SLE, SS, SSc Flashcards
What are the GI manifestations of systemic sclerosis?
esophageal dysmotility with markedly diminished peristalsis
No wrinkles is associated with what condition?
systemic sclerosis
What is unique in the arthritis seen in SLE?
they are nonerosive to bone
What 2 medications will help with dry mouth (xerostomia) seen in Sjogren syndrome
- Cevimeline
- Pilocarpine
90% of SLE cases are in what gender?
women
What are the pulmonary manifestations of systemic sclerosis? (3)
- interstitial lung disease (pulmonary fibrosis)
- pulmonary hypertension
- lung cancer
If a patient is having a severe or life threatening SLE flair what is the acute treatment?
- hospital admission for high dose IV steroids
Baby born to a mother with SLE may develop what 2 manifestations?
- self limited cutaneous rash that will get better in time
- heart blocks (any degree)
- if 3rd degree HB need a pacemaker for life*
- are at higher rate for autoimmune diseases later in life*
What 3 non-pharmacological treatment options are recommended for SLE?
- exercise
- smoking cessation
- sunscreen
Which organ systems does lupus NOT involve?
trick question - it can involve any organ
Patients with Sjogren Syndrome are at high risk for what 5 complications?
- dental caries
- oral candidiasis
- bacterial sialadenitis
- corneal damage
- vaginal dryness and dyspareunia
What is the number 1 symptom in SLE?
profound fatigue
How is drug-induced SLE diagnosis made?
- positive anti-histone antibody
What is necessary to make the diagnosis of Sjogren Syndrome?
- Positive Schirmer test OR imaging with glandular abnormalities
- salivary gland biopsy OR Anti-Ro (SSA) AND/OR Anti-La (SSB) antibodies
What other antibodies, besides ANA, are associated with SLE? (3)
- Anti-DS DNA
- Anti-Sm (Smith)
- Antiphospholipid antibodies
What are the 5 signs of CREST syndrome associated with limited cutaneous SSc?
- calcinosis
- Raynaud phenomenon
- esophageal dysmotility
- sclerodactyly
- telangiectasias
The production of a high number of _________ antibodies are associated with SLE
antinuclear antibodies (ANA)
ANA is a very _______ antibody. It is good to rule OUT SLE.
sensitive
This is a chronic inflammatory autoimmune disease that can affect essentially any organ of the body.
Systemic Lupus Erythematosus (SLE)
What test do we do to measure tear production when working-up Sjogren Syndrome?
Schirmer Test
- normal = 15-25mm
- SS = <10mm
What life saving medication should be administered in a patient with scleroderma renal crisis in systemic sclerosis?
ACE inhibitors
This is a chronic systemic autoimmune disorder associated with collagen deposition in the dermis, blood, and internal organs.
systemic sclerosis
What are the renal manifestations of systemic sclerosis?
scleroderma renal crisis = a deadly hypertensive crisis with BP >200/100
What are the 11 criteria for SLE?
- malar rash
- discoid rash
- oral ulcers
- photosensitivity
- arthritis
- serositis (e.g. pleuritis/pericarditis)
- renal disorder (lupus nephritis)
- neurologic disorder (e.g. seizures, psychosis)
- hematologic disorder (e.g. anemia or any -penias)
- ANA positive
- immunologic disorder
Who primarily gets Sjogren Syndrome?
women in 4th to 6th decade of life
Patients with SLE have mortality rates ranging from __ to __ times higher than that of the general population
2-5 times higher
What is the treatment for Sjogren Syndrome? (3 part)
- routine dental care d/t xerostomia
- routine eye exams d/t xerophthalmia
- if systemic disease: steroids, DMARDs, biologics
What DMARD is the first line treatment for SLE?
hydroxychloroquine
What is the classic sequence of color changes in Raynaud’s phenomenon?
white, blue, red
Anti-Sm (Smith) antibodies are predictor of what organ disease secondary to SLE? (2)
- CNS
- renal
The 1997 ACR Criteria requires ___ / 11 criteria serially or simultaneously to diagnose SLE
4/11
What ethnicity tend to have earlier-onset disease and more severe disease phenotypes for systemic sclerosis?
african americans
A patient presents with joint pain, rash, pleuritic chest pain, and positive ANA at a low dilution (weak positive). On review of medications, she has been taking minocycline for the past year for acne. Which antibody is most likely to be positive?
anti-histone
What antibody is associated with limited cutaneous systemic sclerosis (aka CREST syndrome)?
anti-centromere antibody
What is the natural history of the skin manifestations associated with systemic sclerosis?
- starts off as a “puffy” phase that progresses to a “tight” phase
- starting in the hands/feet and progressing centrally
Neonatal lupus is due to 2 antibodies in the mother crossing the placenta.
- anti-Ro (Anti-SSA)
- Anti-La (Anti-SSB)
What is unique in the oral ulcers seen in SLE?
they are painless ulcers
What antibody is associated with diffuse cutaneous systemic sclerosis?
anti-ScL 70 (anti-topoisomerase I antibody)
What medication can help with dry eyes (xerophthalmia) seen in Sjogren syndrome?
cyclosporin drops (restasis)
What are the 3 hallmark symptoms of Sjogren Syndrome?
- xerophthalmia
- xerostomia
- enlarged salivary glands