Condition Info - BotMed Application

ConditionName

Scleroderma

ConditionDescription

Symptoms

Calcinosis;
Esophageal dysmotility (Reflux, Dysphagia, Achalasia, GERD);
Sclerodactyly;
Telangiectasia;

Renal: sudden hypertensive crisis;

Lung: fibrosis lead to restrictive lung dz and pulmonary hypertension fibrosis;

Cardiac: myocardial pericarditis, heart block, lung dz --> R. ventricular hypertrophy;

Female, organ fibrosis of skin and internal organs (lung, KI, GI);

Tightness of the skin;
CREST: limited;
Skin involvement below the knees and elbows;

PhysicalFindings

ClinicalTests

Manometry;
R/O Barrett’s esophagus which can lead to cancer;

ANA – not specific;

ESR normal;

SCL-70 (anti-topoisomerase) –specific;

Anticentromere AB – most specific to CREST;

RiskFactors

3 Female : 1 Male;

IsRedFlag

IsNPLEX

Consequence

Pulmonary fibrosis;
Heart failure;
Acute renal failure caused by malignant renal hypertension;

Pulmonary HTN -25-50%;
Interstitial lung dz – 10%;

Secondary Raynaud syndrome;

ProgressionFromCondition

TreatmentOptions

Pulmonary hypertension: sildenafil;

Achalasia: pneumathicndilation, myotomy, Botox injection;
Avoid nicotine, caffeine, decongestions;

PharmOptions

ACE inhibitors for renal crisis;
PPI – GERD;
CCB for Raynaud;

WebLink

https://www.merckmanuals.com/en-ca/professional/musculoskeletal-and-connective-tissue-disorders/autoimmune-rheumatic-disorders/systemic-sclerosis