Scleroderma (Systemic Sclerosis)/CREST Flashcards
(19 cards)
What does CREST syndrome stand for?
Calcinosis
Raynaud
Esophageal Dysmotility
Sclerodactylyl
Telangiectasia
What can precipitate Raynaud syndrome?
Cold and emotional stress
What can Raynaud syndrome progress to?
Ulceration and gangrene
What are common skin manifestations?
fibrosis of the hands, face, neck and extremities
Telangiectasia and abnormalities of pigmentation
What are common GI manifestations?
esophageal dysmotility with GERD, large-mouthed diverticula of small and large bowel, diarrhea
What are common Renal manifestation?
Sudden Hypertensive crisis
What are common Pulmonary manifestation?
Fibrosis leading to restrictive lung disease with pulmonary hypertension
What are common Cardiac Manifestations?
Myocardial Fibrosis, Pericarditis and Heart block
RV hypertrophy due to pulmonary hypertension
How does scleroderma lead to diarrhea?
Bacterial overgrowth in large diverticula
What ab is most associated with limited scleroderma (CREST)?
Anti-Centromere (+)
What antibody is most associated with Systemic Scleroderma?
Anti-SCL-70 (+)
What is the most accurate test for Scleroderma?
SCL-70: Anti-topoisomerase
What can be given to slow skin disease in Scleroderma?
Methotrexate
What can be given for renal crisis in Scleroderma?
ACE-I even if Cr is elevated
What can be given for Esophageal Dysmotility in Scleroderma?
PPI for GERD
What can be given for Raynaud In Scleroderma?
CCB
What can you give for Pulmonary Fibrosis in Scleroderma?
Cyclophosphamide to improve dyspnea and PFT
What can be given for Pulmonary Hypertension in Scleroderma?
Bosentan Ambrisentan (endothelin Antagonist)
Sildenafil
Prostacyclin analogs: Iloprost, Treprostinil, Epoprostenol
What can be given for Thromboembolic Disease in Scleroderma?
Riociguat: cGMP Stimulator
