Scleroderma Flashcards
What is Scleroderma?
Hardening or sclerosis of skin
Sclero defined as what?
Disorder of connective tissue characterized by induration and thickening of the skin
Where are abnormalities in Sclero?
Abnormalities of microvasculature and large vessels
Fibrotic changes in muscles, joints, and viscera (esophagus, GI tract, heart, lungs, and kidney)
Cause of Sclero?
Unknown, thought to be autoimmune
Sclero associated with what autoimmune disease?
Polymyositis and MCTD
About 20-30% of Sclero patients have anti what cytotoxic factors?
Anti endothelial
Microchimerism theory?
Cells from mother are transported across the placenta, persist and cause a graft versus host type disease
What is important in pathogenesis of sclero?
Potent vasoconstrictors
What is primary target tissue of Sclero?
Vascular endothelium
How does vascular endothelium present?
Intimal sclerosis, fibrinoid change, and necrosis, decrease in vessel diameter, local ischemia, and fibrosis
What effects are seen in the skin?
Atrophy of skin appendages and loss of hair follicle and sweat gland
What happens to epidermis?
Thinned
What accumulates in the skin?
Excess collagen bundles and other extracellular matrix constituents
Who typically presents with Scleroderma?
Female aged 30-40 with pallor/cyanosis of fingers and toes on cold exposure or emotional upset
What will a patient be complaining about in Sclero?
Puffy face and hands in the morning
What blood vessel sign can be seen in Sclero?
Telangiectasias
When does the edematous phase of the skin occur?
Early
What happens during the indurated phase?
Skin is stiff and bound to underlying structures. Alopecia and hyper pigmentation/depigmentation occur
What happens during Atrophic phase?
Fingers small and pointed, mouth smaller
What happens during late phase?
Skin softens and become pliable
Danger of Raynaud’s?
Infarcted fingers
How common is esophageal dysmotility in patients?
High, 90%
Involvment of duodenum and jejunum leads to what in sclero?
Malabsorption
What cirrhosis frequently overlaps with sclero?
Biliary cirrhosis
What pulmonary problem can occur with sclero?
Diffuse interstitial fibrosis with decreased oxygenation
Patients will have dyspnea with what?
Exertion
What pulmonary finding can you hear on exam?
Inspiratory crackles that can lead to pulmonary hypertension and death
What does chest x-ray show?
Increased vascular markings at bases
What does the first type of renal lesion do?
Causes lesions that reduce flow of renal artery and impinges glomerular function
Second type of renal problem?
Scleromderma renal crisis where sudden collapse of glomerular filtration leads to severe volume depletion
What fixes Sclero renal crisis?
Angiotensin converting enzyme
Is Sclero renal crisis inflammatory? Does it have immune complexes?
No and no
Markers for Sclero renal crisis?
Proteinurea
Hypertension
Azotemia
Microangiopathic hemolytic anemia
Problems in the heart?
Pericardial effusion, cor pulmonale secondary to pulmonary hypertension ad microvascular ischemia
What is linear sclero?
Band of sclerotic induration and hyper pigmentation occurring on single extremity or face
What is morphea?
Patch or patches of sclero skin change
What is limited sclero formerly CREST?
More limited involvement than PSS
What is limited sclero associated with?
Anticentromere pattern ANA on HEP 2 substrate
Limited sclero has more what association?
Lung over renal
Scleroderma highly associated with what disease?
Diabetes
What is eosinophilic fasciitis?
Inflammation and thickening of the deep fascia
What is Eosinophilia-Myalgia syndrome
Abrupt onset of myalgia, fatigue and periphery eosinophilia associated with ingestion of L-tryptophan dietary supplement
What is Toxic oil syndrome of Spian/
Epidemic of acute pneumonitis, scleroderma like skin thickening, neromyopathy, and sicca syndrome that affected thousands in Spain in 1982 due to rapeseed oil
Study that shows positive in 90% of cases?
ANA positive
Hep-2 substrate present in 50-96% of patients?
Anticentromere Antibody
Watch what for impending renal failure?
CBC, urine, creatinine
Look for what showing pulmonary disease?
Chest x-ray, DLCO
Treatment?
No drug, hand protection, moisturizing, keep warm, no smoking
Treatment for pulmonary?
Bosentan (Tracleer)
Treatment for Renal?
ACE inhibitors
Drug used without evidence for many years without proof it works?
D-Penicillamine
