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Practice Topics Exam 8 > Rheum 7 - SLE and Scleroderma and Myositis > Flashcards

Rheum 7 - SLE and Scleroderma and Myositis Flashcards

1
Q

SLE is what

A

Systemic autoimmune disorder characterized by inflammation in multipl organ systms
Spectrum of mild to life threatening issues
May be charactrized by acute or chronic exacerbations and remissions

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2
Q

SLE demographics

A

Peak incidence is between1 5 and 40
W 10x more than M (F majority dec over time)

African descent have greater incidence

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3
Q

SLE etiology

A

Unknown
Sec steroids thought to play a role
Genetics might play role - monozygotic twins have 30% concordance rate, dizygotic and siblings have 5%

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4
Q

SLE - time to diagnosis

A

SLE can be very challenging to diagnose
Avg. time of 2 yrs between initial symptoms and diagnosis
Lupus antibodies develop over time - might be present 9 years prior to onset of clinical diseas

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5
Q

SLE - general s/s

A

Fever - might be due to infection
Fatigue - anemia, infection, meds, fibro
Lymphadenopathy - might inc with exacerbation
Alopecia - diffuse or patchy

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6
Q

ACR criteria for SLE - requirements

A

Need 4 of the 11 criteria
Criteria need not be present simultaneously
SLE evolves over time

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7
Q

ACR criteria for SLE -

A 
1 Malar rash
2 Discoid rash
3 Photosensitivty
4 Oral ulcers
5 Arthritis
6 Serositis
7 Renal disorder
8 Neuro disorder
9 Hematologic disorder
10 Immunologic disorder
11 Antinuclear antibody (ANA)
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8
Q

ACR criteria for SLE - Malar Rash

A

Butterfly rash describes the erythematous rash that covers the cheeks and nose while sparing the nasolabial folds
Might be flat or rasied
Might occur after exposure to sun
Might be hyperpigmented in darker skinned

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9
Q

ACR criteria for SLE - discoid rash

A 
Erythmatous
Raised patches
Adherent keratotic scale
Follicular plugging
Atrophic scarring might occur as lesions age
Might occur in ears (like gout)
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10
Q

ACR criteria for SLE - photosensitivity

A

Malar or discoid rashes might get worse with excessive sun exposure
Flares of SLE activity might happen after sun exposure, even if they are not aware of having photosensitivty

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11
Q

ACR criteria for SLE - Oral ulcers

A

Oral or nasal ulcerations

Might be painless

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12
Q

ACR criteria for SLE - arthritis

A

Nonerosive arthritis that involes 2 or more peripheral joints
Characterized by swelling, effusion, tenderness

Jaccoud’s deformities = reducible deformities caused by ligamentous laxity
In x rays will see NO EROSION of joints - but deformities occur from ligamentous laxity

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13
Q

ACR criteria for SLE - serositis

A

Pleuritis - rub heard, evidence of effusion

Pericarditis - EKG, rub on ascultation, effusion on imagign

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14
Q

ACR criteria for SLE - renal disorder

A

Kidney invovled in more than 1/2 of patients with SLE
Kidney bipsies are done to assess disease activity in pts with protein and casts in their urine
End stage lupus nephritis might be managed with dialysis and transplant

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15
Q

ACR criteria for SLE - neuro disorders

A

seizure OR psychosis
Other possible causes like drugs or metabolic disorders need to be ruled out
MRI of brain, CSF analysis, imaging, EEG in cases of seizures need to be done

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16
Q

ACR criteria for SLE - hematologic disorder

A

Anemia is most common abnormality in lupus
Leukopenia (less than 4000 on 2 separate occasions)
Thrombocytopenia - less than 100,000 platelets in absence of offending agents

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17
Q

ACR criteria for SLE - immunologic disorder

A

Anti DNA antibody
Anti Smith antibody
Pos findings of antiphospholipid antibodies - IgG or IgM, pos lupus anticoagulant

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18
Q

ACR criteria for SLE - Antinuclear antibody (ANA)

A

ANAs present in more than 99% of those with SLE
Absence of it rules out the diagnosis

Broad test that tells you that someone might have an autoimmune disease

Does not always mean that they have an autoimmune disease if they have a positive ANA though

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19
Q

Autoantibodies - SLE

A

Anti DNA (Anti double stranded antibody)_ IS VERY SPECIFIC FOR SLE
Associated with lupus nephritis
May correlate with disease activity

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20
Q

Lupus related syndromes - Subacute cutaneous lupus

A

Presents ONLY with skin involvement (no other systems)
Some evolve to SLE
Important to screen these pts for autoantibodies or renal invovlement

21
Q

Lupus related syndromes - Drug induced lupus

A

Present with fever, myalgia rash, arthritis, serositis

Associated with procainimide, isoniazid, hydralazine

Rarely associated with hematologic, renal, or CNS invovlement

Tx - remove precipitating agent, use NSAIDs

22
Q

Non pharm tx

A

Compliance is key!
Avoid sun exposure to avoid photosens
Naps and adjusmtnet to avoid fatigue
PT

23
Q

Pharm tx

A

NSAIDs (careful with kidneys though)
Hydroxychloroquine (plaquenil)
Corticosteroids
Immunosuppressives - methotrexate

24
Q

Routine lab monitoring

A

Kidney markers - BUN and creatinine
Urinalysis
CBC
C3 and C4

25
Q

Pregnancy and SLE

A

Normal fertility
SLE might flare during or post partum
Pts with lupus nephritis might have severe flares with acute renal failure, preeclampsia, and maternal death

26
Q

SLE flares

A

Episodic increases in disease activity
Labs that indicate flare = C3 and C4 (low compliments), Elevated ESR and CRP, proteinuria, elevated DS-DNA
Corticosteroids can be used to manage

27
Q

Scleroderma is what

A

multisystem disorder characterized by skin thickening and vascular abnormalities

28
Q

Scleroderma demographics

A

Rare
80% F
1/2 before age of 40
Higher prevalence in US

29
Q

Types of Scleroderma

A

Limited

Diffuse

30
Q

Limited Scleroderma

A 
Skin fibrosis in distal extrem and some areas of head and neck
CREST
Calcinois (calcification of skin)
Raynauds
Esophageal dysmotility 
Sclerodactyly (tightness hands)
Telangectasias
31
Q

Diffuse Scleroderma

A

Skin abnormalities extend to the proximal extremities (above elbow or knee) and trunk

32
Q

Scleroderma Findings - most common

A

Skin changes are hallmark of disease!!!
Skin thickening most noticeable in the hands (distal to prox)
Extremity, truncal and facial skin thickening might be seen
Area around the mouth is often affected
Raynaud phenomenon
Scars or open ulcerations on tips of fingers

33
Q

Scleroderma Findings - MSK

A

MSK - arthralgias, joint stiffness, palpable tendon friction rubs over flexor and extensor surfaces (grating noise with movement)

34
Q

Scleroderma Findings - GI

A

Dysphagia with esophageal dysmotility

Difficulty swallowing solid food or pills

35
Q

Scleroderma Findings -CP

A

Fibrosis of lung tissue might cause restrictive defect

Pulmonary hypertension

36
Q

Scleroderma Findings - renal

A

Ominous finding and impotnant cause of death in scleroderma

Used to be fatal before ACE inhibitors

37
Q

Scleroderma - diagnosis

A

Hx and physical exam
90% of pts have positive ANA - CREST in limited

Key = tightness of skin, esp hands associated with rraynauds is strongly suggestive

38
Q

Scleroderma - tx

A 
Raynauds = keep extremeties warm, VD agents like Ca channel blockers
Smoking cessation
Treat gastric reflux
Immunosuppressive agents = not helpful
PT and OT to help with ADLs
39
Q

Scleroderma - prognosis

A

Outcome is related to lung and kidney function
5 yr survivial without organ involvement = 90%, with is 50-70%
Pts with diffuse shorter survivial time than limited

40
Q

Idiopathic inflammatory myopathy (myositis) is what

A

Proximal mm weakness, inflammation and damage to skeletal mm with elevated mm derived proteins

41
Q

Types of myositis

A

Polymyositis - mm involvement primarily

Dermatomyositis - mm and skin

42
Q

Myositis - demographics

A

Peak incidence 40-60 yo
F:M is 2:1
Not hereditary

43
Q

Myositis - muscle

A

Symmetric, proximal mm weakness
Pain not common at onset
Leg and arm large mm weakness might affect stairs, walking, STS, arms overhead
Neck - might not be able to lift head off bed
Pharyngeal involvement - dysphagia, dysphonia, aspiration
Resp (diaphragm) involvement - SOB, resp failure, death

44
Q

Myositis - skin

A

Dermatomyositis - causes cutaneous manifestations

Gottron papules - raised, scaly, red lesions over MCP, PIP, knees (occurs in 70%)

Helitrope rash - purple rash over eyelids with or withour periprbital edema

Shawl sign - vneck pattern red rash in sun exposed areas

45
Q

Juvenile Myositis

A

Dermatomyositis is more common (20:1) than poly in kids
Peak age is 5-14
Rash precedes mm involvement

46
Q

Myositis and malignancy

A

9% of poly and 15% of dermato are associated with malignancy
CA may precede, be concurrent, or after More frequently is ovarian CA

47
Q

Myositis - diagnosis

A

Markers for mm damage are elvated - CK, Aldolase, AST, LDH
EMG - abnormal
MM biopsy confirms diagnosis

48
Q

Myositis - treatment

A

Corticosteroids - high or weeks then taper
Immunosuppressant - methotrexate or azathioprine
PT - improve function

Practice Topics Exam 8 (12 decks)

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