Complications
Ulcers and infarcts occur primarily due to ischaemia.
Ulcers over contractures or joints are due to skin breakdown from stretched skin and repetitive micro-trauma.
Usually Hashimoto's variant.
Screening is recommended at least yearly or with suggestive symptoms.
Can occur from excessive pruritus or infected ulcers; treat with wound care, antibiotics, and treatment of Raynaud's phenomenon if applicable.
Signs of wound infection include purulent discharge, fluctuance, or systemic signs, such as fever or rigors. Assessment for osteomyelitis should also be done for infected digital ulcers, using plain film x-rays or MRI (more sensitive).
Double coverage for Staphylococci as well as anaerobes may be required for infected digital ulcers.
If infections are not responsive to first-line therapies, consultation from an infectious disease specialist is recommended.
Evaluation by an experienced surgeon for debridement may be required for an infected wound.
Can occur from infected skin with spread of infection from ulcers.
An uncommon complication of digital ulceration.
Treatment is with antibiotics, debridement if necessary, and treatment of Raynaud's phenomenon.
Hyperbaric oxygen treatment may be used for severe cases.[72]
Complications of unintentional weight loss with diarrhoea are a sign of bacterial overgrowth and malabsorption and should be treated with antibiotics.[36]
Should be suspected in patients with small bowel involvement (typically have abdominal distention, bloating, and constipation from decreased peristalsis), with these newly developed symptoms.
Diagnosis is supported by a positive glucose hydrogen breath test. Test results are not reliable if the patient is on proton-pump inhibitor.
These patients may need alternating antibiotics intermittently.
Gastrointestinal consultation is warranted in these cases.
After antibiotic therapy, a pro-motility agent may be helpful.
Can be auto-amputation from chronic ischaemia or amputation could be done because of osteomyelitis occurring from an infected non-healing ulcer.
Xerostomia is managed symptomatically with frequent fluid intake and frequent dental care.
Muscarinic agonists, such as cevimeline, can be used if symptoms are severe.
Xerophthalmia may be treated with saline drops and patients are referred to an ophthalmologist for ciclosporin (cyclosporine) ophthalmic drops if symptoms are severe.
Punctal plugs can also be used to treat xerophthalmia.
To avoid this adverse effect, the lowest possible dose should be used initially and given before bedtime.
The dose can be titrated slowly as the BP allows.
Early identification of pulmonary HTN is crucial, as multiple therapies are currently available.
Referral should be made for evaluation by a pulmonary artery hypertension specialist, including right heart catheterisation, before initiation of therapy.
Treatment should be given according to guidelines for the management of idiopathic pulmonary hypertension: for example, American College of Chest Physicians.[64]
Treatment includes endothelin-1 receptor antagonists (bosentan, ambrisentan, macitentan), phosphodiesterase-5 inhibitors (sildenafil, tadalafil), inhaled prostacyclin (iloprost), subcutaneous treprostinil, or intravenous epoprostenol.
These therapies have been shown to be of clinical benefit in scleroderma patients with pulmonary artery HTN.[71]
Needed if patient has dysphagia due to an oesophageal stricture.
Requires radiographic or endoscopic visualisation to diagnose a stricture, as dysphagia may be due to non-synchronised motility without a fixed stricture.
The stricture should be suspected with new onset of dysphagia or failure to thrive.
Strictures may need endoscopic balloon dilation.
A change in the squamous epithelium of the oesophagus to intestinal-type epithelium with metaplasia on biopsy.
May occur as a long-term complication of heartburn.
Therefore, it is important to treat heartburn early.
May occur as a complication of oesophageal stricture and dysphagia. Requires an assessment of swallowing.
Shortness of breath and decreased exercise tolerance may be symptoms of chronic aspiration.
Patients with progressive dysmotility throughout the gastrointestinal tract may require continuous hyper-alimentation as a last resort, if all other methods to treat dysmotility have failed.
Treatment with pro-kinetic agent as well as evaluation for bacterial overgrowth should be done prior to initiation.
Treatment will depend on the type of arrhythmia diagnosed and the clinical condition of the patient.
Anti-arrhythmics or insertion of a pacemaker may be required.
If beta-blockers are used, then selective beta-blockers are recommended in order not to exacerbate symptoms of Raynaud's phenomenon.
Atenolol and metoprolol are B1-selective agents that may be used for some tachycardic arrhythmias.
Diltiazem and verapamil can be used for treatment of arrhythmias when beta-blockers must be avoided.
Patients with partial blocks may suddenly progress to third-degree heart block due to cardiac involvement of scleroderma.
Occurs in cases of long-term methotrexate use.
Liver function tests should be monitored every 3 to 4 months.
To avoid this adverse effect, the lowest possible dose should be used initially.
Adequate hydration is recommended to prevent this complication. Prophylaxis with mesna is also regularly used.
The incidence is lower with intravenous use compared with oral treatment.
Usually early in course of treatment.
Patients may present with cough, hypoxia, and diffuse infiltrates on chest x-ray.
Infection must be ruled out and methotrexate withdrawn. Treated with high-dose corticosteroids.
Decreased risk with monthly pulse intravenous treatment due to less cumulative exposure.
Can give the gonadotropin-releasing hormone analogue leuprolide midway through the menstrual cycle, and time the cyclophosphamide infusion to minimise ovarian exposure.
For men, sperm banking should be addressed.
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