Autoimmune Connective Tissue Disorders

Question 1

SLE can have a particulate ANA pattern

Answer 1

True - so can MCTD

Question 2

Nuceolar pattern of ANA corresponds with SLE

Answer 2

False - scleroderma

Question 3

SLE can have a homogeneous or rim ANA pattern

Answer 3

True

Question 4

Neonatal LE is a variant of SCLE

Answer 4

True

Question 5

List diseases associated with SCLE

Answer 5

-	Associated diseases:
o	Sjogren syndrome
o	RA
o	Sweet syndrome
o	Crohn disease
o	LP
o	Psoriasis
o	Hereditary angioedema
o	PCT
o	GSE
o	TEN
o	Morphoea and dystrophic calcinosis cutis have sometimes followed presentation of SCLE

Question 6

List cutaneous features of antiphospholipid syndrome

Answer 6

-	Cutaneous features:
o	Livedo reticularis
o	Nail changes – nail fold ulcers
o	Vasculopathy
o	Anetoderma
o	Sneddon’s , superficial thrombophlebitis  migrans

Question 7

Acute cutaneous LE is associated with usually fixed lesions

Answer 7

False - usually transient

Question 8

SCLE induced by ..?

Answer 8

induced by hydrochlorothiazide, TNF-a inhibitors, terbinafine, antiepileptics and PPI

Question 9

Dyspigmentation a common consequence of SCLE

Answer 9

True

Question 10

SCLE can be annular (1/3), polycyclic (2/3) but do not demonstrate bullae or vesicles

Answer 10

False - can demonstrate vesiculation, crusting and occasional bullae along the borders

Question 11

SCLE can leave telangiectases

Answer 11

True - no scarring but can leave hypopigmentation and telangiectases

Question 12

Compare SCLE Histo to DLE

Answer 12

SCLE has more epidermal atrophy,
Less BM thickening, hyperkeratosis, follicular plugging and inflammation
Colloid bodies and epidermal necrosis in >50% of cases

Question 13

Midfacial skin is usually involved in SCLE

Answer 13

False- spared

Question 14

Acute cutaneous lupus spares the nasolabial fold

Answer 14

True

Question 15

Presence of small erosions can aid in the clinical differential diagnosis, favouring acute cutaneous lupus

Answer 15

True

Question 16

In lupus panniculitis , tissues are swollen, brawny, warm and tense

Answer 16

False - this is tumid lupus

Question 17

Tumid lupus occurs most commonly on the upper and lower limbs

Answer 17

False - face and torso

Question 18

Jessner’s lymphocytic infiltrate is morphologically similar to tumid lupus

Answer 18

True

Question 19

Tumid lupus has a high prevalence of SLE

Answer 19

False - low prevalence ,with relative lack of serological abnormalities

Question 20

Lupus panniculitis - does not occur in children

Answer 20

False - can occur in children in association with NLE and partial C4 deficiency

Question 21

Lupus panniculitis more common in males

Answer 21

False - females

Question 22

Discoid lupus lesions can overlie lupus panniculitis

Answer 22

True

Question 23

Lupus panniculitis is a septal panniculitis

Answer 23

False - lobular

Question 24

Lupus panniculitis comprise non-tender subcutaneous nodules and plaques arising in crops

Answer 24

False - tender , rest is true

Question 25

Lupus panniculitis often occurs in lower extremities

Answer 25

False - unlike erythema nodosum

Question 26

Lupus panniculitis can present with lipoatrophy or induration

Answer 26

True

Question 27

Chilblain lupus shows tuft resorption on x-ray

Answer 27

True

Question 28

Chilblain lupus - fingers often show curious atrophic swindling sometimes with hyperextended terminal phalanges and nail dystrophy

Answer 28

True

Question 29

Chilblain lupus - 5% develop SLE

Answer 29

False - 15%

Question 30

NLE develops in the first few days of birth

Answer 30

True, but can also present up to 12-16 wk post partum

Question 31

What are the 3 types of hepatobiliary involvement with NLE?

Answer 31

1 . Fulminant liver failure presenting at or shortly after birth 2. Cholestasis a few weeks after birth 3. Transient mild to moderate transaminase elevations a few wk or months after birth

Question 32

How to monitor LFT for NLE

Answer 32

At diagnosis, then every 3 months until 9 months old

Question 33

What is criteria for bullous lupus ?

Answer 33

1. Fulfils criteria for SLE according to ARA criteria 2. Histo reminiscent of DH 3. Bullous lesions in photodistributed areas 4. linear or granular deposits of IgG +/- IgA +/- C3 at DEJ and immune reactants below the basal lamina by electron microscopy

Question 34

Blistering in bullous lupus often parallels systemic flares of SLE particularly affecting the kidneys

Answer 34

True

Question 35

Bullous lupus response to dapsone is often very gradual

Answer 35

False = response can be dramatic with bullae ceasing after 1-2 days

Question 36

Neonatal lupus erythematosus can present as a blueberry muffin baby

Answer 36

True

Question 37

List complications of NLE

Answer 37

1. Cardiac mortality 20% and 2/3 need PPM 2. DIC 3. Scarring/teles 4. Development of AI CT later in life 5. Cardiovascular problems later in life 6. Risk in subsequent pregnancy 25% 7. Risk of NLE in anti-SSA and women 2% F/U need repeat LFT every 3 mo until 9 mo old

Question 38

Drugs that induce dermatomyositis

Answer 38

``` CHILL TNFa Cyclophosphamide Hydroxyurea Ipilimumab (checkpoint inhibitors) Lipid lowering agents - statins>gemfibrozil Losec (PPIs) TNF-a inhibitors ```

Question 39

Risk of malignancy with adult dermatomyositis

Answer 39

15-25%

Question 40

Infections that can induce dermatomyositis

Answer 40

E. Coli Echovirus Picornavirus Cocksackie virus

Question 41

What are features of Anti-Jo1 DM

Answer 41

``` High incidence of ILD Raynaud phenomenon Mechanics hands EROSIVE POLYARTHRITIS PROXIMAL MUSCLE WEAKNESS Antisynthetase syndrome ```

Question 42

Features of Anti-TIFy

Answer 42

Malignancy associated myositis in adult disease, extensive cutaneous disease in adult or juvenile DM, Palmar hyperkeratotic papules Psoriasiform lesions Red on white telangiectatic patches Ovoid palatial patch

Question 43

Anti-NXP2

Answer 43

Malignancy associated myositis Adult and juvenile DM with calcinosis , peripheral oedema, myalgia, dysphagia Mild skin disease

Question 44

Anti-SAE1

Answer 44

Interstitial Lung disease and dysphagia | Amyopathic dermatomyositis

Question 45

Anti-Mi2

Answer 45

Milder muscle disease Cutaneous disease Classic Gottron/Shawl/Ragged cuticles Adult or juvenile DM

Question 46

Anti-MDA5/CADM-40

Answer 46

Amyopathic DM | Cutaneous ulcerations - periungal region and overlying Gottron papules and Gottron sign

Question 47

Anti-SRP (signal recognition protein)

Answer 47

Acute onset necrotising myopathy (severe weakness, high CK0, which may be refractory to treatment. Incr cardiac mortality

Question 48

How does limited SSc differ from diffuse SSc

Answer 48

Limited SSc - CREST - more gradual progression and longstanding Raynaud phenomenon - later onset of internal organ involvement - PAH more common/ILD less common - more favourable long term prognosis Diffuse SSc - sudden onset of Raynaud’s phenomenon - Rapidly progressive , more widespread cutaneous sclerosis. Usually peaks within 12-18 months - >90% have internal organ involvement within the first 5 years - ILD>PAH - kidney disease

Question 49

Telangiectases are associated with the severity of vasculopathy in systemic sclerosis

Answer 49

True (JAAD July 2018 article)

Question 50

What are findings on respiratory function test in SSc

Answer 50

Decreased FEV1, decr FVC but incr ratio FEV1/FVC due to decline in FVC>decline in FEV1 , Ratio >80% Decreased DLCO

Question 51

What are differentials for scleroderma ?

Answer 51

MENSES TOXIN GVHD GENE - morphoea - eosinophilic fasciitis - nephrogenic systemic fibrosis - scleromyxoedema - endocrine: diabetic cheiroarthropathy, PCT - scleroderma TOXINS: silicosis, vinyl chloride, toxic oil, gadolinium, bleomycin, taxanes GVHD Genetic: restrictive dermopathy , Werner syndrome, Hutchinson-Gilford/ progeria , stiff skin syndrome, pachydermoperiostosis, ataxia-telangiectasia, Huriez syndrome