CTD: Sjogren, Scleroderma, DM. Lecture review Qs Flashcards
what is MAGIC syndrome and what are the antibodies against
behcet’s disease and relapsing polychondritis
antibodies against collagen type 2
80% of patients with Sjogren syndrome have what antibody
anti-ro/SS-a
what is the schirmer test
whatman paper applied to lower eyelid
migration <5mm over 5 min abnormal and indicates Sjogren syndrome
what is the most definitive diagnostic tool for Sjogren
lower labial lip salivary gland biopsy
what malignancy is associated with Sjogren syndrome
non-Hodgkin’s lymphoma
what clinical features are seen in Sjogren syndrome
primary: xerostomia, keratoconjunctivitis sicca
secondary: autoimmune connective tissue disease
stiff skin syndrome has been associated with what gene mutation
fibrillin-1
sparing of inguinal folds
fibrillin-1 mutation is implicated in what two CTDs
stiff skin syndrome
systemic sclerosis
in the new scoring system, what histiologic feature has the most points allocated for nephrogenic systemic fibrosis
osseous metaplasia
nephrogenic systemic fibrosis is seen in what patients
pts on hemodialysis or pts recently exposed to gadalinium
what is the most common antibody in mixed connective tissue disease
U1RNP
what is sharp’s syndrome
mixed connective tissue diease
severe arthritis, hand edema (sausage digits), raynaud’s, esophageal dysmotility, pulmonary fibrosis, lymphadenopathy
T/F: you have to see eosinophils on H&E for diagnosis of eosinophilic fasciitis
false
also only 10-40% of cases have peripheral eosinophilia
groove sign, or dry river bed sign, is seen in what disease
eosinophilic fasciitis
what is the major cause of death in systemic sclerosis
pulmonary ds
what organ is most commonly involved in systemic sclerosis
esophagus (90%)
what is the term for the distal nailbed adhering to the ventral nail plate in systemic sclerosis
pterygium inversum unguis
what criteria is needed for the diagnosis of systemic sclerosis
1 major or 2 minor
major: proximal scleroderma to MCP joints
minor: sclerodactyly, digital pitting scars of fingertips, b/l basilar pulmonary fibrosis
CREST syndrome antibodies
anti-centromere
CREST is a form of localized systemic sclerosis. what are the features
Calcinosis, Raynauds, Esophageal dysmotility, Sclerodactyly, Telangiectasias
what is Parry Romberg syndrome
progressive hemifacial atrophy
epilepsy
exopthalmos
alopecia
antibodies in generalized morphea
anti-histone and ant-ssDNA
anti-histone antibody diseases
generalized morphea
drug induced SLE
anti-p155/140 is associated with
cancer in DM
see palmar involvement
anti-CADM5 is associated with
interstitial lung disease in Japanese DM
anti-Mi2 is associated with
good prognosis in DM
shawl sign, cuticular disease
T/F: there is an increased risk of malignancy associated with childhood DM
false
increased incidence of calcinosis cutis
the 2 types of childhood DM
Brunsting = slow course, progressive weakness, calcinosis. steroid responsive. Banker = rapid onset, vasculitis of muscles and GI tract. steroid unresponsive, high mortality
most common malignancies seen in DM (10-50% of cases)
women: ovarian
men: colon
asians: nasopharyngeal
anti-Jo and other anti-synthetase antibodies (anti-PL7, anti-PL12, anti-DJ, anti-EJ) are associated with
interstitial lung ds in DM
anti-Ku antibody is seen in what overlap syndrome
scleroderma and DM
mechanic’s hands are associated with
anti-synthetase antibodies in DM.
indicate pulmonary involvement
labs to check in DM
aldolase, CK muscle biopsy (tricep, quad) and MRI PFTs and CT scan if symptomatic
most important clinical feature of DM
poikiloderma
T/F: bullous DM is a good prognostic feature
false
indicates severe myopathy and lung ds
in DM, skin findings usually precede muscle findings by how long
2-3 months
age distribution seen in DM
bimodal
children (10-15 yrs) and adults (40-65 yrs)
