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SBL derm and HIV > Connective tissue disorders and vasculitides > Flashcards

Connective tissue disorders and vasculitides Flashcards

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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
Q

What are the phases of diffuse cutaneous systemic sclerosis

A

Inflammatory edematous phase –> fibrotic phase

Skin induration, hyper/hypopigmentation –> loss of body hair and impaired sweating

Fibrotic joints –> stiffness

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26
Q

Symptoms of diffuse cutaneous systemic sclerosis

A

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

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27
Q

What is the primary cause of morbidity and mortality in systemic sclerosis

A

Pulmonary involvement

Aspiration pneumonia
Increased incidence of bronchoalveolar carcinoma
type specific complications

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28
Q

Which pulmonary manifestation is associated with diffuse cutaneous systemic sclerosis

A

Interstitial lung disease

chronic dry cough, dyspnea, FINE VELCRO CRACKLES (rales)
Diagnose by pulmonary function test (PFT) and lung CT

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29
Q

Which pulmonary manifestation is associated with limited cutaneous systemic sclerosis

A

Pulmonary artery hypertension

Eight heart catheterization = elevated pulm artery pressure
exertional dyspnea, syncope, angina, right heart failure

30
Q

Renal manifestations of SSc

A

Chronic kidney disease
Renal crisis- abrupt onset of malignant hypertension, hemolytic anemia, progressive renal insufficiency

high dose glucocorticoids can induce crisis

31
Q

MSK and Thyroid manifestations of SSc

A

Carpal tunnel syndrome

hypothyroid from thyroid fibrosis

32
Q

GI manifestations of SSc

A 
primary biliary cirrhosis/cholangitis
GAVE syndrome (watermelon stomach)
Barrett esophagus (inc. risk of esophogeal adenoCA)
33
Q

Common SSc serology

A

(+) ANA

34
Q

Diffuse SSc serology

A

(+) Anti-Scl 70 (anti-DNSA topoisomerase I)
(+) Anti-RNA polymerase III

(+) ANA

35
Q

Limited SSc serology

A

(+) anti-centromere

(+) ANA

36
Q

treatment/management of SSc

A

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
Q

Characteristics of sjogren syndrome

A

Sicca symptoms (hella dryness)
Keratoconjunctivitis sicca
-foreign body sensation - inadequate tear production
-check with schirmer test

38
Q

What is sjogren syndrome strongly associated with?

A

B cell non Hodgkin lymphoma (MALT lymphoma)

39
Q

Sjogren syndrome serology

A

polyclonal hypergammaglobulinemia
(+) anti Ro/SSA
(+) anti La/SSA

(+) ANA
(+) RF

40
Q

What is essential for diagnosis of sjogren syndrome

A

lip biopsy

parotid gland biopsy is for pts w atypical presentation

41
Q

treatment/management of sjogren syndrome

A

Symptomatic treatment
Avoid atropenic drugs and decongestants

no immunomodulatory drug has proved efficacious

42
Q

What are the characteristic skin lesions of dermatomyositis?

A

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
Q

treatment of dermomyositis/polymyositis

A

glucocorticoids

44
Q

describe dermatomyositis (DM)

A

weakness w/o sensory symptoms

proximal muscles early –> distal muscles late in disease course

45
Q

What do you look for once dermatomyositis is diagnosed

A

occult malignancy

46
Q

What labs are associated with dermatomyositis

A

elevated creatine kinase and aldolase

47
Q

What biopsy finding is associated with DM

A

perimysial and perivascular inflammation

perifascicular atrophy

48
Q

Serology of DM

A

Anti Jo-1

anti-M2, anti-MDA5, anti-P155/P140

49
Q

What distinguishes polymyositis from DM

A

PM does not have skin changes like DM

50
Q

What biopsy finding is associated with PM

A

endomysial inflammation with invasion of non-necrotic muscle fibers without features suggestive of another dx

51
Q

Labs and serology associated with PM

A

Labs- elevated CK

Serology- Anti Jo-1

52
Q

Characteristics of inclusion body myositis (IBM)

A

more common in caucasian males

weakness in finger flexion or quads

53
Q

biopsy findings with in IBM

A

rimmed vacuoles, endomysial inflammation, invasion of non-necrotic muscle fibers

54
Q

Labs and serology findings in IBM

A

mild elevation or normal creatine kinase (CK)

anti-cN1A autoantibodies

55
Q

Treatment of IBM

A

IBM is refractory to treatment so it is only supportive

56
Q

What are the small vessel systemic vasculitides

A

IgAV aka Henoch-Schonlein purpura (HSP)

Anti GMB (goodpasture syndrome)

Granulomatosis with polyangiitis (GPA)

57
Q

What are the variable size systemic vasculitides

A

Eosiniphilic granulomatosis with polyangiitis (EGPA)

Behcet syndrome

58
Q

What are the medium vessel systemic vasculitides

A

Thromboangiitis obliterans (Buerger disease)

Polyarteritis Nodosa (PN)

Kawasaki DIsease (KD)

59
Q

What are the large vessel systemic vasculitides

A

takayasu arteritis (TA)

Giant cell arteritis (GCA)(Temporal arteritis)

60
Q

What is the tetrad for IgA vasculitis/Henoch-Schonlein purpura (HSP)

A
  1. Palpable purpura (NO THROMBOCYTOPENIA)
  2. arthritis/arthralgia
  3. abdominal pain
  4. Renal disease
61
Q

How is IgAV diagnosed?

Treated?

A

A biopsy with IgA deposits found

treatment is supportive and glucocorticoids

62
Q

How is anti GBM aka goodpasture syndrome diagnosed?

A

A biopsy (renal usually): deposition of anti basement membrane autoantibodies

a urinalysis

63
Q

How is goodpasture syndrome treated

A

plasmapharesis

glucocorticoids, cyclophosphamide

64
Q

What is the cause of death if anti-GBM is not treated

A

pulmonary hemorrhage

65
Q

What are the hallmarks of granulomatosis with polyangiitis (GPA)

A

Granulomatous inflammation
necrotizing vasculitis
segmental glomerulonephritis (renal involvement: hematuria/RBCs/proteinuria)

66
Q

What is the respiratory tract invlovement in GPA

A

90% nasal involvement
-saddle nose/crusting/bleeding/obstruction

erosive sinus disease

lung: CXR: cavitary lesions

67
Q

Diagnosis of GPA

A

(+) ANCA

Biopsy- vessel changes with granulomas

68
Q

What are the hallmarks of Eosiniphilic granulomatosis with polyangiitis (EGPA) aka Churg-strauss syndrome

A

asthma and eosiniphilia –> vasculitis with granulomas

69
Q

Diagnosis of EGPA

A

CBC with diff: high eosiniphil count
Serology: ANCA (+)
Imaging: CXR or lung CT
Lung Biopsy: granulomas and vascular changes, eosinophils in tissue

70
Q

Describe the phases of EGPA

A

Prodromal phase

Eosinophilia-tissue infiltr

SBL derm and HIV (5 decks)

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