Scleroderma
Sclerodermoid
disorders
disorders
Morphea and LSC
Other Rheum
Auto-inflammatory
Antibodies
100
leading cause of death in patients with systemic sclerosis
What is pulmonary disease (particularly interstitial lung disease)?
100
Name the condition and likely trigger
What is scleral plaque in nephrogenic systemic fibrosis?
Triggered by gadolinium contrast
100
most common subtype of morphea seen in adults.
What is plaque morphea?
100
Relapsing polychondritis has association with ___ malignancy
heme (aka myelodysplastic syndrome)
100
This high spiking fever pattern, accompanied by an evanescent salmon-colored rash, defines this childhood rheumatologic disorder.
What is systemic-onset juvenile idiopathic arthritis (Still disease)?
100
High titer antibodies to this protein are typical of MCTD
anti-U1 ribonucleoprotein (U1-RNP)
200
Major cytokines responsible for fibrosis in scleroderma.
TGF-β (>> IL-4, IL-13)
200
What is eosinophilic fasciitis (Shulman syndrome)?
-dry riverbed/groove sign; peripheral eosinophilia
-rapid onset, painful induration; triggered by strenuous exercise (>>borreliosis/statins)
-Good response to steroids
200
This subtype of morphea may cause limb shortening in children.
What is linear morphea?
200
Rheumatoid arthritis, splenomegaly, and neutropenia comprise this clinical triad.
What is Felty syndrome?
200
This potentially life-threatening complication of systemic-onset JIA is characterized by fever, cytopenias, coagulopathy, and extremely high ferritin levels.
What is macrophage activation syndrome (MAS)?
fever, cytopenias, liver dysfunction, coagulopathy, hypofibrinogenemia, hypertriglyceridemia, and very high serum levels of ferritin (treat w/ high dose steroids)
200
The majority of patients with Sjögren syndrome test positive for these two antibodies.
What are anti-SSA/Ro and anti-SSB/La antibodies?
300
Name this finding
Pterygium inversus unguis (aka ventral pterygium)
adherence of hyponychium to ventral surface of nail plate --> obliteration of distal nail groove
300
Exposure to this contrast agent in patients with renal failure can trigger nephrogenic systemic fibrosis.
What is gadolinium?
300
Name this finding and associated autoantibody
What are En Coupe de Sabre and anti-ssDNA antibody?
300
Tear production is assessed in Sjögren syndrome using this bedside test.
What is the Schirmer test?
300
This inherited autoinflammatory syndrome is characterized by periorbital edema, PAN, myelodysplastic syndrome, arthralgias, polychondritis, & pulmonary infiltrates
VEXAS syndrome
300
These antibodies are classically associated with the limited cutaneous form of systemic sclerosis
What are anti-centromere antibodies?
400
This single clinical presentation alone meets criteria by ACR for systemic sclerosis
What is skin thickening of fingers of both hands extending proximal to MCP joints (=9 points-- need 9 for diagnosis) ?
400
Markedly thickened, hyalinized fascia without inflammation characterizes this congenital disorder of the ECM.
What is stiff skin syndrome?
400
This rare, treatment-resistant morphea variant may lead to squamous cell carcinoma.
What is pansclerotic morphea?
400
Name condition and antibody target
What are relapsing polychondritis and collagen 2?
400
What is first line treatment for JIA/Stills and other autoinflammatory syndromes?
IL-1 antagonists, e.g. anakinra, rilonacept, canakinumab
400
This autoantibody predicts rapidly progressive diffuse disease with renal crisis.
What is Anti-RNA polymerase III?
500
Name the sign
Salt and Pepper sign (aka leukoderma)
500
These are culprit drugs for sclerodermoid reactions
What are Bleomycin, bromocriptine, and D-penicillamine?
500
lichen sclerosus and lipoid proteinosis share this molecular target.
What is ECM-1 (extracellular matrix protein 1)?
500
Which cytokine is central to Still disease and many autoinflammatory syndromes?
(Interleukin-1) via aberrant inflammasome activation
500
This syndrome combines features of Behçet’s disease and relapsing polychondritis.
What is MAGIC syndrome?
500
Most sensitive + specific antibody for sjogren's syndrome
What is anti-fodrin?