Limited Cutaneous Systemic Sclerosis

Limited cutaneous systemic sclerosis (lcSSc) is a subset of systemic sclerosis with skin thickening distal to elbows and knees (and face), long Raynaud history, and anticentromere antibodies.

• Comprises about 60% of systemic sclerosis; onset 40 to 60 years; women more than men (4:1).

• Anticentromere antibodies in 50 to 60%; anti-Th/To, anti-PM/Scl less common.

• Prominent vascular disease: Raynaud, digital ulcers, telangiectasia, pulmonary arterial hypertension (10%), calcinosis.

• Gastrointestinal involvement universal (oesophageal dysmotility, GERD, GAVE, SIBO).

• Slow progression, 10 year survival about 70 to 80%; pulmonary hypertension and interstitial lung disease reduce survival.

• Also presenting as CREST (calcinosis, Raynaud, oesophageal dysmotility, sclerodactyly, telangiectasia).

• Raynaud phenomenon preceding skin changes for years, puffy fingers, sclerodactyly, digital pitting scars, ulcers, gangrene.

• Calcinosis cutis, telangiectasia (face, hands, mucosa), nail fold capillary changes.

• Dysphagia, heartburn, early satiety, bloating, malabsorption; dyspnoea and fatigue (PAH, ILD).

• Scleroderma renal crisis is rare (more in diffuse disease).

• ACR/EULAR 2013 classification (skin thickening of fingers, fingertip lesions, telangiectasia, abnormal capillaroscopy, interstitial lung disease or PAH, Raynaud, antibodies; score 9 or more).

• Antinuclear antibodies, anticentromere, anti-topoisomerase I, anti-RNA polymerase III, anti-U3RNP.

• Nailfold capillaroscopy (dilated loops, drop-out).

• Annual echocardiography, pulmonary function tests with DLCO (DLCO falling out of proportion to FVC suggests PAH), NT-proBNP; right heart catheterisation if suspected; HRCT.

• Upper endoscopy for Barrett oesophagus/GAVE, oesophageal manometry, breath test; blood pressure and creatinine.

• Raynaud: avoid cold and smoking, nifedipine ER 30 to 90 mg daily or amlodipine 5 to 10 mg, add sildenafil 20 mg three times daily or tadalafil 20 to 40 mg, topical nitrates, intravenous iloprost 0.5 to 2 ng/kg/min for 6 hours daily for 3 to 5 days for critical ischaemia.

• Digital ulcers: bosentan 62.5 to 125 mg twice daily (prevents new ulcers), sildenafil; antibiotics; surgical debridement; digital sympathectomy.

• PAH: ambrisentan 5 to 10 mg, macitentan 10 mg, tadalafil 40 mg, riociguat, selexipag, treprostinil with initial combination therapy (see pulmonary hypertension).

• GERD/dysmotility: omeprazole 20 to 40 mg twice daily, metoclopramide 10 mg before meals, domperidone, erythromycin; SIBO rifaximin 550 mg twice daily; GAVE ablation by argon plasma coagulation.

• ILD: mycophenolate 1.5 to 3 g/day, tocilizumab, nintedanib 150 mg twice daily, rituximab.

• Skin: methotrexate 15 to 25 mg weekly in early inflammatory disease; calcinosis: diltiazem, colchicine, sodium thiosulfate, surgical excision.

• Autologous stem cell transplantation for severe early progressive disease.

• Annual screening (echocardiography, PFTs) for PAH and ILD.