Scleroderma

What is Scleroderma?

Scleroderma is a rare group of autoimmune connective tissue diseases, with two main forms: localized scleroderma, which is limited to the skin and tissue beneath it, and systemic sclerosis, which also involves blood vessels and internal organs.^[1] Both involve hardening of the skin from excess scar-like tissue.

Systemic sclerosis is the form linked to higher mortality, and it is divided into limited and diffuse cutaneous types based on how much skin is thickened.^[1]

Common symptoms

Symptoms depend on the type of scleroderma and which organs are involved.^{[1, 2]}

• Tight, thickened skin, often starting in the fingers and hands
• Raynaud's phenomenon, in which fingers or toes turn white or blue in cold or stress
• Heartburn, swallowing difficulty, bloating and constipation from the digestive tract
• Fatigue, and joint pain or stiffness
• Shortness of breath or cough if the lungs are involved, and, less often, heart or kidney problems^[1]

Localized scleroderma is not associated with Raynaud's phenomenon or internal organ involvement.^[1]

Causes and risk factors

The cause of scleroderma is unknown, and its development is complex.^[1] It is considered an autoimmune disease in which immune activation, damage to small blood vessels and fibrosis occur together.^[2] Genetic and environmental factors are thought to contribute, and it is not inherited in a simple way.

How it is diagnosed and treated conventionally

Diagnosis is made by a physician from skin findings, symptoms such as Raynaud's phenomenon, and blood tests for specific antibodies. Antinuclear antibodies are found in more than 90% of cases of systemic sclerosis.^[1] Imaging and organ testing look for lung, heart and kidney involvement. We do not diagnose or monitor scleroderma.

Treatment depends on which organs are affected. The 2023 EULAR guideline covers Raynaud's phenomenon, digital ulcers, pulmonary hypertension, kidney crisis, skin and lung fibrosis, and digestive and joint problems, with drugs such as mycophenolate, nintedanib, rituximab and tocilizumab recommended for fibrotic disease.^[3] Non-drug care such as exercise, smoking cessation, cold avoidance, and mouth and hand exercises is recommended to complement drug treatment, not replace it.^[4]

Why Scleroderma calls for a whole-person approach

Scleroderma involves several processes at once, including inflammation, damage to small blood vessels, and fibrosis, and these can affect many organs.^[2] Treatment is tailored to each person's pattern of disease and goals of care.^[2]

Supportive care can address some of the daily burdens that medicines do not fully relieve, always with the rheumatology team leading.

Blood vessels and circulation

Vascular injury is a hallmark of systemic sclerosis, and it shows up in daily life as Raynaud's phenomenon and, in some people, digital ulcers.^{[2, 3]} Cold avoidance is part of standard non-drug advice.^[4]

Fibrosis and skin tightness

Excess collagen deposition thickens the skin and stiffens the hands and mouth.^[1] Hand and mouth exercises are recommended in systemic sclerosis.^[4]

Fatigue, function and mood

Systemic sclerosis is highly disabling, and the research on non-drug care for it is limited.^[5] A review for the EULAR fatigue guideline found that exercise reduced fatigue in rheumatoid arthritis, lupus and spondyloarthritis, but the reduction in systemic sclerosis was not statistically significant.^[6]

Digestive involvement

The digestive tract is one of the organs most commonly affected, which is why reflux, swallowing trouble and bloating are frequent complaints.^[1] These need medical evaluation.

Acupuncture as supportive care in Scleroderma

What the research shows

There are no clinical trials showing that acupuncture helps scleroderma itself. A review of complementary therapies in systemic sclerosis noted that some studies suggest acupuncture, among other approaches, may be beneficial, but that large randomized trials have not been done.^[7]

The closest evidence is for Raynaud's phenomenon. A meta-analysis of 6 trials with 272 people found that acupuncture was associated with more remission and fewer daily attacks, but a network analysis found no significant effect of any acupuncture type, and the authors rated the evidence as very low quality with a high risk of bias and urged caution.^[8] These trials were not specific to scleroderma. An earlier review of treatments for secondary Raynaud's phenomenon, which includes scleroderma, found only one acupuncture trial and clear evidence only for calcium channel blockers and iloprost.^[9]

How acupuncture may work

We found no studies explaining how acupuncture might act in scleroderma. The Raynaud's trials reported outcomes such as remission rates and attacks, not mechanisms.^[8] Any explanation for scleroderma would be speculation.

What treatment involves

Treatment uses fine, sterile, single-use needles, usually left in place for about 20 to 40 minutes, with a course of visits and reassessment. Gentle technique is advisable in people with thickened or fragile skin, and needling over skin ulcers or very tight skin should be avoided.

Serious problems from acupuncture are rare. In prospective studies, about 9 in 100 patients had a minor reaction such as soreness or bleeding at a needle site, and serious events occurred in about 1 in 10,000 patients.^[10] Tell the practitioner first if you have digital ulcers, a bleeding disorder, take blood thinners or immunosuppressants, are pregnant, or have a pacemaker (relevant to electroacupuncture).

Ayurvedic medicine as supportive care in Scleroderma

The Ayurvedic view

Ayurveda has no exact equivalent of systemic sclerosis. Practitioners may relate hardening, tight skin to dry, rough or stiff tissue and to imbalances of Vata and Kapha. This is a traditional framework, not a biomedical diagnosis, and it should not guide medical decisions.

Therapies that may be used

Care is individualized and gentle, and may include:

• Warm, regular meals suited to digestion, and steady sleep and rest routines
• Gentle warm oil massage of the hands and body, keeping the skin warm and avoiding cold
• Stress-reduction practices such as breathing exercises and meditation
• Herbs selected by a practitioner after review of your medicines, from tested sources, since some Ayurvedic products contain heavy metals^[11]

Panchakarma is not appropriate during pregnancy, acute illness or frailty, and it should not be used in systemic scleroderma without your rheumatologist's agreement, because purgative and enema therapies can affect fluids, digestion and medicines.

What the research shows

There are no clinical trials of Ayurvedic treatment for scleroderma. A PubMed search found none. Turmeric (curcumin) appears among complementary therapies that some studies suggest could be helpful in systemic sclerosis, but a review notes that large randomized trials have not been conducted.^[7]

Herbal medicine as supportive care in Scleroderma

Herbs and formulas that have been studied

Most research is on herbs for Raynaud's phenomenon, not scleroderma. Chinese formulas studied for primary Raynaud's phenomenon include Danggui Sini Tang and Buyang Huanwu Tang.^[12] Other herbs studied for Raynaud's include Ginkgo biloba, in a single trial.^[13] A survey of 100 people with systemic sclerosis found 55% had used herbal products, most often green tea, St John's wort and turmeric.^[14]

What the research shows

There is no clinical evidence for scleroderma itself, and the Raynaud's evidence is weak. A review of 8 Chinese herbal trials with 674 people in primary Raynaud's found that methodological quality was generally poor, and concluded the benefits were inconclusive.^[12] A review of natural formulations in scleroderma, drawing on 22 articles, reported that natural products can modulate inflammatory mediators and collagen production, and suggested they might serve as an adjunct treatment. It was an overview, not a pooled analysis of trials, so it does not show that any herb works in people.^[15] The survey of people with systemic sclerosis noted that studies on the effectiveness of these products are insufficient.^[14]

Safety and herb-drug interactions

Herbs can interact with medicines, which matters in scleroderma because many people take immune-suppressing drugs, blood pressure and vasodilator drugs, and blood thinners. A review of herb-drug interactions describes St John's wort lowering ciclosporin and tacrolimus levels, with organ rejection in some cases, and ginkgo causing bleeding with warfarin or aspirin.^[16] Case reports link combining warfarin with herbs including dang gui, dan shen, ginger, ginseng and Boswellia to bleeding and other serious effects.^[17]

Herbal and dietary supplements account for about 20% of drug-induced liver injury in the United States.^[18] About one fifth of Ayurvedic products bought online contained detectable lead, mercury or arsenic.^[11] Herbs should come from tested sources and be prescribed individually. Tell us if you are pregnant or breastfeeding, or have liver or kidney disease. Kidney involvement is a serious complication of scleroderma, so kidney function should be known before any herb is used.

Translating traditional medicine into modern biological language

The links drawn here are interpretation, not equivalence. Chinese medicine often describes cold fingers and hardened skin as cold with blood stasis and obstruction in the channels, and Chinese formulas for Raynaud's are written to warm and move blood. Researchers studying natural products in scleroderma look at inflammatory and oxidative mediators, immune cell function and collagen synthesis.^[15]

Ayurveda's Vata imbalance, with cold, dry and rough qualities, describes the same experience of cold and stiffness in traditional terms. No study has shown that a dosha or a TCM pattern corresponds to a specific biological process in scleroderma.

How this care fits with your medical care

Scleroderma needs specialist care, usually from a rheumatologist, with regular monitoring of the lungs, heart and kidneys. For a condition like this, we ask that you are under a physician's care for it and that your treating team knows you are receiving complementary care. Our role is supportive, for comfort, stress, fatigue and circulation-related symptoms, after your condition is under medical management.

Do not stop or change prescribed medicines without your prescriber. Please bring your diagnosis and type of scleroderma, recent test results, and a full list of medicines and supplements.

When to seek urgent medical care

Systemic sclerosis can cause serious organ problems that need urgent evaluation.^{[1, 3]}

• A sudden severe headache, very high blood pressure, or a sharp drop in urine output, which can signal kidney crisis
• New or worsening shortness of breath, chest pain, fainting or swelling of the legs
• A finger or toe that stays white, blue or black, or a digital ulcer that is painful, red or draining pus
• Trouble swallowing food, vomiting blood or black stools
• Fever with spreading redness or a wound that does not heal

If you have severe symptoms, call 911 or go to the nearest emergency department.