Clinical Immunology focuses on the immune system’s health. Learn about the diagnosis and treatment of allergies, autoimmune diseases, and immunodeficiencies.
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The management of scleroderma has undergone a revolution in the last two decades. While the term “cure” remains elusive, “remission” and “disease control” are realistic goals. Treatment at Liv Hospital is organ-based, meaning we deploy specific therapies for specific complications. The strategy is often dual-tracked: vascular therapies to improve blood flow and immunosuppressive therapies to halt inflammation and fibrosis. This requires a dynamic, aggressive approach, usually combining medications to achieve the best outcome.
Treating the vasculopathy is the foundation of care, as poor blood flow drives both symptoms and tissue damage.
Drugs like nifedipine or amlodipine are the first line of defense. They relax the smooth muscles of the blood vessel walls, reducing the frequency of Raynaud’s attacks. Patients are often titrated to the highest tolerable dose.
Initially designed for other purposes, drugs like sildenafil and tadalafil are potent vasodilators used in scleroderma. They are particularly effective for healing digital ulcers and are also a mainstay in treating Pulmonary Arterial Hypertension.
Bosentan and ambrisentan block endothelin, a powerful substance made by the body that constricts blood vessels. These are advanced therapies typically reserved for preventing new digital ulcers and treating pulmonary hypertension.
In severe cases where ischemia threatens a digit (gangrene risk) or in advanced pulmonary hypertension, intravenous epoprostenol or iloprost is administered. These mimic the body’s natural vessel dilators and require careful hospital management.
To stop the body from attacking itself and laying down collagen, the immune system must be modulated.
Methotrexate is often the first choice for early diffuse skin disease. MMF has become the gold standard for scleroderma-associated Interstitial Lung Disease (SSc-ILD). Clinical trials have shown MMF to be effective in stabilizing lung function with a manageable side-effect profile.
This is a more potent alkylating agent reserved for severe, rapidly progressive lung disease. Due to higher toxicity risks, it is usually used for a short “induction” period to stop the disease in its tracks, followed by maintenance with MMF.
This is a breakthrough medication specifically approved for Scleroderma-associated Interstitial Lung Disease. Unlike immunosuppressants, nintedanib interferes with the fibroblast’s ability to create scar tissue. It has been proven to slow the rate of decline in lung function and is often used in combination with MMF.
For a select group of patients with early, rapidly progressive diffuse scleroderma who are at high risk of organ failure, Autologous Stem Cell Transplantation is a consideration. This procedure involves harvesting the patient’s own stem cells, using high-dose chemotherapy to “wipe out” the dysfunctional immune system, and then rebooting it with the stored stem cells. It carries significant risks but offers the possibility of long-term drug-free remission and improved survival.
High-dose PPIs (e.g., omeprazole, lansoprazole) are non-negotiable for most patients to prevent esophageal scarring.
Drugs like domperidone or erythromycin help stimulate stomach emptying and intestinal motility, combating bloating and constipation.
Cyclical courses of antibiotics (e.g., rifaximin, ciprofloxacin) are used to treat Small Intestinal Bacterial Overgrowth (SIBO), reducing diarrhea and improving nutrient absorption.
The treatment for Scleroderma Renal Crisis is specific and counterintuitive to general nephrology. ACE Inhibitors (like captopril or enalapril) are the absolute required treatment. They must be started immediately upon suspicion of a crisis. Interestingly, dialysis may be needed temporarily, but many patients recover kidney function after a year or two of aggressive ACE inhibitor therapy, allowing them to come off dialysis—a phenomenon unique to scleroderma.
Digital ulcers require meticulous hygiene, protective dressings, and sometimes surgical debridement. Off-loading pressure from the fingertips is essential.
Hand therapy is vital to prevent contractures. Paraffin wax baths can help soften the skin before stretching exercises. Mouth stretching exercises are prescribed in order to avoid microstomia (small mouth opening).
Pulsed dye laser therapy is effectively used to treat telangiectasias (red spots) on the face, offering significant cosmetic improvement and psychological benefit.
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Doctors are very cautious with steroids (like prednisone) in scleroderma because high doses can trigger Scleroderma Renal Crisis. If used, they are kept at low doses for short periods.
Nintedanib is an anti-fibrotic drug recently approved to slow down the scarring process in scleroderma-associated lung disease.
It is not a guaranteed cure, but it can “reset” the immune system and stop disease progression for many years. It is a significant procedure reserved for severe cases.
Treatment involves keeping them warm and clean, using vasodilator medications to improve blood flow, and sometimes using specialized dressings. Smoking cessation is critical.
While diet can’t cure the fibrosis, eating small, frequent meals, avoiding late-night eating, and reducing fiber (if you have slow motility) can significantly reduce symptoms.
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