Scleroderma Flashcards
Scleroderma aka…
Systemic Sclerosis
What are the two most distinguishing features in Scleroderma
Raynauds and ANA abs
What is the most common cause of death for a pt with scleroderma
Cardiac (30%)
Lung (25%)
What separates limited vs diffuse scleroderma
Limited (65%)
-Skin thickening confined to extremities, distal to elbows/knees
-Face involvement
Does NOT mean they lack internal organ involvement
Diffuse (30%)
- Skin thickening extending to the proximal portion of the extremities
- Trunk involvement
If a person has diffuse scleroderma
When would we suspect onset of Raynauds
Close to the Dx of scleroderma
What systemic manifestations are more common in diffuse Scleroderma compared to limited
ILD, often severe
Myocardial Dysfunction
Pulm HTN (group 2 and 3)
Renal crisis
More severe GI Dysmotility/ Gut Failure
What are the two Abs found in diffuse Sclerodrema
Antitopoisomerase (Scl-70), anti-RNA polymerase III
If a pt has limited scelroderma when would we suspect onset of Raynauds
Onset several years before scleroderma diagnosis
What type of PULM HTN is seen in Limited vs Diffuse Scleroderma
Limited: Group 1
Diffuse: Group 2 and 3
What are the Abs seen in limited scleroderma
Anticentromere, anti-Pm-Scl, anti-Th/To
What is CREST syndrome
Synonym for limited scleroderma
Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, & Telangiectasis
What is a sign of early scleroderma manifestation
Diffuse puffiness
What is the common Tx for Raynauds
Cold avoidance and CCB
What is the approach for a pt over the age of 30 with new onset Raynauds
Patients >age 30 with new onset Raynaud’s should have ANA and nailfold capillary examination
Esp if have severe painful episodes of vasospasm, signs of digital ischemia or tissue damage, or systemic signs/sx of secondary dx
How does scleroderma effect the heart
Ischemia-reperfusion injury secondary to small arterial disease of the myocardium leads to contraction band necrosis and tissue fibrosis
- Arrhythmias
- Cardiomyopathy
- Overt symptoms of heart failure
Pericardial effusion = poor prognosis
How does scleroderma effect teh GI systems
Small and large brown peristalsis
Intermittent Constipation and Diarrhea
(Mimics IBS)
Can have bacterial overgrowth and malnutrition
May even lead to bowel rupture
A scleroderma with an ubrupt onset of HTN
What is the concern
Renal Crisis
More common in Diffuse Type Scleroderma(10-15%)
Abrupt onset of hypertension—malignant hypertension
-MOST DREADED complication of systemic sclerosis
10% present without hypertension and have poor outcome
Elevated creatinine, proteinuria, hematuria
Onset usually within 3-5 years of disease onset
TREAT with ACEI: Captopril
What makes somone high risk of developing renal crisis in Scleroderma
Early stages of diffuse scleroderma
Corticosteroid use
Pts who produce antibodies to RNA-polymerase III
What is the risk with w pregnant pt and scleroderma
increased risk for pre-term delivery, small for gestational age newborns, and possibly hypertension
Pulmonary fibrosis is more common in limited or diffuse scleroderma
Diffuse
What is the Ab specific for Diffuse Sclerodema
Antitopoisomerase I antibodies (Anti-SCL 70)
What is the Ab specific to limited scleroderma
Anticentromere AB
How often should scleroderma pts get baseline PFTS
Baseline and every 4-12 months depending on stage of disease and pt symptoms
What are the two suggestive factors for pulm HTN on PFTs for Scleroderma
Isolated reduction in Diffusing Capacity of Lung for Carbon Monoxide (DLCO)
OR
A reduction out of proportion to the degree of decline in FVC
What are the findings in the nail beds of scleroderma pts
Nailfold capillary dropout and dilated capillary loops
What is the treatment approach for skin conditions of scleroderma
Both mycophenolate mofetil (MMF) and cyclophosphamide (CYC) have shown evidence of benefit in the management of active scleroderma skin disease.
mycophenolate mofetil (MMF) is preferred FIRST due to milder side-effect profile!
What medication should be avoided in Sclerodemra
Steroids
What CCB should be used for Raynauds
Nifedipine
Most studied
DO NOT use diltiazem or verapamil (less selective for vascular smooth muscle)
What two medications can be used to treat delayed gastric emptying
Metoclopramide and erthyromycin
What is the role of octreotide in scleroderma
Used to treat severe GI Dysmotility
What is the treatment for bacterial overgrowth
Rotating ABX : tetracycline and metronidazole
Systemic therapy for MSK s/s in scelroderma
Overlapping inflammatory features may benefit from systemic therapy
- Methotrexate
- Azathioprine
- IVIG
- Rituximab
What are the Rx for MGMT of ILD
Both mycophenolate mofetil (MMF) and cyclophosphamide (CYC) have shown evidence of benefit in the management of ILD
Lung transplant in severe disease
