounds and the presence of antibodies to nuclear antigens (ANA) and to ds-DNA |
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Term
| What are the most common presentation/signs and symptoms of SLE? |
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Definition
-butterfly rash on face or malar flush
-low-grade fever
-nondeforming arthritis
**range of presentation that depends on which body systems are involved |
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Term
| How do you control symptoms of SLE, mild? severe? |
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Definition
1)Rest, NSAIDs for arthraigia and hydroxychloroquine for rashes, avoidance of sunlight
2)Systemic corticosteroids and immunosuppressive agents, including DMARDs |
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Term
What is the incidence of getting systemic lupus erythematosus?
What is the prevalence? |
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Definition
1)varies from 1.8-7.6/100,000 per year in the US
2)20/100,000 in the US |
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Term
| What is gender and ethinicity are more commonly affected by SLE? |
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Definition
-Female: male ratio vary from 3:1 to 9:1 in adults, and 2:1 in prepubertal children
-More common in African Americans |
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Term
| What is the initiating event in systemic lupus erythematosus? |
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Definition
Event: abnormal form of apoptotic cell death, where cells undergo apoptosis and a antigenic bleb for self-DNA forms on the surface of the dead cell
-impairment of normal phagocytic clearance processes for apoptotic cells
-antigenic apoptotic cells are transferred to lymphoid tissue & taken up by APCs for presentation to T-cells
-activated T-cells stimulate B-cells to produce antibodes which attack antigenic material, forming immune complexes that deposit in tissue |
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Term
| What are some of the adverse environmental exposure settings in which the abnormal form of apoptotic cell death occurs in SLE? |
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Definition
-sunlight exposure (associated w/ disease onset and flares)
-viral infection (Epstein-Barr virus exposure associated w/SLE in children)
-Certain drugs |
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Term
| How is drug-induced Lupus different from SLE? |
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Definition
Epidemiologically:
-Male to female distribution equal.
-Average age of onset 50yrs
Clinically:
-arthralgias or arthritis
-1/2 patients have serositis
Serologically:Drug induced SLE
-95% patients are antihistone +
-Sm and dsDNA are rarely +
Serology: SLE
-most patients dsDNA +
-80% patients antihistone + |
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Term
| List the most common drugs involved in drug-induced Lupus? (3 most common) (7 others) |
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Definition
Most common:
1)Isoniazid
2)Hydralazine
3)Procainamide
Others:
1)Minocycline
2)Aldomet
3)Diltiazem
4)Infliximab
5)Sulfas
6)Amiodarone
7)Beta blockers |
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Term
What is a malar rash?
What is a discoid rash? |
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Definition
1)fixed erythema sparing the nasolabial fold
2)erythematous patches w/ keratotic scaling. scarring |
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Term
| List some other clinical presentations of SLE? (12) |
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Definition
1)Fever: low-grade (active disease) or high fever (possible infection)
2)Malaise and chronic fatigue
3)Skin rashes: malar rash and discoid rash
4)Scalp lesions and alopecia
5)Photosensitivity
6)Mucocutaneous: ulcers in nose & mouth
7)Pain & swelling joints: polyarthritis is symmetrical, nonerosive, & nondeforming
8)Arthralgia: severe pain in joints w/ only tenderness found clinically
9)Chest complaints: hemoptysis, dyspnea, orthopnea, chest pain
10)Vasculitic rashes & splinter hemorrhages in nail bed
11)Peripheral extremity changes induced by cold: Raynaud's phenomenon
12)Abdominal pain & tenderness: enlarged liver & spleen, constipation, vascular insult, peritoneal serositis, chronic pancreatitis |
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Term
| What are some cerebral symptoms of SLE? (6) |
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Definition
1)Mood disturbance (rare psychosis)
2)Cognitive dysfunction
3)Agoraphobia
4)HA (occasional migrainous
5)Movement disorders (St Vitus dance)
6)Memory loss |
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Term
| The most common form of neurologic involvement is ____ ___, occuring in __% of SLE patients 10 years after diagnosis. |
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Definition
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Term
| What are the ocular presentations of SLE? (5) |
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Definition
1)scleritis
2)episceritis
3)KCS, uveitis
4)rare optic neuritis
5)cotton wool spots |
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Term
| Describe the sensory or sensorimoter clinical presentations for SLE? |
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Definition
-symptoms of a stroke may be apparent
-spinal cord disease may mimic MS |
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Term
| What 2 diseases/conditions must be ruled-out when diagnosing SLE? |
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Definition
1)Chronic cutaneous lupus (rarely progresses to systemic disease-5%)
2)Drug-induced lupus erythematosus |
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Term
| For a patient to be diagnosed with SLE they must. . . |
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Definition
-Have biopsy-proven lupus nephritis w/ ANA or anti-dsDNA
-OR: satisfy 4 of ACR criteria, including at least 1 clinical and 1 immunologic ctriterion
ex: ACR criteria
Derm: malar rash, discoid rash, photosensitivity
Oral ulcers; serositis; arthritis; nephritis
Neurologic: psychosis, seizures, stroke |
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Term
| For a patient to be diagnosed with drug-induced SLE they must. . . |
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Definition
-Have acute lupus-like clinical signs and symptoms not involving a major organ
-Antihistone antibodies w/out dsDNA
-Concurrent use of drug considered to induce SLE |
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Term
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Definition
-Autoimmune disease should be dealt w/ using DMARDs (biologic & nonbiologic)
-NSAIDs (antiinflammatory doses)
-Intermittent corticosteroids |
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Term
| How do you treat drug-induced SLE? |
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Definition
-Stop implicated medication first (disease tends to resolve w/in 6 months of drug discontinuation)
-NSAIDs
-Antimalarials
-Ocassionally steroids and cytotoxic agents for drug-induced vasculitis or serositis |
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Term
| What is Sjogren Syndrome (SS)? |
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Definition
| A systemic autoimmune disease that mainly affects the exocrine glands and usually presents as persistent dryness of the mouth & eyes due to functional impairment of the salivary and lacrimal glands |
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Term
| What is the histologic characteristic of Sjogren on a biopsy? |
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Definition
| Focal lymphocytic infiltration of exocrine glands |
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Term
| True/False. Sjorgrens is classified secondary when symptoms are found in association w/ other systemic autoimmune diseases (RA, SLE). |
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Definition
| True. Only primary disease in otherwise healthy patients |
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Term
| What are some non-sicca signs and symptoms of Sjogrens syndrome? |
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Definition
1)Fever
2)Generalized pain
3)Fatigue
4)Weakness
5)Sleep disturbances
6)Anxiety and depression
**much greater impact on quality of life of patients than sicca features |
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Term
What is the prevalence of Sjogrens?
What is the incidence? |
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Definition
1)~2-4 million persons
2)4/100,000 primary
Secondary SS: 11-19% in SLE patients; 7% in RA patients |
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Term
| Who is most commomly affected by Sjogrens? Ethnicity, gender, age |
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Definition
-Primarily affects Caucasian premenopausal women
-Female:male ratio: 14:1 to 24:1
-All age groups are affected, onset typically between 40-60 yrs old |
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Term
| What areas of the body are affected by Sjogrens that have clinical presentation? (14) |
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Definition
1)Mouth- oral dryness
2)Eyes- corneal ulcers, conjunctivitis
3)Skin- cutaneous dryness, lesions
4)Nose and Throat- dysphagia
5)Joints- arthraigia, non-erosive symmetric arthritis
6)Lungs-obstructive chronic pneumopathy, lung disease
7)Cardiovascular-Raynaud phenomenon, pericarditis, autonomic disturbances
8)Liver-associated w/ Hep C virus infection, primary biliary cirrhosis, & autoimmune hep
9)Kidneys-renal tubular acidosis, glomerulonephritis
10)CNS- peripheral neuropathy (common), cranial nerve involvement (V, VII, & VIII)
11)GIT- altered esophageal motility, chronic gastritis, less frequent malabsorption syndromes
12)Ears- sensorineural hearing loss
13)Thyroid- 1/3 have thyroid disease
14)Salivary glands- chronic or episodic swelling of major salivary glands (10-20% patients) |
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Term
| Describe the clinical presentation seen in mouth of a patient w/ Sjogrens? % affected, signs, symptoms |
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Definition
-Xerostomia occurs in >95% of patients
-oral dryness (xerostomia), soreness, caries, periodontal disease, oral candidiasis, parotid swelling
-Surface of tongue becomes red & lobulated w/ partial or complete depapillation |
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Term
| Describe the clinical presentation seen w/ skin of a patient w/ Sjogrens? |
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Definition
-Exocrine glands of skin leads to cutaneous dryness
-Females: dryness of vagina & vulva may result in dyspareunia & pruritus
-Wide spectrum of cutaneous lesions, small-vessel vasculitis most frequent
-Photosensitive to UV exposure |
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Term
| Describe the clinical presentation seen in nose and throat of a patient w/ Sjogrens? |
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Definition
-Reduction or absence of respiratory tract glandular secretions lead to dryness of nose, throat, & trachea
-Lead to hoarseness & chronic, nonproductive cough
-Dysphagia: difficulty swelling present |
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Term
| Describe the clinical presentation seen in joints of a patient w/ Sjogrens? |
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Definition
-Arthraigia, non-erosive symmetric arthritis
-Joint involvement, generalized arthraigias, seen in 25-75% of patients
-Joint deformity & mild erosions rare, except for cases associated w/ RA |
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Term
| About how many (%) of patients w/ primary Sjogrens have thyroid disease? What is most common? |
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Definition
1/3
Subclinical hypothyroidism most frequent, especially in patients w/ antithyroid autoantibodies |
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Term
| Describe the clinical presentation seen in salivary glands of a patient w/ Sjogrens? |
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Definition
-Chronic or episodic swelling of major salivary glands (parotid & submandibular glands) reported in 10-20% of patients
-Start unilaterally but become bilateral |
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Term
| What are the 4 lab diagnoses for Sjogrens? |
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Definition
1)Elevated ERS
2)Leukopenia & thrombocytopenia
3)Positive ANA and/or RF: secondary SS associated w/ other rheumatic disease (inflam markers present & elevated)
4)Salivary gland biopsy: focal lymphocytic sialadenitis in sampled glands |
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Term
| What are focal lymphocytic sialadenitis? |
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Definition
| Multiple, dense aggregates of 50 or more lymphocytes in perivascular or periductal areas |
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Term
| How do you treat a patient for Sjogrens? |
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Definition
1)Address the symptoms- ex: dry eye w/ artifical tears
2)Treat andy underlying autoimmune rheumatic disease
3)Severe cases: treat w/ DMARD therapy |
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Term
|
Definition
Relatively rare chronic disorder characterized by diffuse fibrosis of skin and internal organs (hardening).
Present in virtually all patients w/ Raynaud phenomenon and antinuclear antibodies |
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Term
| Scleroderma is a systemic disease that most commonly targets what? (7) |
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Definition
1)Peripheral circulation
2)Muscles
3)Joints
4)Gastrointestinal tract
5)Lung
6)Heart
7)Kidney |
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Term
| What is the prevalence of scleroderma? |
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Definition
| ~250 patients/million in US |
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Term
| What is the gender affect of scleroderma and age of onset? |
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Definition
Female to Male ratio= 4-5:1
Average age of diagnosis: ~50 yrs |
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Term
| List the systemic features, in order of frequency, for scleroderma? (9) |
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Definition
1)Raynaud phenomenon (vascular constriction in digits)
2)Gastroesophageal reflux (w/ or w/out dysmotility)
3)Skin changes
4)Swollen fingers
5)Arthraigias
6)Pulmonary fibrosis
7)Pulmonary/ Systemic hypertension
8)Renal involvement: scleroderma renal crisis (SRC) develops in ~10% of patients. Onset malignant hypertension.
9)Fatigue |
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Term
| What is the most dramatic clinical feature of scleroderma? |
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Definition
-Skin fibrosis (thickening
-Prominent skin changes: marked telangiectasia that occurs on skin of face, palmer surface of hands, & mucus membranes |
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Term
| What symptoms are encountered in the early presentation of scleroderma? |
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Definition
1)Musculoskeletal discomfort
2)Fatigue
3)Weight loss
4)Heartburn associated w/ Gastroesophageal reflux disease |
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Term
| What are the 2 types of scleroderma? |
|
Definition
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Term
| Describe limited scleroderma? |
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Definition
-long history of Raynaud phenomenon
-gastroesophageal reflux & dysphagia
-swelling or skin thickening of fingers
-infrequent systemic symptoms such as arthralgias, weight loss, & dyspnea |
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Term
| Describe diffuse scleroderma? |
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Definition
-new onset of Raynaud phenomenon
-rapid change in skin texture w/ new onset of edema, pruritus, and pain
-signif. systemic symptoms w/ severe arthraigias, weight loss, & tendon friction rubs
-early evidence of internal organ involvement such as dyspnea or hypertension |
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Term
| The American College of Rheumatology diagnosis criteria for scleroderma includes what? |
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Definition
-either thickened skin changes proximal to metacarpophalangeal joints or at least 2 of the following:
-Sclerodactyly
-Digital pitting
-Bibasilar pulmonary fibrosis |
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Term
| How would you treat scleroderma? |
|
Definition
-No single disease modifying drug exists
-treat each individual organ dysfunction ex: treat renal hypertension w/ ACE inhibitor |
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