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CREST Syndrome also known as the limited cutaneous form of systemic sclerosis (lcSSc)

* C – Calcinosis cutis
* R – Raynaud’s phenomenon
* E – Esophageal involvement
* S – Sclerodactyly (tightened shiny skin of fingers)
* T – Telangiectasia

Systemic sclerosis points in favour are Dysphagia for solids
Raynauds phenomenon.. pain & cyanosis of fingers in winter
Fibrosis and atrophy of skin manifested as thick shiny skin in fingers
Radical furrowing around the mouth is characteristic in the later stage
progressive breathlessness

Diagnosis- ACR criteria

Major features centrally located skin sclerosis that affects the arms, face, and neck.
Minor features
sclerodactyly, erosions, atrophy of the fingertips, and bilateral lung fibrosis.

Cutaneous Systemic sclerosis is diagnosed when a patient has 1 major and 2 minor criteria.

Evaluation Complete blood count with ESR.. can have elevated ESR, thrombocytopenia
CPK levels elevated if the muscle is involved
RFT increased in renal involvement
CRP increased
ANA profile.. anti scl 70 & Anticentromere antibodies are elevated anti-histone antibodies are non-specific
Spirometry and DLCO to assess lung involvement
HRCT thorax..reveal thickening of the alveolar walls and intestinal tissue and honeycomb-appearing lungs in ILD.
oesophagal manometry
ECG and Echo to assess cardiac involvement.

Treatment
1.Antifibrotic agents
Corticosteroids (in inflammatory myositis, pericarditis, refractory arthritis, alveolitis)
Methotrexate,Chlorambucil,Mycophenolate mofetil,Cyclosporine,tacrolimus
Thalidomide, Low-dose intravenous cyclophosphamide, Statins
2.Raynaud’s phenomenon Non-pharmacologic measures
avoiding exposure to cold temperature and wearing layers of warm, loose-fitting clothing, socks and gloves, smoking cessation if present pharmacological
calcium channel blockers, prostanoids, tadalafil and bosentan.
3.kidney involvement-ACE or angiotensin II inhibitor
4.GI tract involvement -proton pump inhibitors and H2 blockers
5.lung involvement-calcium channel blockers, prostaglandins and cyclophosphamide
Chest physiotherapy & breathing exercises
6.Inflammatory myositis- high doses of corticosteroids (eg, prednisolone with a starting dose of 1 mg/kg/d)
7. cardiac treated as per complication seen

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