Systemic sclerosis, Scleroderma Flashcards
Classification for diagnosing scleroderma
ACR/EULAR criteria again
Skin thickening on fingers of both hands extending beyond the metacarpophalangeal joints - 9 points, sufficient for diagnosis
Fingertip pitting scars - 3
Fingertip ulcers - 2
Telangiectasia - 2
Visible/abnormal Nailfold capillaries - 2
Pulmonary hypertension or interstitial lung disease - 2
Raynauds phenom - 3
Scleroderma related antibodies - 3
anti-centromere
anti-topoisomeraise 1
anti-RNA pol
First signs of scleroderma and diagnosing very early SSc
Puffy fingers - sclerodactyly is the most defining characteristic, and earliest
Raynaud phenomenon - is almost always present in SSc
If it is suspected, preform:
Capillaroscopy
Serology for anti-TOPO1 and anti-Centromere abs
if either one is positive diagnose as very early SSc., and investigate whether it has progressed to early SSc with evidence of systemic/organ sclerosis:
1) Esophageal manometry - a tube inserted into the stomach, that measures the force applied to it by the esophagus and sphincter when you swallow.
2) Chest HRCT for lung evaluation
3) Echocardiography for Restrictive CMP eval
Treatment of Scleroderma and treatment window
Treatment window is only in very early/early stage SSc, Before established skin fibrosis and atrophy has set in.
When it is still in the puffy fingers stage with only mild digital ulcers.
Skin symptoms of scleroderma
Puffy fingers
Raynauds phenom
Ulceration on fingertips
Scarring on fingertips
Subcutaneous calcinosis
Taut, flat face without wrinkles
Telangiectasia, petechia, spider nevi
Name of the skin scoring system for SSc
Rodnan skin score measuring theckening of the skin over the body regions.
What are the two types of scleroderma and their relative incidence
Limited scleroderma - 80%
Diffuse scleroderma - 20%
Characteristics of diffuse scleroderma
Skin on the entire body is involved
Raynauds phenomenon only precedes skin disease by 1 or 2 years, or presents simultaneously
Organ systems are involved, lung, heart, GI, Kidney, present early in course
Nailfold capillaries are dilated and also have dropout, avascular areas
Anti-topo1 is highly specific for diffuse form
Characteristics of limited scleroderma
Only the facial skin and skin on the distal arms and legs are affected.
Raynauds precedes skin changes by many years
GI and pulmonary are the only organs affected, and to a milder degree
Nailfold dilation, no dropouts
Anti-Centromere abs highly specific
Slowly progressive with better prognosis, except in those that develop pulmonary hypertension
Vasculopathy of scleroderma
A proliferative vasculopathey that progresses to an obliterativ e vasculopathy of the arterioles.
Can cause acute renal disease and scleroderma renal crisis.
Increased vasoconstrition
Myo-intimal proliferation and fibrosis of the vessels
Thrombosis increased
Causes rapidly progressive AKI
Malignant hypertension
Treat with ACEIs/ARBs, NOT with corticosteroids.
GI dysfunction of SScs
Esophageal dysmotility
Esophageal reflux
Impaired gastric emptying and gastric paresis
Bacterial overgrwoth, Malabsorption syndrome,
Large colonic diverticuli
Anal sphincter dysfunction
Primary billiary cirrhosis
Main cause of death in scleroderma,
Survival/prognosis of the diffuse and limited cases
End stage Pulmonary fibrosis and its complications
10 year surv. ~50% in diffuse form
Normal life span for most cases of limited form, with the exception of patients that develop pulmonary HTN.
SSc treatment of lung fibrosis
Low, medium, and high intensity treatments, stepped up until remission is induced
Low: Azathiprine, mycophenolate mofetil
Medium: Cyclophosphamide, Rituximab
High: Hematopoietic stem cell transplant
Lung transplantation if pulmonary status indicates it.
SSc treatment of renal disease
manage the hypertension
Mild/moderate hypertension: ACE-Is and close monitoring
Severe htn: ACEIs/ARBs and CCBs,
IV vasodilators, nitroprusside,
