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Connective Tissue Diseases > Connective Tissue Diseases > Flashcards

Connective Tissue Diseases Flashcards

1
Q

DLE antibody

A

Anti-smith

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2
Q

Histo of DLE

A

interface dermatitis with lymphocytes
follicular plugging
perivascular and periadnexal infiltrate
increased mucin

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3
Q

DIF of DLE

A

continuous granular deposits of Ig and complement at DEJ

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4
Q

Where to biopsy DLE

A

Established lesional skin, but not burned-out scar

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5
Q

Lupus vulgaris

A

Cutaneous TB

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6
Q

DLE Treatment

A

First line: Sunscreen, Topical/IL steroids, Topical immunosuppressants
Second line: antimalarials, syst retinoids
Third line: cytotoxic agents, thalidomide, abx

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7
Q

Subacute LE Antibody

A

80% ANA +, speckled pattern

70% Ro/SSA +

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8
Q

HLA associated with SCLE

A

HLA-DR3

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9
Q

Drug-Induced SCLE

A 
HANG NAIL
HCTZ
ACE-I and Antihistamines
NSAIDs
Griseofulvin
Nifedipine
Anticonvulsants
Interferons
Lamisil
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10
Q

Antibody in neonatal lupus

A

Ro/SSA

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11
Q

Antibody in neonatal lupus that spares congenital heart block

A

U1RNP

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12
Q

C4 deficiency

A

hyperkeratosis palms and soles
annular SCLE lesions
homozygous = SLE, mesangial glomerulonephritis, membranous nephropathy

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13
Q

Homozygous C2 deficiency

A

severe infections
SLE
atherosclerosis

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14
Q

SLE ACR Criteria

A 
SOAP BRAIN MD (4+ criteria for dx)
Sun sensitivity
Oral ulcers
Arthritis
Pleuritis, pericarditis
Blood (hemolytic anemia, thrombocytopenia, leukopenia)
Renal ds
ANA+
Immunologic (anti-dsDNA, anti-Smith, antiphospholipid)
Neuro (seizures, psychosis)
Malar rash
Discoid rash
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15
Q

Rowell syndrome

A

SLE with erythema multiforme-like lesions

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16
Q

ASAP syndrome

A

Acute Syndrome of Apoptotic Pan-Epidermolysis associated with SLE

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17
Q

Bullous SLE HLA association

A

HLA-DR2

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18
Q

Bullous SLE antibodies

A

Type VII collagen antibodies

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19
Q

Bullous SLE histo

A

neutrophils at DEJ within dermal papillae

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20
Q

Bullous SLE treatment

A

Dapsone

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21
Q

Sneddon syndrome

A

AD in young women
livedo reticularis + cerebrovascular infarcts
due to hyalinaizing vasculopathy
may also have SLE

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22
Q

Hughes syndrome

A

antiphospholipid syndrome phenotype

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23
Q

SLE histo

A

vacuolar interface

mucinous

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24
Q

Antibodies specific for nephritis in SLE

A

anti-dsDNA, anti-C1q

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25
Q

Antibody for drug-induced SLE

A

antihistone

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26
Q

Drugs associated with drug-induced SLE

A 
MMC is HIPP
Methyldopa
Minocycline
Chlorpromazine
Hydralazine
Isoniazid
Procainamide
Phenytoin
also TNF-alpha inhibitors induce ANA positivity
Penacillamine unmasks SLE
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27
Q

DIF of SLE

A

granular IgG +/- IgM at DEJ
Lupus band
negative does not exclude diagnosis
non-lesional sun-exposed skin for bx

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28
Q

Gottron’s sign vs Gottron’s papules

A 
Sign = violaceous atrophic patches of knuckles, knees, elbows
Papules = lichenoid polygonal papules on knuckles
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29
Q

Cutaneous findings of Dermatomyositis

A 
Heliotrope rash
Gottron's sign
Gottron's papules
Poikiloderma (holster sign)
Shawl sign
Proximal nail fold telangiectasias
Ragged cuticles/mechanics hands
Calcinosis cutis
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30
Q

Antibodies of dermatomyositis

A

anti-Jo1 (antisynthetase) = reynaud’s, mechanic hands, interstitial lung ds
ANA 60-80%
anti-Mi-2 (anti-nuclear helicase) = good prognosis, shawl sign

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31
Q

Site of muscle biopsy for dermatomyositis

A

tricep or quad (not deltoid)

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32
Q

Markers to follow in dermatomyositis

A

CK, aldolase, ESR

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33
Q

Two types of childhood dermatomyositis

A

Brunsting (mc) = weakness, calcinosis, steroid responsive

Banker = vasculitis, GI, steroid unresponsive, high mortality

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34
Q

Childhood dermatomyositis gene

A

DQA1*0501

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35
Q

Dermatomyositis histo

A

atrophic epidermis
BMZ thickening
dermal mucin
perivascular and periadnexal lymphocytic infiltrate

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36
Q

Dermatomyositis Treatment

A

Prednisone 1mg/kg/day, slow long taper

methotrexate, myophenolate mofetil, azathioprine, IVIG, cyclosporin, tacrolimus, infliximab

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37
Q

Generalized morphea antibodies

A

antihistone (87%), anti-ssDNA

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38
Q

Anti-histone antibodies

A

generalized morphea, drug-induced SLE

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39
Q

Morphea histo

A

squared biopsy
atrophic epidermis
broad hyalinzed collagen bundles
lymphocytes and plasma cells

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40
Q

Atrophoderma of Pasini and Pierini presentation

A

brownish-gray, oval, atrophic lesions on posterior trunk of young women
Cliff drop sign
Asx

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41
Q

Risk of not treating morphea in children

A

impede limb development

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42
Q

Parry-Romberg Syndrome presentation

A 
progressive hemifacial atrophy
epilepsy
exopthalmos
alopecia
hyperpigmentation followed by atrophy of dermis, SQ, fat, muscle, sometimes bone
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43
Q

Parry-Romberg Syndrome antibodies

A

anti-ssDNA

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44
Q

CREST antibody

A

anticentromere

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45
Q

Systemic sclerosis major and 3 minor criteria

A

Major: proximal scleroderma to MCP
Minor: sclerodactyly, digital pitting scars, b/l basilar pulm fibrosis

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46
Q

Pterygium inversum unguis

A

distal nailbed adherent to ventral nail plate in scleroderma

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47
Q

Systemic sclerosis antibodies

A

anti-topoisomerase (anti Scl-70)
ANA - antinucleolar
anti-RNP (raynaud, polyarthralgia)

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48
Q

Major cause of death in systemic sclerosis

A

Pulmonary disease

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49
Q

Systemic sclerosis histo

A

perivascular lymphocytic infiltrate with plasma cells at dermis-SQ junction
hyalinized collagen bundles
dermal sclerosis
loss of SQ fat
“pepper-dot” epidermal nuclear reaction in centromere positive
loss of CD34+ dendritic cells

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50
Q

Three possible causes of scleroderma

A

Borrelia infection
Genetic susceptibility: Fibrillin-1
Exposures: L-tryptophan, rapeseed oil

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51
Q

Scleroderma treatment

A

PT, quit smoking
UVA1
D-penicillamine
system specific therapies: nifedipine, ACE-I

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52
Q

Eosinophilic fasciitis presentation

A

after strenuous exercise
peau d’orange or pseudo-cellulite skin
“groove sign” or “dry river bed sign”
eosinophilia

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53
Q

Eosinophilic fasciitis labs

A

ESR, TGF-alpha1, eosinophilia

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54
Q

Eosinophilic fasciitis histo

A

patchy lymphohistiocytic & plasma cell infiltrate in fascia and muscle
thickening of fascia and SQ septae

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55
Q

Eosinophilic fasciitis treatment

A

systemic steroids, histamine blockers

usually complete recovery in 1-3 years

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56
Q

Another name for Mixed Connective Tissue Disease

A

Sharp’s Syndrome

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57
Q

Mixed connective tissue disease presentation

A 
scleroderma, LE, DM, RA features
hand edema (sausage digits), Raynaud's, arthritis, pulm ds
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58
Q

Mixed connective tissue disease antibodies

A

ANA with particulate/speckled pattern

anti-U1 RNP

59
Q

MCTD treatment

A

prednisone 1mg/kg

60
Q

Nephrogenic systemic fibrosis presentation

A

hemodialysis

thickened, sclerotic, edematous papules/plaques sparing face

61
Q

Nephrogenic systemic fibrosis histo

A

spindle cells, new collagen bundles

no plasma cells

62
Q

Nephrogenic systemic fibrosis stain

A

CD34+

63
Q

Stiff skin syndrome gene mutation

A

Fibrillin-1 gene

64
Q

Stiff skin syndrome presentation

A

‘rock hard’ skin, esp buttocks/thighs
spares inguinal fold
no internal involvement

65
Q

Other names for Sjogren syndrome

A

Sicca syndrome, Mikulicz disease

66
Q

What are the forms of chronic cutaneous lupus

A

Discoid, hypertrophic, LE-LP overlap, Chilblain, Tumid, Lupus panniculitis

67
Q

Who does DLE most commonly affect

A

African American women (2:1), young adults

68
Q

What does Langue au chat refer to

A

cat’s tongue - carpet tack like follicular plugging that occurs in DLE

69
Q

What is the most common location for DLE to occur

A

Above the neck - concha bowl and scalp

70
Q

What do you need to worry about in long standing DLE

A

SCC

71
Q

Childhood DLE has a higher progression to?

A

SLE. Childhood DLE has equal incidence M:F

72
Q

What percentage of patients with DLE have SLE

A

5-15%

73
Q

What percentage of SLE patients have DLE

A

25%

74
Q

What percentage of patients with DLE will have a positive DIF on histology

A

75%

75
Q

LE-LP overlap syndrome most commonly affects what anatomical area

A

palmoplantar involvement. Also oral with scarring alopecia

76
Q

What mutation has been reported in familial forms of chilblain lupus?

A

TREX1 mutation

77
Q

How often does tumid lupus have overlying epidermal involvement?

A

Never

78
Q

Tumid lupus is considered an overlap with what other disease entities?

A

REM (reticular erythematous mucinosis) and jessner’s lymphocytic infiltrate

79
Q

What percentage of patients with tumid lupus will eventually develop SLE?

A

50%

80
Q

What percentage of patients with tumid lupus will have overlying DLE?

A

1/3

81
Q

What anatomical area does lupus panniculitis usually spare?

A

distal extremities

82
Q

What percentage of patients with SCLE will have SLE?

A

30%

83
Q

What percentage of patients with SCLE will have a positive ANA?

A

80%

84
Q

What antibody is often present in 70% of SCLE patients?

A

Ro/SSA

85
Q

10-15% of SCLE pts develop internal symptoms, what do these include?

A

Arthritis, leukopenia, renal, CNS, and vascular complications

86
Q

What is the common clinical description of SCLE lesions?

A

Sun exposed scaly, annular plaques in white women (15-40)

87
Q

30% of SCLE cases are drug-induced, what are the common causes?

A

HANG NAIL – HCTZ, ACE-I, NSAIDs, Griseofulvin, Nifedipine (CCBs), Anticonvulsants, Interferon, Lamisil

88
Q

What is the most important treatment for both DLE and SCLE?

A

Sunscreen

89
Q

Neonatal lupus is due to transferring of ___ antibody across placenta from affected mothers

A

Ro/SSA antibody

90
Q

What is the risk that a second child will have neonatal lupus?

A

25%

91
Q

What medication can lower the risk of neonatal lupus ~ 7% if initiated prior to 10 weeks gestation?

A

Hydroxychloroquine

92
Q

What is the most serious complication associated with neonatal lupus?

A

Congenital heart block

93
Q

If only __ ab is present in neonatal lupus, then there is no risk of heart block?

A

U1RNP

94
Q

What are the other associations important in neonatal lupus?

A

thrombocytopenia, hepatobiliary disease

95
Q

What is the clinical presentation seen in neonatal lupus?

A

periocular edema and erythema (‘raccoon’s eyes’) – none at birth, presents in first few weeks of life

96
Q

Who does SLE affect?

A

Young to middle aged women (6:1), and African Americans (4:1)

97
Q

Name the 11 criteria for SLE?

A

SOAP BRAIN MD – Serositis, Oral ulcerations, Arthritis, Pleuritis, Blood abnormalities (thrombocytopenia, leukopenia), Renal abnormalities (proteinuria), +ANA, Immunological abnormalities, Neurological abnormalities, Malar Rash, Discoid lesions

98
Q

What is Rowell Syndrome?

A

EM-like lesions occurring in Lupus

99
Q

What does ASAP syndrome stand for and what is its significance?

A

Acute Syndrome of Apoptotic Pan-Epidermolysis – TEN like presentation seen in SLE

100
Q

What does Bullous SLE attack? HLA associated?

A

Type VII Collagen – HLA DR2 prominence

101
Q

What medication does bullous SLE respond well to?

A

Dapsone

102
Q

What is Sneddon Syndrome?

A

Livedo reticularis, cerebrovascular infarcts, HTN ( may have SLE)

103
Q

What immunologic markers are specific for renal disease in SLE patients?

A

Anti-dsDNA, and anti-C1q

104
Q

What are the medications implicated in drug-induced SLE?

A

HIP MMC – Hydralazine, Isoniazid, Procainamide, Phenytoin, Minocycline, Methyldopa, Chlorpromazine

105
Q

What medication unmasks true SLE?

A

Penicillamine

106
Q

What is the age distribution seen in DM?

A

Bimodal – children (10-15 years old) and adults (40-65)

107
Q

Inflammatory muscle involvement without skin involvement is known as?

A

Polymyositis

108
Q

In DM, skin findings usually precede muscle findings by how long?

A

2-3 months

109
Q

T/F: Bullous DM is a good prognositic feature?

A

False – poor prognostic indicator – severe myopathy with lung disease

110
Q

What is the most important clinical feature seen in DM?

A

Poikiloderma

111
Q

Mechanic’s hands are associated with what antibodies?

A

anti-synthetase antibodies – indicated pulmonary involvement

112
Q

What labs do you want to check in your DM patients?

A

Aldolase, Creatine Kinase (PFT’s w/ CT if symptomatic, MRI with muscle biopsy if labs are positive)

113
Q

Where should patients have their muscle biopsies from?

A

triceps (as deltoids may not show histologic evidence of myopathy until later in the disease)

114
Q

Anti-Ku antibody is seen in what overlap syndrome?

A

Scleroderma and DM

115
Q

Anti-synthetase antibodies (Anti-Jo1, anti-PL7, anti-PL12, anti-DJ, anti-EJ) correlate with what?

A

interstitial lung disease in DM

116
Q

What are the most common malignancies seen in DM (10-50% of cases) in men, women, and asian men?

A

Men: Colon, Women: Ovarian, Asian: Nasopharyngeal

117
Q

What are the two types of childhood DM?

A

Brunsting (slow course, progressive weakness, calcinosis, steroid responsiveness)

Banker Type (vasculitis of muscles and GI tract, rapid onset, steroid unresponsive, and high death rate)

118
Q

T/F: Is there an increase in malignancy in childhood DM?

A

False – no increase in malignancy. Also noted to have an increase in calcinosis cutis.

119
Q

Anti-Mi2 has what type of prognosis in DM?

A

Good prognosis – shawl sign, cuticular disease

120
Q

Anti-CADM5 is associated with?

A

Interstitial lung disease in Japanese in DM

121
Q

Anti-p155/140 is associated with?

A

Cancer in DM – palmar involvement noted w/ this

122
Q

What antibodies are associated with generalized morphea?

A

Anti-histone, and anti-ssDNA

123
Q

What is Parry Romberg Syndrome?

A

Progressive hemifacial atrophy, epilepsy, exophthalmos, alopecia

124
Q

CREST Syndrome is a form of localized systemic sclerosis. What clinical features in seen in CREST?

A

CREST; C-calcinosis, R-Raynaud’s, E-esophageal dysmotility, S- sclerodactyly, T - telangiectasias

125
Q

What antibodies are seen in CREST syndrome?

A

Anticentromere ab

126
Q

What criteria is needed for diagnosis of Systemic Sclerosis:

A

1 major or 2 minor; Major: proximal scleroderma to MCP joints, Minor: Sclerodactyly, digital pitting scars of fingertips, b/l basilar pulmonary fibrosis

127
Q

What is the terms of the distal nailbed adhering to the ventral nail plate?

A

Pterygium inversum unguis

128
Q

What antibody is associated with systemic sclerosis?

A

anti-topoisomerase Scl-70

129
Q

Exposure to what other agents have resulted in scleroderma like reactions?

A

L-tryptophan, contaminated rapeseed (toxic oil syndrome), silicosis, bleomycin, vinyl chloride

130
Q

What organ is most commonly involved in systemic sclerosis?

A

esophagus (90%)

131
Q

What is the major cause of death in systemic sclerosis

A

pulmonary disease

132
Q

Groove sign, or dry river bed sign is seen in what disease?

A

Eosinophilic Fasciitis

133
Q

T/F: Do you have to seen eosinophils on H&E for diagnosis of eosinophilic fasciitis

A

False – also, only 10-40% of cases have peripheral eosinophilia

134
Q

What is Sharp’s syndrome?

A

Mixed Connective Tissue Disease – severe arthritis, hand edema (sausage digits), raynaud’s, esophageal dysmotility, pulmonary fibrosis (MC cause of death), lymphadenopathy

135
Q

What is the most common antibody seen in MCTD?

A

U1RNP

136
Q

Nephrogenic systemic fibrosis is often seen in what patients?

A

Patients on hemodialysis or pts recently exposed to gadalinium

137
Q

In the new scoring system, what histologic feature has the most points allocated for nephrogenic systemic fibrosis?

A

osseous metaplasia

138
Q

Stiff skin syndrome has been associated with what gene mutation?

A

Fibrillin -1 (also been implicated in systemic sclerosis) – sparing of inguinal folds

139
Q

What clinical features are seen in Sjogren syndrome?

A

Primary: xerostomia, keratoconjunctivits sicca; Secondary: 2 items mentioned + autoimmune connective tissue disease

140
Q

What association is most concerning with Sjogren’s syndrome?

A

Assoication with Non-hodgkin’s lymphoma

141
Q

What is the most definitive diagnostic tool for Sjogren?

A

Lower labial lip salivary gland biopsy

142
Q

What is the Schirmer test?

A

Whatman paper applied to the lower eyelid. Migration <5 mm over 5 min is abnormal

143
Q

80% of patients with Sjogren syndrome have what antibody?

A

Anti-Ro/SS-A

144
Q

What is MAGIC syndrome?

A

Behcet’s disease and relapsing polychondritis – directed against type-II collagen antibodies

Connective Tissue Diseases (1 decks)

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