Ch. 43 Systemic sclerosis Flashcards
(39 cards)
What is the difference between limited cutaneous SS and diffuse cutaneous SS
Limited-up to elbows and knees, face (most often just hands and feet)
Diffuse-trunk, upper arms and legs
Name the 7 main conditions on ddx for sclerodermoid conditions
- Scleroderma
- Generalized morphea
- Scleredema
- Scleromyxedema
- Eosinophilic fasciitis
- Chronic GVHD
- Nephrogenic systemic fibrosis
Drugs:
-Bleomycin exposure
Paraneoplastic: -POEMS -carcinoid -amyloidosis -Paraneoplastic scleroderma-like syndrome Immunologic: -Fibroblastic rheumatism
Metabolic:
- PCT-porphyria
- Diabetic cheiroroarthropathy
Neoplastic:
-Carcinoma en cuirasse
Neurologic:
-reflex sympathetic dystrophy, spinal cord injury
Toxin mediated:
- Toxic oil syndrome
- eosinophilic myalgia (L tryptophan)
- silicosis
Drug induced: bleomycin, taxanes, vinyl chloride, chlorinated hydrocarbons
other: lipodermatosclerosis
Genetics: Stiff skin syndrome ad many others…
Incidence and prevalence systemic sclerosis
20 per 1 million
275 per 1 million
Average age onset scleroderma
35-50
Name 5 RF for worse prognosis systemic sclerosis
African american Male sex Older age ILD/internal organ involvment More diffuse disease (trunk involvement) Elevated ESR
What are 3 pathogenic mechanisms causing systemic sclerosis
- Vascular dysfunction with increased VEGF
- Immune activation with autoantibody production
- Extra-cellular matrix dysregulation: sclerosis characterized by deposition of collagen and other extracellular matrix proteins
What are the diagnostic criteria for systemic sclerosis
Skin thickening proximal to MCPS = automatic dx
Otherwise need 9 points
Skin thickening distal to MCPS or skin puffiness
Finger tip lesions-fingertip scar or ulcers
Nailfold capillary changes
Telengiectasias
Raynauds
PAH or ILD
Autoantibodies
What are the 8 diagnostic criteria for systemic sclerosis
Skin thickening proximal to MCPS = automatic dx
Otherwise need 9 points
Skin thickening distal to MCPS or skin puffiness
Finger tip lesions-fingertip scar or ulcers
Nailfold capillary changes
Telengiectasias
Raynauds
PAH or ILD
Autoantibodies
What 3 antibodies are most specific for scleroderma and what are their clinical correlates
Anti-centromere with CREST/limited and PAH
Anti SCL70 (Topoisomerase I) with diffuse SS and ILD
RNA polymerase III with rapidly progressive diffuse skin disease and renal involvement
What are 5 major differences (beyond skin) between limited and diffuse sclerosis
1) Lungs
- PAH more in limited, ILD more in diffuse
2) Raynauds
- longstanding hx raynauds in limited vs. acute onset in systemic
3) Skin progression
- slowly progressive skin disease in limited, quickly in diffuse (peak in 1-1.5 yrs
4) onset internal organ involvement
- 10-15 yrs limited, most in first 5 yrs in diffuse
5) more kidney/SRC in diffuse
Prognosis worse in diffuse
What are the 4 major internal organs affected in systemic sclerosis
Lungs: PAH, ILD
GI: GERD, esophageal dysmotility
Renal: renal crisis, htn
Cardiac: fibrosis/restrictive pericarditis, CHF from PAH
Leading cause death scleroderma
lung disease
What are 5 features that suggest secondary raynauds
Later onset > age 25 Digital ulcerations Fingertip scars Abnormal nail fold capillaroscopy Sclerodacltyl ANA+
Name 12 physical findings of scleroderma
- Puffy/edematous fingers–>skin thickening and sclerodactlyl
- Raynauds
- Nailfold changes –> alternating dilation with drop out
- Salt and pepper skin (leukoderma with hypopigemntation with preservation follicular pigment)
- Calcinosis cutis-often over joints, extremities
- Telengiectasias-face, lips, palms
- Fingertip pitted scars
- Microstomia and peri-oral wrinkles
- Beaked nose
- Tendon friction rubs
- Flexion contractures
- Dry skin, loss sweating,
- Ulcers-f fingertip and over joints
Name 6 pathology findings scleroderma
- compact or hyalinized collagen
- excessive deposition of collagen
- atrophic eccrine and pilosebaceous glands,
- loss of subcutaneous fat
- sparse lymphocytic infiltrate in the dermis and subcutis
- Trapped adnexal structures
Name 8 treatments for raynauds
- avoid cold, warming packs, stop smoking= 1st line
- calcium channel blockers=2nd line
- endothelia receptor antagonists- bosentan
- PDE-5 inhibitors-sildenafil
- ARBs-losartan
- alpha adrenergic blockers-prazosin
- SSRI-fluoxetine-4th line
- botox
Name 5 treatments for cutaneous ulcers from raynauds
Avoid aggressive debridement, moist adherent dressings
All therapies listed for raynauds
ASA or clopidogrel
Endothelial receptor antagonists- bosentan
IV prostanoid (e.g. epoprostenol)
High dose statins
Name 8 treatments for calcinosis cutis
- excision
- shock wave litho
- sodium thiosulfate-IV or injection or topical
- warfarin
- bisphosphonates
- CCBs-diltiazem
- aluminum hydroxide
- probenecid
Name 4 treatments cutaneous sclerosis
Often not treated unless internal involvement:
- Topical steroids
- Phototherapy
- Methotrexate-if no ILD
- MMF -esp if ILD
Treatment scleroderma renal crisis
ACEi
Name 2 major treatment internal organ involvement scleroderma
MMF or cyclo
What are the clinical features of eosinophilic fascitis
- Hx strenuous physical activity in 30%
- Pain and edema that progresses to fibrosis and psuedo-cellulite appearance, rippling
- Distribution symmetric extremities and usually spares the hands, feet and face
- Groove sign
What is the groove sign
“groove sign” refers to linear depressions where veins appear to be sunken within the indurated skin.
Name 3 laboratory findings in eosinophilic fascitis
- Elevated ESR
- Peripheral eosinophilia
- hypergammaglobulinemia
