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DOOS 2 > MSK2-Table 1 > Flashcards

MSK2-Table 1 Flashcards

1
Q

What is systemic lupus erythematosus (SLE)?

A

A multisystem autoimmune connective tissue disease characterized by the presence of numerous autoantigens, autoantibodies, and circulating immune complexes with widespread immunologically determined tissue damage

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2
Q

What pt populations does SLE more frequently affect?

A

Non-white, AA more susceptible with severe disease, 90% female of child bearing age

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3
Q

When is SLE most frequently diagnosed?

A

Between 15-45 years old

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4
Q

What causes early death in SLE?

A

Active lupus and infection

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5
Q

What causes late deaths in SLE?

A

Sequelae of chronic lupus, chronic medications, CVD, infections

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6
Q

What is the most likely pathogenesis of SLE?

A

Likely genetic component in the setting of immune dysregulation, environment, and hormonal triggers

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7
Q

What environmental triggers are implied with SLE?

A

UV lights, microorganisms, tobacco, others

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8
Q

What are the 2 ways SLE autoantibodies cause tissue injury?

A

1) Formation of immune complexes and depostition of Ig at dermal- epidermal jxn and in renal tissue
2) damage or destruction of specific cells causing inflammation, activated PMNs, and release of oxygen radicals and lysozymes that damage and destroy tissues

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9
Q

What drugs can induce SLE?

A

Procainamide, hydralazine, isoniazid, methyldopa, quinidine, chlorpromazine, phenothiazines, OCPs, anticonvulsants

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10
Q

What are the clinical features of SLE?

A

Arthritis, arthralgia, and fever

Specifically polyarthralgia

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11
Q

What is the most common constitutional symptom in SLE?

A

Fatigue, occurs in >90%

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12
Q

What is the onset of symptoms like?

A

Gradual onset with flares and remission

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13
Q

What can happen with pregnancy and SLE?

A

May onset during pregnancy and may be present in women with hx of spontaneous abortion
Babies SGA

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14
Q

What vascular condition is common in SLE?

A

Raynauds

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15
Q

2/3 of pts with SLE have what?

A

Skin signs- lupus dermatitis

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16
Q

How might the skin conditions present?

A

vasculitis, purpura or periungual erythema. Classic malar butterfly rash and discoid lesions

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17
Q

What CNS manifestations present in SE?

A

Epilepsy, depression, dementia, psychosis, hemiplegia, chorea, ataxia, pripheral neuropathy

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18
Q

What are abdominal manifestations in SLE?

A

Ab pain, peritonitis, splenomegaly, pancreatitis, GI ulcers

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19
Q

What are ocular manifestations in SLE?

A

Keratoconjunctivitis, episcleritis, retinal vasculitis & retinal exudates

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20
Q

What is diagnostic criteria for SLE?

A

4 or more of the following during the course of the disease:
-—Malar Rash —Oral Ulcers
—Arthritis
—Serositis (heart/lungs)
—Renal Disease (proteinuria, cellular casts)
—Hematologic Disorders (hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia)
—Immune Disorders (including false + syphilis)
—Neurological Disorders (sz., psychosis)
—Discoid Rash
—Photosensitivity
—+ANA

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21
Q

what immunologic criteria is most common in lupus?

A

Positive ANA

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22
Q

How should SLE be managed?

A

REFER

- NSAIDS, exercise, sun protection, smocking cessation, antimalarials, corticosteroids

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23
Q

What is the mainstay tx for SLE and what needs to be monitored?

A

Antimalarials - Watch for retinal toxicity

- baseline and annual eye exams with visual field testing

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24
Q

When are corticosteroids mainstay?

A

If antimalarials are insufficient

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25
What corticosteroid combo is superior for preventing renal failure in SLE?
Prednisone + cyclophosphamide
26
What should be used to tx SLE in pregnancy?
hydroxychloroquine and prednisone/prednisolone
27
What comorbid conditions are associated with SLE that need to be assessed for and tx?
- -Accelerated atherosclerosis - -Pulmonary hypertension - -Antiphospholipid syndrome - -Osteopenia or osteoporosis - -Non-Hodgkin lymphoma
28
What preventative measures can be taken with SLE?
—Sun protection (clothing and sunscreen) —Vaccination (influenza and pneumococcal) —Caution with live-attenuated vaccines and immunosuppression —Suppress recurrent infections (UTIs) —Prevent osteoporosis (steroids) —Prevent and control CVD and obesity
29
What are the general characteristics of sjogrens syndrome?
Autoimmune disorder causing destruction of salivary and lacrimal glands- exocrine
30
What is the most common pt population for sjogrens syndrome?
Most common in middle aged females (9:1 ratio female: male)
31
What is the pathophys involved in sjogrens?
B lymphocyte hyperactivity Infiltration of the exocrine glands by lymphocytes, resulting in inflammation and destruction of tissue Infiltration of viscera and skin leading to extrglandular conditions
32
What are the cardinal clinical features of sjogrens?
Exocrine malfunction: dry mouth, dry eyes, enlargement of the parotid glands Constitutional: fatigue, fever, wt loss, lymphadenopathy Extraglandular: articular and raynaud phenomenon, B cell lymphomas
33
What is the diagnostic criteria for sjogrens?
4 of 6 of the following or 3 or 4 of the objective criteria ( III, IV, V, VI) I. Ocular symptoms II. Oral symptoms (dry mouth, swollen glands) III. Ocular signs (Shirmer’s test, Rose Bengal) IV. Histopathology (biopsy minor salivary glands) V. Salivary gland testing VI. Reactive antibodies to RO/SS-A or La/SS-B
34
What are other tests that can be done to diagnose sjogrens?
Shirmers test- evaluates tear secretions, less than 5mm in 5 min is positive Corneal abrasion- stain with slip lamp evalv Biopsy of lower lip mucosa- confirms lymphocytic infiltrate gland fibrosis
35
What is the treatment of sjogrens?
Symptomatic tx- artificial tears and saliva, ocular and vaginal lubricants, oral lubricants ,Protect eyes, Increase PO intake NSAID- for arthralgia Antimalarials/ methotrexate- 1st line for extraglandular Nifedipine and prostacycline for raynauds ACEI for HTN
36
What is systemic sclerosis (scleroderma)?
Multi-system autoimmune dz of unknown cause with chronic and progressive features
37
What is the hallmark of scleroderma?
Vasculopathy and excess collagen deposits
38
What does scleroderma lead to?
Fibrosis and degenerative changes of skin and internal organs Ulcerations, ischemia, atrophy and gangrene of the skin and digits
39
What are early manifestations of scleroderma?
Raynauds and skin calcinosis | Wrist pain, wheezing, skin pigments, joint pain, hair loss and alopecia, eye burning itching or discharge
40
When it the typical age of onset in scleroderma?
30-50
41
What gender is most affected by scleroderma?
Females… 4.6: 1 ratio
42
What are the 2 types of systemic sclerosis?
Diffuse cutaneous | Limited cutaneous- crest syndrome
43
What does diffuse typically affect?
Skin, heart, lungs, GI tract, and kidneys | Typically worse!
44
What does crest syndrome affect?
Skin of the face, neck, elbows, knees In late stages it may cause isolated pulmonary HTN Raynauds associated with critical ischemia of the digits
45
What are the cardinal clinical features of scleroderma?
Skin – most begin with swelling in the fingers and hands, may spread to the trunk or the face Raynauds Polyarthralgias and esophageal dysfxn Tendon or bursal friction rubs often seen prior to thickening of the skin
46
What are the systemic and MSK changes in scleroderma?
Arthritis, arthralgia, crepitus of tendon sheaths, non-progressive myopathy with weakness and atrophy
47
What are the skin changes in systemic sclerosis?
Non pitting edema- “sausage” swelling of fingers Skin shiny and atrophied with fingertip ulcers Decreased facial pigment and presence of telangiectasias Tightening over bony prominences Flexion contractures Loss of adipose tissues Symmetric and bilateral skin changes
48
What is the hallmark skin change in crest?
Telangiectasias
49
What is the mneumonic for CREST?
``` Calcinosis Raynauds Esophageal dysfxn Sclerodactyly Telangiectasias ```
50
What are GI changes associated with Systemic sclerosis?
Reflux esophagitis, hiatal hernia, loss of normal peristalsis, dilatation of large and small bowel, pain, constipation, diarrhea, primary biliary cirrhosis
51
What are some complications of scleroderma?
Cardiac failure, renal failure (emergency), pulmonary fibrosis, R heart failure, malabsorption, malnutrition, osteoporosis
52
How is scleroderma diagnosed?
Primarily a clinic diagnosis
53
Can scleroderma be cured?
No, only supportive and organ specific tx
54
What are the organ specific tx for scleroderma?
Antihistamines and low-dose steroids for early skin involvement PPI for GERD ACEI for Renal Ca+ Channel Blockers or ARBs for Raynaud’s Bosentan or sildenafil for pulmonary HTN PT with daily stretching to avoid contracture
55
What is polymyalgia rheumatica?
inflammatory rheumatic condition characterized clinically by aching and morning stiffness in the shoulders, hip girdle, neck, and torso
56
What are 15% of pts with PMR also diagnosed with?
Temporal arteritis ( GCA)- complication is blindness
57
What gender is more commonly affected?
Female >male, 2-3:1
58
What is the typical pt population?
Female, northern European, age around 50 but peaks 70-80
59
What gene is PMR associated with?
HLA_DR4
60
What are the cardinal clinical features of PMR?
``` Stiffness is predominant symptom in shoulders, hip girdle, neck, and torso ÷Most severe after rest and in the AM ÷Stiffness may last more than 30 minutes ÷Bilateral, proximal, symmetrical ÷Onset may be abrupt ```
61
What are other symptoms of PMR?
systemic malaise, fatigue, depression, anorexia, weight loss, and low-grade fever
62
How is PMR diagnosed?
Empirical criteria: - Age >50 at onset - Proximally and bilaterally distributed aching and morning stiffness (lasting at least 30 minutes or more) persisting for at least two weeks. The stiffness should involve at least two of the following three areas: neck or torso, shoulders or proximal regions of the arms, and hips or proximal aspects of the thighs - Erythrocyte sedimentation rate (ESR) ≥40 mm/hr - Rapid resolution of symptoms with low-dose glucocorticoids. Symptoms are generally 50 to 70 percent better within three days in patients with PMR started on prednisone at a dose of 10 to 20 mg/day. The lack of response to initial therapy strongly suggests an alternative diagnosis
63
What MUST you assess for if your pt is diagnosed with PMR?
GCA- >50, fever, elevated ERS, anemia with temporal artery tenderness, HA
64
How is PMR treated?
Low dose corticosteroid therapy - Prednisone 15mg PO Qday - may need for up t 2 years - higher dose plus ASA is GCA
65
What is fibromyalgia?
A controversial chronic pain syndrome characterized by persistent, widespread pain and tenderness to palpation at defined tender points in soft tissue structures
66
What is the prevalence of fibromyalgia?
Female : male = 9:1 ration
67
How is fibromyalgia diagnosed?
Diagnosis of exclusion | r/o: inflammatory disorders and thyroid by obtaining CBC, ESR, CRP, and TSH
68
What is fibromyalgia strongly associated with?
Mood disorders
69
What are the cardinal clinical features of fibromyalgia?
- -Non-articular, diffuse and poorly localized musculoskeletal pain, usually accompanied by fatigue and sleep disturbance- “Pain all over!” - --Diffuse tenderness on exam - ---constellation of additional symptoms- fatigue, stiffness, poor sleep, panic disorder, depression, HA/ “migraine”, irritable bowel, dysmenorrhea, and parasthesias may be seen
70
Are narcotics indicated for tx of fibromyalgia?
NO
71
How is fibromyalgia managed?
—Amytriptyline 10mg 1-3 hours prior to bed, titrate to 25-50mg —Cyclobenzaprine is an alternative SNRIs may be helpful if fatigue is severe —Anti-convulsants- gabapentin and pregabalin (for sleep disturbance and pain) —Pregabalin dose- 25-50mg PO QHS and titrate up to 300-450mg QDAY Aerobic exercise to improve conditioning is very important --- education and psych counseling

DOOS 2 (14 decks)

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