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Connective tissue disorders and vasculitides Flashcards

1
Q

Common features of SLE

A 
Malar rash and photosensetivity
Pericarditis
Libman-sacks endocarditis
Increased risk of MI due to accelerated atherosclerosis
Nephritis
Fatigue, fever, malaise, weight loss
Inflammatory, symmetric, non-erosive arthritis
thrombosis
seizures, psychosis
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2
Q

SLE serology

A

Correlates with disease activity:
(+) ANA
(+) anti-ds DNA

Doesn’t correlate with disease activity:
(+) Sm (smith)

Complement activation promotes inflammation
decreased C3 or C4 means increased consumption

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3
Q

SLE treatment

A

avoid sun exposure, wear sunscreen
NSAIDS
Glucocorticoids (topical or systemic)
Hydroxychloroquine

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4
Q

SLE mortality in the EARLY years after diagnosis

A

Infections (especially from opportunistic organisms)

Active SLE, chiefly due to kidney or CNS disease

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5
Q

SLE mortality in the LATER years after diagnosis

A

Accelerated atherosclerosis- linked to 5x higher incidence of MI

thromboembolic events

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6
Q

Management/preventative measures of SLE

A

Minimize other conventional risk factors for atherosclerosis
Avoid smoking
Influenza vax every year
Pneumococcal vax every 5 years
preventative cancer screening due to increased risk

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7
Q

What should you watch for with management of SLE with corticosteroid use

A

monitor for avascular necrosis of bone

monitor for osteoporosis with long term use

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8
Q

Type 1 antiphospholipid antibody

A

causes biologic false-positive tests for syphilis

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9
Q

Type 2 antiphospholipid antibody

A

Lupus anticoagulant

risk factor for venous and arterial thrombosis and miscarriage

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10
Q

Type 3 antiphospholipid antibody

A

Anti-cardiolipin antibodies

directed at a serum cofactor Beta2GPI

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11
Q

Treatment for APS

A

anticoagulation continued indefinitely

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12
Q

SLE/APS

A

cotton wool spots

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13
Q

Characteristics of Lupus-like syndrome/drug induced lupus

A

promote demethylation of DNA
No renal or neurologic symptoms
(+) ANA
(+) Anti-histone antibodies

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14
Q

Some meds that can cause drug induce lupus

A 
Minocycline
methyldopa
quinidine
methyldopa
TNF inhibitors
procainamide
Isoniazid
Hydralazine
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15
Q

Sulfa drugs can lead to what?

A

SLE flare

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16
Q

Neonatal lupus affects children born of mothers with what?

A

*Anti Ro (SSA) Abs
Anti La (SSB) Abs
can potentially happen in Sjogren pts too

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17
Q

What is the major complication of neonatal lupus

A

Permanent complete heart block

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18
Q

What is discoid lupus erythematosus characterized by?

A

Well-defined inflammatory plaques that evolve into atrophic, disfiguring scars

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19
Q

Hallmarks of scleroderma

A

Thickening and hardening of the skin
Microangiopathy and fibrosis of the skin and visceral organs
Obliteration of eccrine sweat and sebaceous glands –> dry itchy skin

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20
Q

What is the first symptom seen in caucasian scleroderma patients?

In african american patients?

A

Secondary Raynaud phenomenon in caucasians

Hyper/hypopigmentation in AAs

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21
Q

Localized Scleroderma

A

in children
Discreet areas of discolored skin induration
NO Raynaud’s
NOT systemic
Histologically indistinguishable from systemic
patches = MORPHEA

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22
Q

Limited cutaneous systemic sclerosis

A

aka CREST syndrome

Cutaneous calcinosis
Raynaud's
Esophageal dysmotility (GERD)
Sclerodactyly
Telangiectasia
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23
Q

When is diagnosis of limited systemic sclerosis typically made

A

in advanced disease

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24
Q

What are the vascular manifestations of limited systemic sclerosis

A

vascular manifestations are more pronounced than dcSSc

digital ischemia
progressive pulmonary artery HTN (presents as SOB)

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25
What are the phases of diffuse cutaneous systemic sclerosis
Inflammatory edematous phase --> fibrotic phase Skin induration, hyper/hypopigmentation --> loss of body hair and impaired sweating Fibrotic joints --> stiffness
26
Symptoms of diffuse cutaneous systemic sclerosis
Soft tissue swelling, pruritis fatigue, stiffness, malaise arthralgia, muscle weakness, carpal tunnel Raynaud (later than in limited) Internal organ involvement - RENAL CRISIS and INTERSTITIAL LUNG DISEASE
27
What is the primary cause of morbidity and mortality in systemic sclerosis
Pulmonary involvement Aspiration pneumonia Increased incidence of bronchoalveolar carcinoma type specific complications
28
Which pulmonary manifestation is associated with diffuse cutaneous systemic sclerosis
Interstitial lung disease chronic dry cough, dyspnea, FINE VELCRO CRACKLES (rales) Diagnose by pulmonary function test (PFT) and lung CT
29
Which pulmonary manifestation is associated with limited cutaneous systemic sclerosis
Pulmonary artery hypertension Eight heart catheterization = elevated pulm artery pressure exertional dyspnea, syncope, angina, right heart failure
30
Renal manifestations of SSc
Chronic kidney disease Renal crisis- abrupt onset of malignant hypertension, hemolytic anemia, progressive renal insufficiency high dose glucocorticoids can induce crisis
31
MSK and Thyroid manifestations of SSc
Carpal tunnel syndrome hypothyroid from thyroid fibrosis
32
GI manifestations of SSc
``` primary biliary cirrhosis/cholangitis GAVE syndrome (watermelon stomach) Barrett esophagus (inc. risk of esophogeal adenoCA) ```
33
Common SSc serology
(+) ANA
34
Diffuse SSc serology
(+) Anti-Scl 70 (anti-DNSA topoisomerase I) (+) Anti-RNA polymerase III (+) ANA
35
Limited SSc serology
(+) anti-centromere (+) ANA
36
treatment/management of SSc
no therapy to date significantly alters disease course manage organ system involvement high doses of glucocorticoids may be associated with development of renal crisis
37
Characteristics of sjogren syndrome
Sicca symptoms (hella dryness) Keratoconjunctivitis sicca -foreign body sensation - inadequate tear production -check with schirmer test
38
What is sjogren syndrome strongly associated with?
B cell non Hodgkin lymphoma (MALT lymphoma)
39
Sjogren syndrome serology
polyclonal hypergammaglobulinemia (+) anti Ro/SSA (+) anti La/SSA (+) ANA (+) RF
40
What is essential for diagnosis of sjogren syndrome
lip biopsy parotid gland biopsy is for pts w atypical presentation
41
treatment/management of sjogren syndrome
Symptomatic treatment Avoid atropenic drugs and decongestants no immunomodulatory drug has proved efficacious
42
What are the characteristic skin lesions of dermatomyositis?
Gottron's patches/papules- raised violaceous lesions overlying dorsa of DIP, PIP, and MCP joints Heliotrope rash- periorbital edema, purplish suffusion over eyelids Periungual erythema V-neck erythema- Poikiloderma (shawl sign)
43
treatment of dermomyositis/polymyositis
glucocorticoids
44
describe dermatomyositis (DM)
weakness w/o sensory symptoms proximal muscles early --> distal muscles late in disease course
45
What do you look for once dermatomyositis is diagnosed
occult malignancy
46
What labs are associated with dermatomyositis
elevated creatine kinase and aldolase
47
What biopsy finding is associated with DM
perimysial and perivascular inflammation | perifascicular atrophy
48
Serology of DM
*Anti Jo-1* anti-M2, anti-MDA5, anti-P155/P140
49
What distinguishes polymyositis from DM
PM does not have skin changes like DM
50
What biopsy finding is associated with PM
endomysial inflammation with invasion of non-necrotic muscle fibers without features suggestive of another dx
51
Labs and serology associated with PM
Labs- elevated CK Serology- Anti Jo-1
52
Characteristics of inclusion body myositis (IBM)
more common in caucasian males | weakness in finger flexion or quads
53
biopsy findings with in IBM
rimmed vacuoles, endomysial inflammation, invasion of non-necrotic muscle fibers
54
Labs and serology findings in IBM
mild elevation or normal creatine kinase (CK) anti-cN1A autoantibodies
55
Treatment of IBM
IBM is refractory to treatment so it is only supportive
56
What are the small vessel systemic vasculitides
IgAV aka Henoch-Schonlein purpura (HSP) Anti GMB (goodpasture syndrome) Granulomatosis with polyangiitis (GPA)
57
What are the variable size systemic vasculitides
Eosiniphilic granulomatosis with polyangiitis (EGPA) Behcet syndrome
58
What are the medium vessel systemic vasculitides
Thromboangiitis obliterans (Buerger disease) Polyarteritis Nodosa (PN) Kawasaki DIsease (KD)
59
What are the large vessel systemic vasculitides
takayasu arteritis (TA) Giant cell arteritis (GCA)(Temporal arteritis)
60
What is the tetrad for IgA vasculitis/Henoch-Schonlein purpura (HSP)
1. Palpable purpura (NO THROMBOCYTOPENIA) 2. arthritis/arthralgia 3. abdominal pain 4. Renal disease
61
How is IgAV diagnosed? Treated?
A biopsy with IgA deposits found treatment is supportive and glucocorticoids
62
How is anti GBM aka goodpasture syndrome diagnosed?
A biopsy (renal usually): deposition of anti basement membrane autoantibodies a urinalysis
63
How is goodpasture syndrome treated
*plasmapharesis* glucocorticoids, cyclophosphamide
64
What is the cause of death if anti-GBM is not treated
pulmonary hemorrhage
65
What are the hallmarks of granulomatosis with polyangiitis (GPA)
Granulomatous inflammation necrotizing vasculitis segmental glomerulonephritis (renal involvement: hematuria/RBCs/proteinuria)
66
What is the respiratory tract invlovement in GPA
90% nasal involvement -saddle nose/crusting/bleeding/obstruction erosive sinus disease lung: CXR: cavitary lesions
67
Diagnosis of GPA
(+) ANCA Biopsy- vessel changes with granulomas
68
What are the hallmarks of Eosiniphilic granulomatosis with polyangiitis (EGPA) aka Churg-strauss syndrome
asthma and eosiniphilia --> vasculitis with granulomas
69
Diagnosis of EGPA
CBC with diff: high eosiniphil count Serology: ANCA (+) Imaging: CXR or lung CT Lung Biopsy: granulomas and vascular changes, eosinophils in tissue
70
Describe the phases of EGPA
Prodromal phase | Eosinophilia-tissue infiltr

SBL derm and HIV (5 decks)

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