Scleroderma
OVERVIEW
- Scleroderma = autoimmune mediated widespread collagenous deposition
- limited form: Calcinosis, Raynauds, Esophageal dysfunction, Sclerodacytaly and Telangiectasia (CREST syndrome)
- widespread form: aggressive diffuse cutaneous form
HISTORY
- above
- fatigue
- decreased ROM @ joints
- tough skin
EXAMINATION
- AIRWAY: mouth narrowing and tightening of skin around neck
- CVS: pericarditis, myocardial fibrosis, conduction defects, pulmonary hypertension, malignant hypertension
- RESP: pulmonary fibrosis
- RENAL: renal failure
- GI: GORD
- HANDS: distal ulceration with gangrene, fixed flexion deformity of fingers, Raynauds, ischaemia with pallor of the digits, sclerodactaly, calcinosis
INVESTIGATIONS
- FBC
- U+E
- ECG
- anti-centromere antibodies (80%)
MANAGEMENT
Resuscitation
- ABCDE approach
- possible difficult airway – may need AFOI
- difficult IV access
Specific treatment
- immunosuppression (methotrexate)
- steroids
- care with vasoconstrictors
- risk of ileus, malabsorption, nutritional deficiencies, GORD, stress ulceration
- optimise right ventricular function
Supportive care
- protective lung ventilation
- SpO2 monitoring can be difficult
Critical Care
Compendium
Chris is an Intensivist and ECMO specialist at The Alfred ICU, where he is Deputy Director (Education). He is a Clinical Adjunct Associate Professor at Monash University, the Lead for the Clinician Educator Incubator programme, and a CICM First Part Examiner.
He is an internationally recognised Clinician Educator with a passion for helping clinicians learn and for improving the clinical performance of individuals and collectives. He was one of the founders of the FOAM movement (Free Open-Access Medical education) has been recognised for his contributions to education with awards from ANZICS, ANZAHPE, and ACEM.
His one great achievement is being the father of three amazing children.
On Bluesky, he is @precordialthump.bsky.social and on the site that Elon has screwed up, he is @precordialthump.
| INTENSIVE | RAGE | Resuscitology | SMACC