Systemic Sclerosus Flashcards
What is systemic sclerosus?
A autoimmune connective tissue disease characterised that results in fibrosus of the skin and internal organs
How is systemic sclerosus clasified?
Limted systemic sclerosus
Diffuse systemic sclerosus
Systemic sclerosus sine scleroderma
What are the key differences between the two major subtyes of systemic sclerosus?
Limited (key antibody is anti-centromere Ab)
- cutanoues sx limited to the distal extremities and face
- tend to develop internmal organ involvment later and have a better prognosis
- long preceeding history of raynauds phenomenon
- Pulmonary artery hypertension more common than ILD
Diffuse Key antibodies: anti-topoisomerase I antibody (scl-70) and anti RNA polymerase III
- distal and proximal extremities, trunk and face.
- rapidly progressive, more widespread cutanoues sclerosus
- sudden onset raynauds
- > 90% have internal organ involvement
- ILD > PAH
- increased risk of breast can (with anti-RNA polymerase III)
Which subtype of scleroderma has a malignancy risk?
DIffuse
Anti-RNA polymerase III antibody
Most common malignancy = breast cancer
List the cutaneous features of systemic sclerosus?
Raynauds (>90%)
Puffyness / sweling of the fingers
Skin thickening of both hands, extending beyond the MCP joints
Sclerodactyl
Digital pitting
Digital ulceration
Calcinosis cutis
Nail fold telangectasisa, with dilated capillary loops and drop out
Matt telangectasia (face and palms)
Youthful facies
Beaked nose
Microstomia (<3 fingers in the mouth)
Xerosis and sicca syndrome
Dyspigmentation- (salt and pepper sign)
List the extra-cutaneous features of SSc?
Esophageal dysmotility
GORD
Pulmonary arterial hypertension (limited > diffuse)
Interstitial lung disease (diffuse > limited)
Scleroderm renal crisis
- oligourea
- hypertension
Cardiomyopathy
Heart Failure
Arthralgia
Joint contractures
tendon friction rubs
myopathy
What is the diagnostic criteria for SSc?
ACR / EULAR criteria
Need >= to 9 points
Major criteria
1. skin thickinging of the fingers, extending proximal to the MCP joint in both hands - 9 points
Minor
2. Skin thickening of the fingers
- puffy fingers (2)
- sclerodactyly (4)
3. Figertip lesions
- pitting (3)
- ulceration (2)
4.Telangectasias (2)
5. raynauds (3)
6. Abnormal nail folds (2)
7.Lung disease (either PAH or ILD) (2)
8. Antibodies (3)
- anti centromere, anti topoisomerase I or RNA polymerase III
What is systemic sclerosis sine scleroderma?
Systemic involvement + Raynauds + abnormal capillaries WITHOUT skin sclerosus
How do you treat a scleroderma renal invovlement
A medical emergency
Contact on call renal team at local hosptial and admit to hosptial
Guided by renal
- ACE inhibitors are the mainstay of therapy
What is the leading cause of mortality for scleroderma?
Lung disease
(prev renal, but now well treated)
Patients with SSc and anti RNA polymerase III antibodies are more likely to have what features?
Rapid progressive skin disease
Renal involvement
Increased malginancy - Breast Cancer
DDx for SSc?
Chronic GVHD
Morphea
Extra-genital Lichen sclerosus
Eosinphillic fascitiis
Other AICTD - Lupus, Mixed AICTD
Mucinoses
- sclerodema
- Scleromyxedema
Paraneoplasic
- POEMS syndrome
- Amyloidosis
- Carcinoid syndrome
Diabetic cheroarthropathy
Toxin mediated sclerosus
- Nephrogenic systemic fiborsus
- toxic oil syndorme
- Silicosis
- eosinophillia-myalgia syndrome
Genetic
- werner syndrome
- stiff skin syndorme
Which subtype of scleroderma is most common?
Localised - 2/3 of cases
Survival rates of systemic sclerosus?
Limited
- 88% at 10 years
- 61% at 20 years
Diffuse:
- 72% at 10 years
- 40% at 20 years
Who does systemic sclerosus affect?
Age - rare in children, young - middle aged adults
Female predominance (4 - 5 x)
No ethnicity predomninance
Other AICTD / AI disease
Poor prognoatic factors in SSc
○ Male sex
○ Older age at diagnosis
○ African American
○ Internal organ involvement (esp pulmonary)
○ Cutaneous sclerosis affecting the trunk
○ Elevated ESR
Common AI associations with SSc?
Thyroid
Srojrens
DM
primary bilary cirrhosis
SLE
RA
HLA associations with SSc?
DPB1, DRB1 and DQB1
Pathophysiology of SSc
Vasculopathy
Immune mediated inflammation
Fibrosis
Investigations for SSc?
FBC
UEC
LFTs
ANA
ENA
dsDNA
C3 / C4
Nail fold dermoscopy / capillaroscopy
LFTs inc DCLO
CXR + /- HRCT
Echo
Blood pressure
Urine microscopy
Urine Prt : Cr ratio
Renal USS
Skin biopsy for histology and DIF
Nutritional screen (common complication)
Skin directed therapy for SSc?
Physiotherapy / manual lymphatic drainage
Emolliants
Topical antipuritic agents
Topical corticosteriods
Topical calcinurin inhibitors
Vitamin D analgouges
Phototherapy - UVA1, PUVA
Extracorporeal photophoresis
Immunosuppressant therpy
- MTX (NOT IF ILD)
- MMF
- IVIG
- Rituximab
- Tyrosine kinase inhibiotrs (Imatinib)
- Cyclophosphamide
- Tocilizumab (IL-6 receptor inhibiotr)
Nb: AZA not effective as a monotherapy
Managemetn of Calcinosis?
Surgery, CO2 laser
Sodium thiosulphate
Calcium Channel Blockers
Minocycline
Cochicine / Rituximab
Anakinra
Treatment of Telangectasia is SSc?
PDL
IPL
Treatment of Raynauds in SSc?
Management of digital ulceration in SSc?
What history questions do you need to ask men with systmic sclerosus?
About erectile dysfunction (affects 70 - 80 % of men)
Overview of management of SSc?
