Autoimmune Connective Tissue Disorders Flashcards
SLE can have a particulate ANA pattern
True - so can MCTD
Nuceolar pattern of ANA corresponds with SLE
False - scleroderma
SLE can have a homogeneous or rim ANA pattern
True
Neonatal LE is a variant of SCLE
True
List diseases associated with SCLE
- 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
List cutaneous features of antiphospholipid syndrome
- Cutaneous features: o Livedo reticularis o Nail changes – nail fold ulcers o Vasculopathy o Anetoderma o Sneddon’s , superficial thrombophlebitis migrans
Acute cutaneous LE is associated with usually fixed lesions
False - usually transient
SCLE induced by ..?
induced by hydrochlorothiazide, TNF-a inhibitors, terbinafine, antiepileptics and PPI
Dyspigmentation a common consequence of SCLE
True
SCLE can be annular (1/3), polycyclic (2/3) but do not demonstrate bullae or vesicles
False - can demonstrate vesiculation, crusting and occasional bullae along the borders
SCLE can leave telangiectases
True - no scarring but can leave hypopigmentation and telangiectases
Compare SCLE Histo to DLE
SCLE has more epidermal atrophy,
Less BM thickening, hyperkeratosis, follicular plugging and inflammation
Colloid bodies and epidermal necrosis in >50% of cases
Midfacial skin is usually involved in SCLE
False- spared
Acute cutaneous lupus spares the nasolabial fold
True
Presence of small erosions can aid in the clinical differential diagnosis, favouring acute cutaneous lupus
True
In lupus panniculitis , tissues are swollen, brawny, warm and tense
False - this is tumid lupus
Tumid lupus occurs most commonly on the upper and lower limbs
False - face and torso
Jessner’s lymphocytic infiltrate is morphologically similar to tumid lupus
True
Tumid lupus has a high prevalence of SLE
False - low prevalence ,with relative lack of serological abnormalities
Lupus panniculitis - does not occur in children
False - can occur in children in association with NLE and partial C4 deficiency
Lupus panniculitis more common in males
False - females
Discoid lupus lesions can overlie lupus panniculitis
True
Lupus panniculitis is a septal panniculitis
False - lobular
Lupus panniculitis comprise non-tender subcutaneous nodules and plaques arising in crops
False - tender , rest is true
Lupus panniculitis often occurs in lower extremities
False - unlike erythema nodosum
Lupus panniculitis can present with lipoatrophy or induration
True
Chilblain lupus shows tuft resorption on x-ray
True
Chilblain lupus - fingers often show curious atrophic swindling sometimes with hyperextended terminal phalanges and nail dystrophy
True
Chilblain lupus - 5% develop SLE
False - 15%
NLE develops in the first few days of birth
True, but can also present up to 12-16 wk post partum
What are the 3 types of hepatobiliary involvement with NLE?
1 . Fulminant liver failure presenting at or shortly after birth
- Cholestasis a few weeks after birth
- Transient mild to moderate transaminase elevations a few wk or months after birth
How to monitor LFT for NLE
At diagnosis, then every 3 months until 9 months old
What is criteria for bullous lupus ?
- Fulfils criteria for SLE according to ARA criteria
- Histo reminiscent of DH
- Bullous lesions in photodistributed areas
- linear or granular deposits of IgG +/- IgA +/- C3 at DEJ and immune reactants below the basal lamina by electron microscopy
Blistering in bullous lupus often parallels systemic flares of SLE particularly affecting the kidneys
True
Bullous lupus response to dapsone is often very gradual
False = response can be dramatic with bullae ceasing after 1-2 days
Neonatal lupus erythematosus can present as a blueberry muffin baby
True
List complications of NLE
- Cardiac mortality 20% and 2/3 need PPM
- DIC
- Scarring/teles
- Development of AI CT later in life
- Cardiovascular problems later in life
- Risk in subsequent pregnancy 25%
- Risk of NLE in anti-SSA and women 2%
F/U need repeat LFT every 3 mo until 9 mo old
Drugs that induce dermatomyositis
CHILL TNFa Cyclophosphamide Hydroxyurea Ipilimumab (checkpoint inhibitors) Lipid lowering agents - statins>gemfibrozil Losec (PPIs) TNF-a inhibitors
Risk of malignancy with adult dermatomyositis
15-25%
Infections that can induce dermatomyositis
E. Coli
Echovirus
Picornavirus
Cocksackie virus
What are features of Anti-Jo1 DM
High incidence of ILD Raynaud phenomenon Mechanics hands EROSIVE POLYARTHRITIS PROXIMAL MUSCLE WEAKNESS Antisynthetase syndrome
Features of Anti-TIFy
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
Anti-NXP2
Malignancy associated myositis
Adult and juvenile DM with calcinosis , peripheral oedema, myalgia, dysphagia
Mild skin disease
Anti-SAE1
Interstitial Lung disease and dysphagia
Amyopathic dermatomyositis
Anti-Mi2
Milder muscle disease
Cutaneous disease
Classic Gottron/Shawl/Ragged cuticles
Adult or juvenile DM
Anti-MDA5/CADM-40
Amyopathic DM
Cutaneous ulcerations - periungal region and overlying Gottron papules and Gottron sign
Anti-SRP (signal recognition protein)
Acute onset necrotising myopathy (severe weakness, high CK0, which may be refractory to treatment.
Incr cardiac mortality
How does limited SSc differ from diffuse SSc
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
Telangiectases are associated with the severity of vasculopathy in systemic sclerosis
True (JAAD July 2018 article)
What are findings on respiratory function test in SSc
Decreased FEV1,
decr FVC but incr ratio FEV1/FVC due to decline in FVC>decline in FEV1 , Ratio >80%
Decreased DLCO
What are differentials for scleroderma ?
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
