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Flashcards in Connective Tissue Disease Deck (65):
1

Signs of active disease in DLE

Perifollicular erythema and easily extractable anagen hairs

2

Childhood DLE

Lower rate of photosensitivity and a higher rate of systemic involvement

3

DLE DIF

Old lesional skin

4

Concomitant DLE in SCLE

20%

5

SCLE DIF

Granular fluorescence throughout the cytoplasm and nucleus, particulate

6

SCLE drugs

Hydrochlorothiazide MC per Andrews

ACE inh, CCB's, IFNs, AEDs, griseofulvin, glyburide, piroxicam, penicillamine, spironolactone, terbinafine, statins

7

Acral dermal mucinosis or telangectasias

May predominate in some NLE cases

*tel may occur in non sunexposed areas and be persistent

8

HLA in NLE


DR-3

Japanese infants have anti-dsDNA and 8% progress to SLE

9

C' deficiency associated with lupus

C2 and C4
Tend to have SCLE annular lesions
C4 may have hyperkerarosis of palms and soles

10

Skin involvement in SLE

80%

11

DIF in SLE

IgG, IgM, IgA or C3 in a granular pattern at BMZ

12

Nailfold changes in SLE vs DM

SLE - wandering capillary loops
DM/scleroderma - symmetrical dilation and drop out

HHT - ecstasia of half of the capillary loop

13

Lupus hairs

Short frontal hairs from chronic telogen effluvium and increased fragility

14

Palisaded neutrophilic and granulomatous dermatitis

May be seen in SLE, RA or other IC mediated disease

15

Systemic symptoms in SLE

IC mediated

16

MC earliest systemic sx in SLE

Arthralgias

17

MC COD in SLE

First 5 years - infection and inflammatory SLE lesions
Late - thromboses

18

MC cardiac finding in SLE

Pericarditis

19

Raynauds in SLE

15%
Less renal dz associated = less morality

20

Have been reported in DLE evolving to SLE

Insulin receptor antibodies

21

UV sensitivity in SLE

Both UVA and UVB

22

Gene susceptibility in lupus

Defective CRP response in flares
APRIL (TNF alpha family) polymorphism sin SLE
Increased expression of TNF alpha and IFN inducible protein myxovirus protein A noted in cutaneous lupus
Polymorphisms in C1qA are associated with systemic and cutaneous lupus

23

Immune alterations in lupus

Reduced T suppressor function

Overproduction of gamma globulins and B cells and reduced clearance of IC may contribute to C' mediated damage

Externalizations of cellular ag like SSA in response to sun,ignited may lead to cell injury by antibody dependent cellular toxicity

HLA-DR4 people, who are slow acetylators, predisposed to hyralazine induced LE

Penacillamine and etanercept may unmask disease with anti dsDNA

Pegylated IFN and ribavirin have produced SLE

L-canavanine (alfalfa sprouts) - can induce or worsen SLE

Smoking associated with increased disease activity and interferes with antimalarial drugs

Globulin increased, especially gamma and alpha 2 fraction

24

MC urine findings in lupus

Albumin, RBC's, and casts

25

ANA positive rate in SLE

95%

26

Lupus band

Continuous granular along the Dej in 75% of well established DLE lesional skin

Positive in sun exposed skin in SLE

A positive test in non sun exposed skin correlates with dsDNA and renal disease

27

SLE

Increased risk of osteoporosis independent of steroids

28

Lupus treatment

Sun avoidance
vit d and ca supplementation
Local - steroids, CCI, ILTAC
Systemic - antimalarials (consider another agent if no response in 3 mo) --> retinoids --> immunosuppressants --> rituximab

steroids for flares (limit to 3 weeks unless renal or CNS)

29

Antimalarial side effects

Ocular, erythroderma, EM, purpura, urticaria, nervousness, tinnitus, Abducens nerve paralysis, leukopenia, thrombocytopenia, psoriasis, N/V/D

*may exacerbate (except in small doses) or cause hepatic necrosis in PCT

30

Tocilizumab

IL-6 inhibitor
May cause neutropenia

31

Wong type DM

DM with clinical findings of PRP

32

In DM: anti jo 1, anti pl-7, anti pl-12, anti-DJ and anti EJ

Correlate with pulmonary disease which is frequent COD

33

Higher risk of malignancy in DM

Highest in first 2 years of dx, age, constitutional sx, rapid onset, lack of raynauds, super high esr or ck, LCV

34

Childhood DM

Brunsting type - slower course, progressive weakness, calcinosis, steroid responsive

Banker - vasculitis of muscle and GI tract, rapid onset of severe weakness, steroid unresponsiveness, high death rate

*malignancy not associated but insulin resistance might

35

Initial immune response in DM

IFN alpha and beta induced with secondary stimulation of IFN gamma

36

Juvenile DM

DQA1*0501 in 85%

TNF alpha 308A allele associated with increased TNF synthesis and thrombospondin-1 and small v. Occlusion

Strep m protein may have homology to myosin

37

Adult DM

IL-1 alpha, TGF beta, myoblast production of IL-15 may be pathogenic

Terbinafine DM cases related to apoptosis induced by the drug

38

Path of DM vs lupus

More likely to become atrophic, less eccrine, fewer vertical columns of lymphocytes and fibrous tract remnants

39

ANA pos rate in DM

60-80%

40

Crest eponym

Thibierge-Weissenbach syndrome

41

MC initial presentation of SS

Raynauds

42

Scleroderma nail fold changes

Dilated capillary loops, nailfold capillary hemorrhage in 2 or more fingers

*HHT has dilation of only 1/2 of of the loop with no avascular areas

43

Childhood scleroderma

Less raynauds, more cardiac (1/2 deaths)

44

Major COD in scleroderma

Pulmonary

45

MC organ involved in scleroderma

Esophageal in 90%

46

Telangectasias in scleroderma associated with

Pulmonary vascular disease

47

MC cancers in scleroderma

Malignancy associated in 10%
Lung and breast MC

48

Most specific ANA Pattern in scleroderma

Anti nucleolar

49

Linear scleroderma ab

Anti-SS DNA

50

Sclerodermoid conditions

Occupational exposure to silica, epoxy resins, polyvinyl chloride, vibratory stimuli

Bleomycin, INH, pentazocaine, valproate sodium, vit k injections, Spanish rapeseed oil, tryptophan, nitrofurantoin, hydantoin

Presenting scleroderma like sx may be 1st sign of multiple myeloma and amyloidosis

51

Pathogenesis of scleroderma

Anticardiolipin and anti beta2 glycoproteins play a role
Plasma d diner correlates with macrovascular complications

Unregulated proteins - monocyte chemoattractant protein 1, pulmonary and activation-regulated chemokine, macrophage inflammatory protein-1, IL-8, PDGFR-beta, and TGF beta --> these factors stimulate ECM production, TGF-beta production and activation and chemoattraction of T cells

PHET (protein highly expressed in testes) - ab noted in diffuse cutaneous scleroderma and lung involvement

CD40 increased on fibroblasts - binding with ligand increases IL-6, 8, and monocytes chemoattractant protein 1

Etanercept shown to decrease TGF-beta1, tissue hydroxyproline, dermal fibrosis, and alpha SMA positive cells - bc TH2 cells reduce net collagen 1 synthesis through TNF alpha, biological should be used with caution

52

Balicatib

Cathepsin K inhibitor that causes drug induced morphea

53

HFS with sclerodactyly

Capecitabine

54

Raynauds treatment

Stop smoking - 3 to 4 times more likely to have digital vascular complications

Nifedipine is first line - may use diltiazem if worsening esophageal function

Vasodilation drug (CCBs, angiotensin II R antagonists, topical nitrates and prostanoids), ginkgo, L-arginine may reverse digital necrosis

55

Eosinophilic fasciitis

May occur after strenuous exercise
Indurstion of forearms and legs, peau d orange, hands/face spared, dry riverbed sign
Steroid responsive , hydroxyzine and cimetidine may also help
Environmental triggers - L-tryptophan, borrelia, trichloroethylene

Polycythemia vera, metastatic colorectal CA, multiple myeloma have been associated

May see sys sx- carpal tunnel, neuropathy, sz, optic neuropathy, pleuropericard eff, pancytopenia, hemolysis, sjogrens, LAD, pernicious anemia and IgA nephropathy

Striking elevation in TGF-beta1
TH17 mediated pathway

Eosinophilia in 10-40%

56

MCTD DIF

Particulate epidermal nuclear IgG deposition

57

Anti-TS1 RNA antibodies

MCTD with lupus like clinical features

58

Ab noted in NSF

Anti phospholipid

59

Immunostains in NSF

Increased CD34 and procollagen 1 - cells may represent circulating fibroblasts recruited to the dermis

60

Skin findings in sjogrens

Vasculitis (accounts for most patients with waldenstroms hypergamma) pruritus, xerosis, annular erythema, decreased sweating

61

Pathogenesis of sjogrens

Aquaporin family of water channels is imp
CD4 cells
IL-12 and IFN gamma upregulated
Th1 cytokines mediate the functional interactions bw APC's and cd4 cells

62

Increased malignancy in sjogrens

NHL

*patients with palpable purpura, low C4 and cryoglobulinemia at a higher risk of malignancy

63

Sjogrens Tx

Pilocarpine and cevimeline to stimulate salivation
Topical CSA and topical IFN for oral lesions
Acid maltose losenges

64

JIA

Evanecsent eruption, neutrophilic panniculitis
IL1 beta, IL6, IL18 and s100 proteins important in patho

65

Correlate with dz activity in relapsing polychondritis

Elevated ESR, CRP and urinary type II collagen levels
Th1 disease