1- A disease depends on :
a- Vascular lesions >> endoth. damage >> .. >> obliterative arterial lesions >> ischaemia ...
b-Fibrotic lesions >>and internal organs .. ??
c- Autoimmune mechanism >> may precipitate the vascular lesions or activate the fibrotic mechanism , beside its own harming effect
2-C/P
- Skin ; ( edematous >> fibrotic >> atrophic )
- Non pitting edema >> and then become tight
- Skin tightness >> shiny , atrophy , ulceration
- "Fish mouth " and wrinkles
- " Salt and pepper " areas of hyperpigmentation and hypopigmentation
- Raynaud's phenomenon ( pallor>cyanosis>erythema) 2/3 .. What are the precipitating factors of raynaud's phenomenon ??
- Vasculitis infarction
- Atrophy and resorption
- Sclerodermaomyositis ( sclerosis + DM )
- GIT
- Dysphagia ( lower dysphagia )
- Reflux " due to laxity of the lower eso. sphincter " >> aspiration pneumonia and hoarseness
- Malabsorption $ " due to over bacterial growth "
- Intestinal pseudo-obstruction ..... so , the patient presents with a long duration of constipation followed by a sever diarrhea ..
so doctors prefer to give a prophylactic course of antibiotic every month ..
-IPF >>
- Pulmonary HTN ..
- Myocardial fibrosis >>
- Conduction and contraction defects
- ENT
- Sicca-like $
- Hoarseness of voice due to acid reflux
-Genetalia ( due to fibrosis )
- Erectile dysfunction in males
- Dysparonia in females
- Hypothyrodism ( due to fibrosis of the secretory acini )
- Scleroderma kidney ( also due to fibrosis )
- Malignant HTN
- Renal failure
- Scleroderma renal crisis ^abrupt onset
3- Investigations
- To detect the different affection ... ??
- Anti-Scl 70 ... Specific , but only in 20-30 %
- ANA +ve in 90% & Rh.f +ve in 30 % "sero +ve"
4- Treatment
- No specific satisfactory drug therapy
- symptomatic and life style modification ... ??
- Vasodilators
- ACEI from the start .. to prevent the Renal crisis .. and never stop if only produce cough >> shift to ARBs
- Viagra in winter , .. to females ??!!
- SSRI >> vasodilator + decrease TGF-Beta
- Iloprost " prostacyclin analogues substance "
-high dose CS " > 30 ".. is contra-indicaed as it can precipitate renal crisis ...
one of those erythema marginatum pictures is WRONG. the bulls-eye appearing lesion is erythema MIGRANS and seen with lyme disease. You do not want to make that mistake on diagnosis.
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