5 - Systemic Sclerosis Flashcards
Who gets systemic sclerosis?
Choctaw Indians
Black women >50yrs
Systemic sclerosis is aka?
Scleroderma
Scleroderma has a STRONG association w?
Raynaud’s phenomenon
What is scleroderma?
Heterogeneous rheumatic diseaese characterized by thickening and fibrosis of the skin with accompanying vascular lesions especially in lungs and kidneys
Strongest risk factor for scleroderma?
Family history
Besides fhx what else is linked to sclerosis?
Autoimmunity
CMV
Environmental (silica dust)
RAYNAUDS phenomenon
Types of sclerosis?
Systemic
- limited
- diffuse
Localized
- morphea
- linear
S/S of systemic sclerosis?
Skin thickening (puffy) Carcinosis (PIP, fingertips) Skin tightening Digital flexion contracturs Telangectasis Vascular Cardiac (arrhthmias/pericardial effusions) Pigmentation/depigmentation (vitiligo-like)
Steroids and systemic sclerosis?
Dont help and
Cause renal crisis
Treat with ACEIs
Sclerosis Most frequent symptoms?
- Raynaud phenomenon
- GERD w/ w/o dysmotility
- skin changes
- swollen fingers
- arthralgias
Labs for scleroderma?
ANA (95% sensitive)
Anti-SCL 70 Anticentromer AB (limited scleroderma)
Imaging/tests for systemic slcerosis?
CSR High res CT (GI) EKG Echo (pulm HTN) Stress testing PFTs
Why order PFT (scleroderma)
Baseline + q 4-12 mo
Looking for:
- reduction in DLCO
- reduction OOP to decline in FVC
Youre working up you systemic sclerosis pt and there is a nagging thin in your mind brain, what office test should you run?
Nailfold capillary evaluation
Bedside exam using ophthalmoscope to visualize nailfold capillaries
Normal: thin, linear, uniform
Scleroderma: capillary dropout and dilated capillary loops
What meds will help cure systemic sclerosis?
No drug will alter the natural course of the disease
What is the progression for the skin manifestations?
Reach peak involvement over first 18-24 months then gradually improve w or w/o therapy
Immune system targeted therapy for systemic scleroderma?
Cyclophosphamide - pulmonary sxs
Don’t use
Corticosteroids - RENAL CRISIS
Symptom specific tx with scleroderma?
Lots of info, not a great flash card
Raynauds: CCB
HTN/Renal crisis: ACEI
Intersitital lung disease: cyclophosphamide
Pulmonary HTN: endothelin receptor antagonist, PDE5 inhibitor
GERD: PPI
Delayed gastric emptying: metoclopramide/domperidone
Mild lower GI: loperamide/fiber
Small bowel hypermotility: ocreotide
Bacterial overgrowth: tetracycline/metronidazole
Describe limited scleroderma?
Symmetrical skin thickening of :
- fingers
- distal arms
- legs
- face
- neck
Limited scleroderma onset post raynauds?
Slow progression after onset of raynauds
Limited scleroderma is a later visceral disease but:
Digital ischemia and pulmonary HTN are more prominent
Labs for limited scleroderma?
ANA (80-95%)
SCL-70 Pos (20%)
Anticentromere pos (50%)
- (HIGHLY SPECIFIC)
What is crest syndrome?
A subtype of limited scleroderma
- invovles fingers distal to MCP
WTF does CREST mean?
C: calcinosis cutis R: raynauds E: esophageal dysmotility S: sclerodactyly T: Telangectasias
CREST syndrome labs
- hint its the same as limited scleroderma
ANA (80-95%)
SCL-70 Pos (20%)
Anticentromere pos (50%)
- (HIGHLY SPECIFIC)
What is raynauds syndrome?
Episodic exaggerated vasospasm of the digital arteries in association with exposure to cold temp
Can be primary (idiopathic) or secondary
raynauds is highly associated with?
Scleroderma (90%)
- may precede diagnosis by years
Pts >30 with new onset raynauds?
ANA
Nailfold capillary exam
Other raynauds warning signs (for scleroderma)
- Severe painful episodes of vasospasms
- Signs of digital ischemia
- Tissue damage
- Systemic s/s of 2ndary dx
Tx for raynauds?
Avoid cold
CCB
Diffuse scleroderma affects?
Same as limited
- fingers
- distal arms
- distal legs
- face
- neck
+
- trunk
- proximal aspect of limbs
Diffuse vs limited scleroderma?
Diffuse is worse
It has more significant visceral disease
Limited is more prone to pulmonary HTN but diffuse is more prone to?
Renal crisis
Interstitial lung disease
Pulmonary fibrosis
Other less common problems with diffuse?
Cardiac disease
- pericarditis
- myocardial fibrosis
- heart failure
Labs for diffuse scleroderma?
ANA
SCL-70 pos
Anticentromere NEG