Please enable JavaScript.
Coggle requires JavaScript to display documents.
Scleroderma (Raynaud's (Sequelae (Ischemia (Gangrene/digit loss, Bone…
Scleroderma
Raynaud's
-
-
-
-
Nailfold capillary microscopy + (+)scleroderma-specific auto-ab is best predictor that Raynaud's is secondary.
-
-
Renal Crisis
-
-
-
-
Symptoms
-
Renal insufficiency/failure (active sediment, proteinuria)
-
-
End-organ manifestations
Treatment
ILD: mycophenolate/cyclophosphamide. Pred? Lung tx. #
Pulm Arterial HTN: monitor PFTs from drop in DLCO. Drugs = bosentan, sildenafil, epoprostenol, treprostinil. #
GI
-
Esophagus
-
Dysmotility, lower 2/3: Lower esophageal sphincter, Stricture, Telangiectasia
-
-
Lungs
-
-
ILD (80% postmortem exam): NSIP, UIP, Bibasilar pulm fibrosis
-
-
-
-
-
-
Cardiac
-
-
-
Pericardial: Pericardial effusion (can be large, exudative, risky for renal disease/crisis). Pericarditis is rare.
-
Epidemiology
-
-
-
Less common in children, elderly
Possible environmental link: vinyl chloride, silica
Eosinophilic Fasciitis
-
Affects
Skin thickening, spares hands/fingers/face. Groove sign.
No systemic symps, Raynaud's, nailfold capillary changes.
-
-
Treatment:
Prednizone (high dose, be sure it's not Scleroderma or else might precip renal crisis)
-
-
Localized scleroderma
-
Circumscribed plaques of skin on trunk, face, extremities. Waxy/shiny. Thickened. May be diffuse.
-
-
-
What is scleroderma?
Abnormal collagen deposition, tissue fibrosis, and microvascular change involving almost all organ systems.
-
-
-
-
Systemic Sclerosis
-
Diffuse (dcSSc)
-
Clinical Features
-
All over, including face, chest, abdomen, proximal skin
-
-
-