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Systemic sclerosis (CREST Syndrome (Calcinosis (Calcium deposits in…

- - - - Three phases:
        Edematous phase
        Indurative phase - firm and thickened skin
        Atrophic phase
      - begin distally in the extremities with proximal advancement
        
        dryness of skin, hair loss and damage to oil and sweat glands
        
        “salt and pepper appearance” -hypopigmented areas with loss of pigment around hair follicles
    - - Pinched nose, Loss of wrinkles
        Loss of facial expression, Teleangiectasias
        Thinning of lips, Microstomia
        Wrinkles around the mouth perpendicular to lips
    - - ILD (interstitial lung disease- mech.fibrosis) and PAH (pulmonary arterial hypertension- mech.vasulopathy)
        
        Leading cause of death
        
        Symptoms of ILD -exertional dyspnea and dry cough
        
        Symptoms of PAH –exertional dyspnea and fatigue
        
        Diffuse SSc carries a risk of ILD
        
        Limited SSc leads to PAH
      - Investigation of ILD:
        PFTs-pulmonary function tests,
        HRCT-thoracic high-resolution computed tomohraphy, ECHO
        
        Investigations of PAH:
        PFTs, ECHO,
        RHC-Right heart catheterization is absolutely requires for PAH,
        Elevated BNP-B type natriuretic peptyde
    - - Esophagus dysmotility
        
        GERD - gastroesophageal reflux disease
        
        Gastroparesis
        
        Small bowel bacteria overgrowth
        
        Rectal incontinence
      - Dyspepsia to impaired motility of the stomach
        
        Cramping and diarrhea to small bowel involvement and bacterial overgrowth
        
        Pseudo-obstruction is common
        
        Constipation - colon involvement
        
        Malabsorption and weight loss are seen
        
        Bleeding due to erosive esophagitis
        
        Hepatitic is uncommon, although patients with limited sclerodermia may develop the complication of primary biliary cirrhosis
    - - Pain, swelling and stiffness of the joints.
        
        Infammation of tendon sheats
        
        Carpel tunnel syndrome
        
        Acrosclerosis with osteolysis –resorption of bone
    - - patchy fibrosis of the myocardium.
        
        A Raynaud's - like reactivity.
        
        pericardial fluid: often asymptomatic
        
        bradyarrhythmias are less commonly.
    - - Scleroderma Renal crisis:
        Malignant hypertension, Hypertensive encephalopathy, Severe headache, Retinopathy, Seizures, Hematuria/proteinuria/oliguria ( rapid deteriorationof renal function), LVF and pericardial effusion
        
        Mechanism of renal crisis: activation of renin angiotensin system.
        
        Laboratory test in scleroderma renal crisis
        elevated renal function tests, trombocytopenia, microangiopathic hemolysis ( schistocytes-50%), elevated renin levels, ADAMTS 13 -normal.
        
        reatment in scleroderma renal crisis
        ACE inhibitors
        dialysis up to 75% will require
    - - Sicca syndrome (dry eyes and dry mouth) are common in patients with scleroderma but are usually not as severe as in patients with primary Sjögren’s syndrome.
        
        Erectile dysfunction
        
        Biliary cirrhosis
        
        Depression
        
        Hypothyroidism