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Scleroderma

The Mayo Clinic (2022) defines scleroderma as a disease that involves the hardening and tightening of the skin. It may also affect blood vessels, internal organs and the digestive tract. If only the skin is affected, it is called morphea. There is no known cure for scleroderma; however, treatments can ease symptoms and slow progression. Onset usually affects the hands, feet and face, but the arms, chest, abdomen and legs can also be involved. Early symptoms may include swelling and itchiness. In addition, the skin colour may change or appear shiny. If the organs become affected, for example, the heart or lungs, it may cause shortness of breath, dizziness, increased blood pressure, irregular heartbeat, heart failure and edema in the legs.

Causes

The Mayo Clinic (2022) explains that scleroderma results from an overproduction and accumulation of collagen within the tissues. The cause of scleroderma is unknown; however, the immune system likely plays a role. It is hypothesized that genetics and the environment are triggers that contribute to its development.

Complications

The Mayo Clinic (2022) report the following complications of scleroderma:

  • Raynauds’s phenomenon may be present whereby blood flow is restricted to the fingers and toes and skin sores may develop. Tissue can become severely affected and die.
  • Lungs can become scarred affecting breathing and exercise tolerance and high blood pressure in the blood vessels that lead to the lungs.
  • The kidneys may become involved which can lead to kidney failure or decreased function.
  • The heart may become scarred which can lead to abnormal heartbeats congestive heart failure or inflammation around the heart.
  • The mouth can become smaller and narrower leading to difficulty brushing, or cleaning. Saliva production may be reduced leading to dental decay.
  • Digestive issues include heartburn, difficulty swallowing, cramps, bloating, constipation or diarrhea. Absorption of nutrients may be affected.
  • Joint flexibility and movement can become restricted.

Treatment

According to the American College of Rheumatology (2022), no drug treatment has been proven to stop or reverse the disease’s main symptom: the thickening and hardening of the skin. Therefore, the focus is to curb symptoms with drugs and prevent further complications. However, research continues into new treatments for scleroderma.

Bongi et al. (2011) conducted a randomized controlled trial that Manual Lymph Drainage (MLD), precisely the Vodder method, can reduce hand edema and improve hand function and perceived quality of life. At Flow Lymphatic Health Clinic, all of our Massage Therapists and Physios are certified in the Vodder method of MLD. In the Bongi et al. (2011) study, they treated patients who had edema in their hands. We believe that MLD could also be applied in earlier stages of the disease and may slow down the progression or manage symptoms. MLD can be beneficial for wound healing as well. Unfortunately, when organ function becomes involved, MLD should not be used in the treatment of scleroderma. However, naturopathic, osteopathic, physiotherapy and acupuncture would be recommended at any stage of the disease.

JZ Bown, our Registered Acupuncturist at Flow, explains how Traditional Chinese Medicine (TCM) treats scleroderma. People with scleroderma are diagnosed the same as every other health condition through syndrome differentiation. This means seeing the patient for their conditions which are unique to the individual, as well as considering the overall understanding of the symptoms. This is why TCM treatment plans may differ among people with scleroderma and why relying on a registered professional to discern and treat what is for your optimal overall outcome is essential. TCM treatments may provide long-lasting effects such as improved skin elasticity, colour and texture recovery. In addition, TCM works to restore balance and strengthen the body’s defence (immune) system. A favourable side effect of bringing the body system to greater balance is symptoms of other pre-existing conditions may also improve. As a guide, treatment for scleroderma involves herbal medicine every day for months and a commitment to dietary changes. Acupuncture and Tui Na (TCM massage) are also utilized in managing scleroderma. The potential long-lasting effects make it appealing for those willing to commit to a TCM practitioner’s treatment plan. 

Dr. Pushpa Chandra, the Naturopathic Doctor (ND) at Flow, offers the following treatments for scleroderma:

  • Balance the biochemistry by calming the immune system and reducing the triggers of scleroderma is the key. In addition, gut microbiome probiotics such as Lactobacillus acidophilus and Lactobacillus Bifidus are recommended. 
  • Vitamin D supplementation. Diaconu et al. (2021) found that low levels of Vitamin D are typical in scleroderma patients and could be associated with their varying disease patterns.
  • Avoid gluten, legumes and dairy. 
  • Reduce exposure to pesticides. Rubio-Rivas et al. (2017) conducted a meta-analysis and found evidence for occupational and environmental exposures contributing to scleroderma.
  • Glutathione treatments are recommended
  • Curcumin can be used as an anti-inflammatory for pain.

Ivan Duben, an osteopathic practitioner affiliated with Flow Lymphatic Health, shares his point of view on treating scleroderma. Systemic Sclerosis (scleroderma), also known as “hard skin,” is an autoimmune disease with a propensity towards multisystem involvement, including the organs. Scleroderma is typically associated with any disease affecting blood vessels in the form of vasculopathy which has evolved from metabolic dysfunction, inflammation, and tissue degeneration. The persistence of autoantibodies reflects a breakdown in the body’s terrain as it develops fibrosis of the skin and, eventually, the organs (Calderon & Pope, 2021). 

Scleroderma is almost always preceded and accompanied by some form of edema. Whether or not a patient presents with localized or systemic edema, Ivan’s approach to care does not change. The goal is to support the health of connective tissues by enhancing the biological terrain vitality. Recent findings have concluded that moving the disease process beyond the state of management via medications such as glucocorticoids, vasodilators, and immunosuppressants, will require a change in the biological composition of the terrain. The biological shift would require modification of the disease process, which would promote the decrease of inflammation in the form of cytokines and mediating cell surface molecules (Zhaoet al., 2022). 

Improving the body’s ecosystem allows systemic dysfunction to reverse itself gradually. Soft tissue techniques focus on restoring blood flow by reducing the influence of adhesions between structures inside the body. Liberating tissues will restore optimal blood flow to organs and tissues, thereby allowing detoxification pathways to reduce inflammatory factors by reducing edema. Typical osteopathic manual therapy (OMT) is designed to maintain joint mobility, which has been shown to enhance clinical outcomes. Much of the scleroderma disease process occurs in the fascia’s deepest layers, requiring (OMT) soft tissue techniques (Aun et al., 2021). Ivan Duben believes there is no doubt that the excess formation of scar tissue will decrease blood flow in both localized and systemic scleroderma. As an osteopathic practitioner, Ivan does not diagnose or practice medicine and remains distinct from the Physician trained American osteopaths. Ivan can, however, refer to a collection of symptoms provided by physicians that are common in his practice. 

In many cases, the soft tissue techniques are not applied directly to areas exhibiting symptoms of scleroderma, as this would be too painful. For example, Ivan has found significant benefits in restoring optimal function, shape, and position of the liver, especially with relation to the diaphragm. No other organ in the body influences the workings of the immune/detoxification terrain of the body more than the liver. Ivan also adds that a consultation with a professional regarding eliminating food triggers and supporting the body’s microbiome is critical in transforming the body’s terrain from a toxic ecosystem to a thriving and resilient environment.

Patients affected by scleroderma can receive physiotherapy treatment through Vancouver Coastal Health at the Mary Pack Arthritis Program in Vancouver. A doctor’s referral is needed; this is usually a rheumatologist and often through the Scleroderma Clinic at the centre. Treatment is generally multi-disciplinary and may include physiotherapy (PT), occupational therapy (OT) and social work (SW).  

A primary focus is on patient education to achieve self-management of the condition, including skin (sclerosis, calcinosis, digital ulcers), Raynaud’s, oral health and nutrition, pain and fatigue, effects on daily life (work, activities of daily living, social and leisure participation). Exercises for range of motion (ROM) and stretching, especially hands and oral/facial, are taught by PT and OT. PT treatment routinely includes manual therapy (including massage, stretching, and mobilization). PT also addresses general strength and conditioning to prevent proximal muscle weakness and cardiovascular training, including management of dyspnea and monitoring exertion. OT provides additional adaptations for skin and joint protection and hand and foot care, especially in the presence of Raynaud’s and digital ulcers. SW may give additional counselling for the psychosocial aspects of coping with a debilitating chronic disease. Referrals may be made for outpatient/community-based Speech-Language Pathologists (to address dysphagia), Registered Dietitians (for nutrition), and Registered Nurses (for wound care).

Written by:

Denise Drisdelle, RMT

JZ Bown, R.Ac.

Heather Walker, PT

Ivan Duben, D.O.M.P.

Dr. Pushpa Chandra, ND

 

References

American College of Rheumatology. (2022). Scleroderma. https://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Scleroderma

Aun, J. A., Knox, R. S., & Roberts, J. E. (2021). The enigmatic fascia: eosinophilic fasciitis. Journal of osteopathic medicine, 121(6), 555–559. https://doi.org/10.1515/jom-2020-0284

Bongi, S. M., Del Rosso, A., Passalacqua, M., Miccio, S., & Cerinic, M. M. (2011). Manual lymph drainage improving upper extremity edema and hand function in patients with systemic sclerosis in edematous phase. Arthritis care & research, 63(8), 1134–1141. https://doi.org/10.1002/acr.20487

Calderon, L. M., & Pope, J. E. (2021). Scleroderma epidemiology update. Current opinion in rheumatology, 33(2), 122–127. https://doi.org/10.1097/BOR.0000000000000785

Diaconu, A. D., Ostafie, I., Ceasovschih, A., Șorodoc, V., Lionte, C., Ancuța, C., & Șorodoc, L. (2021). Role of Vitamin D in Systemic Sclerosis: A Systematic Literature Review. Journal of immunology research, 2021, 9782994. https://doi.org/10.1155/2021/9782994

Mayo Clinic. (2022). Scleroderma. https://www.mayoclinic.org/diseases-conditions/scleroderma/symptoms-causes/syc-20351952

Rubio-Rivas, M., Moreno, R., & Corbella, X. (2017). Occupational and environmental scleroderma. Systematic review and meta-analysis. Clinical rheumatology, 36(3), 569–582. https://doi.org/10.1007/s10067-016-3533-1

Zhao, M., Wu, J., Wu, H., Sawalha, A. H., & Lu, Q. (2022). Clinical Treatment Options in Scleroderma: Recommendations and Comprehensive Review. Clinical reviews in allergy & immunology, 62(2), 273–291. https://doi.org/10.1007/s12016-020-08831-4