Scleroderma Flashcards
Scelroderma in general
All present with thickened, scleortic skin lesions and other manifestations
Patho of SSc
Microangiopathy leading to inflammation and fibrosis of the skin
3 processes - severe fibroproliferative vascular lesions of small arteries and arterioles
Progressive deposition of colalgen in skin and internal organs
Alkterations of HMI and CMI
Patho of scleroderma part 2
Microvasc injury due to endothelial cell duysfunction and cytokine med inflammation
Subendothelial fibrosis and perviascular inflammation
As chronic inflammation progresses, prolif of smooth muscle cells, platelet agg, and IV thrombosis
Fibroblasts are recrutied and ifferentiate into myofibroblasts that overproduce ocllagen and under produce ECM degrading enzymes so get tissue fibrosis
Epidemiology
Present at any ag
e
More in women 30-50
Initial presentation
Limited has skin scleoris on hands, distal forearm, face and neck…uindolent course of years
Diffuse has skin changes on chest, absdomen, upper arms, and shoulders…more rapid
Diffuse SSc
Raynauds and within weeks to mojnths get swelling and pururits of the fingers, hands, distal limbs, and face
Also have alregalfia, fatigue, etc.
Loss of hair and decreased sweating
Flexion contractures of fingers and sitffness of the wrist, elbows, shoulder, hips, kenees, and ankles bc of fibrosis
Other organ involvement
Esophageal dysmotility from loss of peristalsis and incompentence of lower sphincter
Chronic GE reflux
Pulm dz from fibrosis or pulmonary HTN from progressive vascular fibrosis
MSK can be arthralgia or myalgia
Cardiac - pericarditis, myocarditis, arrythmias
Limited SSc manifestations
CREST
Calcinosis cutis Raynaud Esophageal dysmotility Sclerodactyly Telangectasia
Complications
Depends on organs
Pulm arterial HTN can lead to right heart failure
Cardiac has poor prognosis
Myocardial dfibrosis leads to dysfunction
Fibrosis of conduction system
Renal crisis is result of progressive vadculopathy…presents in 1st 5 years of illness with renal fialure and severe HTN
Dx - clinical
Diffuse - think skin induration, raynaud and other organs
Limited - think raynaud, GE reflux, and scleordactyly
Dx labs
CBC, creatinine and AutoABs
ANA, antitopo 1 (anti-SCl-70), anti centromere (ACA) and anti-RNA poly 3
ANA positive in 95%
Anti-scl70 and anti-RNA poly are mre specific to diffuse
ACA is specific for limited
Managment and prognosis
No real therapy
Maybe methotrexate or mycophenolate
Most death from pulm fibrosis, pulm arterial HTN, cardiac arrythrmias or renal criss
