Scleroderma

Scleroderma

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

-MSK ^ Non erosive ^

• 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 "

-Mycophenolate : prevents internal organ fibrosis
-high dose CS " > 30 ".. is contra-indicaed as it can precipitate renal crisis ...