Initial Management of Scleroderma

Introduction to Treatment

The American College of Rheumatology recommends mycophenolate mofetil (MMF) as the first-line treatment for systemic sclerosis with skin involvement, regardless of antibody status, to address both skin thickening and potential interstitial lung disease 1

Pulmonary function tests (PFTs) with DLCO should be performed to detect subclinical interstitial lung disease, which is present in many patients at diagnosis 1
High-resolution CT (HRCT) of the chest is the primary tool to diagnose ILD 1
Echocardiogram should be used to screen for pulmonary arterial hypertension 1
Blood pressure monitoring is essential to establish baseline for scleroderma renal crisis surveillance 1
Modified Rodnan skin score (mRSS) assessment at 17 anatomical sites should be performed, with scores 0-3 at each site for a maximum of 51 points 2

MMF has surpassed cyclophosphamide as the initial treatment of choice and is the most commonly prescribed first-line drug for diffuse cutaneous SSc, with a mean mRSS improvement of -4.90 points 1
MMF addresses both skin thickening and potential ILD progression simultaneously 1
Methotrexate 25 mg per week is an alternative first-line treatment if MMF is not tolerated or if musculoskeletal involvement is predominant, with approximately 5-point improvement in mRSS 1
For early diffuse SSc specifically, methotrexate may be considered for skin manifestations 3

Corticosteroids should be avoided as monotherapy, as they are associated with substantial long-term morbidity in fibrotic lung disease and increase risk of scleroderma renal crisis in early diffuse cutaneous SSc 1
If steroids are used, patients must be carefully monitored for blood pressure and renal function 3

Proton pump inhibitors (PPI) should be used for prevention of gastro-oesophageal reflux, oesophageal ulcers, and strictures 3
Prokinetic drugs should be used for symptomatic motility disturbances (dysphagia, GORD, early satiety, bloating, pseudo-obstruction) 3
ACE inhibitors should be used in treatment of scleroderma renal crisis if it develops 3

PFTs should be performed every 3-6 months to track forced vital capacity (FVC) 1
mRSS should be assessed at each visit to quantify skin improvement (minimal clinically important difference is 3.5-5.3 points) 2
Repeat HRCT should be performed at defined intervals to assess fibrosis progression 1

A high mRSS (>24) at baseline is associated with increased risk of mortality and lower rates of survival without progression 2
High skin scores or moderate scores with progressive ILD are criteria for considering autologous hematopoietic stem cell transplantation in patients with early diffuse cutaneous SSc 2

Steroids should not be used as monotherapy, as they increase scleroderma renal crisis risk 1
Skin disease should not be underestimated, as it is associated with significant physical and psychological morbidity 4
Treatment should not be stopped prematurely, as inadequate treatment duration leads to relapse 4