Systemic Sclerosis and Inflammatory Myopathies Flashcards
(41 cards)
What are the two main subsets of scleroderma?
limited cutaneous and diffuse cutaneous
What genes are involved in scleroderma?
HLA-DRB1, STAT4, interferon
What environmental factors are associated with scleroderma?
silica, organic solvents, vinyl chloride
What are the three pathologic pillars of systemic sclerosis?
inflammation (synovitis, myositis), vasculopathy (raynaud’s, abnormal capillaries, ischaemic changes) and interstitial fibrosis (in skin = scleroderma, but also happens in other organs e.g. lung, gut, renal)
What is the difference between diffuse cutaneous and limited cutaenous?
diffuse has a more rapid onset, has earlier internal organ involvement, more constitutional symptoms
in limited cutaneous skin changes are limited to arms and face
Which antibodies are associated with diffuse cutaneous scleroderma?
Scl-70 antibodies, anti-RNA polymerase I, III, ANA with nucleolar pattern
What is CREST syndrome?
limited cutaneous scleroderma: Calcinosis, Raynaud’s, oEsophageal dysmotility, Skin changes, Telangiectasia
What antibodies are associated with limited cutaneous scleroderma?
anti-centromere antibodies
Is pulmonary arterial hypertension more common in diffuse or limited?
equal 10% in both
Is ILD more common in diffuse or limited?
diffuse
Is scleroderma renal crisis more common in diffuse or limited?
diffuse
What organ involvement is associated with anti Scl-70 antibody?
intestitial lung disease
What organ involvement is associated with centromere antibodies?
protection from ILD and renal disease
What organ involvement is associated with anti RNA polymerase III antibodies?
renal, skin, malignancy
What is the leading cause of death in scleroderma?
cardiopulmonary manifestations - pulmonary fibrosis, pulmonary hypertension
What is the treatment for scleroderma?
no overall disease modifying therapy - organ specific treatment
How do you diagnose interstitial lung disease?
high resolution CT shows non specific interstitial pneumonitis
Which patients with ILD should be treated?
patients with early diffuse scleroderma with positive anti-Scl70 or elevated CRP, or based on the grade of disease on HRCT, FVC% or DLCO % less than lower limit of normal or clinically meaningful decline, desaturating on 6MWT, symptoms from ILD
How often should patients get spirometry?
for every 3-4 months for the first 5 years and then yearly
What conservative measures can be used to treat ILD?
treat GORD, quit smoking, vaccinations, supplemental O2
What is first line therapy for ILD?
mycophenolate 3g/day
What are other reasons patients with scleroderma may have declining DLCO other than ILD?
neuromusclar dysfunction (myositis), thoracic restriction (scleroderma), aspiriation (GORD), deconditioning, pulmonary hypertension
How do you diagnose pulmonary hypertension?
with right heart catheter
What screening should be done for scleroderma patients for PAH?
TTE and RFTs OR pro-BNP and RFTs
