Systemic Sclerosis - Causes Symptoms and Management

Abstract

Autoimmune conditions are on a rise nowadays as the food and lifestyles practices have been changed and mankind is consuming a number of toxic substances unknowingly. That with time, obstructs the minute channels of the body and normal functioning of the body starts to hamper at that level, over the period of time, things get worse impacting the electrical signals and other cellular level communication, when this happens normal functioning of our body is wrecked and unwanted/superfluous signals results in deteriorating effects in the body. Likewise, Systemic sclerosis, also known as Chronic Autoimmune Connective Tissue Disorder – Causes, Symptoms & Treatment, is a chronic autoimmune connective tissue disorder characterized by fibrosis, vascular abnormalities, and immune dysregulation. It primarily affects the skin but can involve internal organs such as the lungs, kidneys, heart, and gastrointestinal tract. Early diagnosis and multidisciplinary management are essential to improve outcomes. This article provides an in-depth review of systemic sclerosis, including its causes, clinical features, diagnostic approach, and management in the modern and Indian traditional medicinal system of Ayurveda.

Introduction

Scleroderma, derived from the Greek words "skleros" (hard) and "derma" (skin), refers to a group of autoimmune disorders marked by excessive collagen deposition leading to skin hardening and fibrosis. Among these, systemic sclerosis (SSc) is distinguished by its potential to affect internal organs beyond the skin. The disease manifests through progressive skin thickening, Raynaud’s phenomenon, and multi-organ complications, often leading to significant morbidity and mortality. Approximately 50-300 cases per million worldwide, more common in females (3-4 times more than males).

Classification

It is classified broadly into two types

1. Limited cutaneous systemic sclerosis (lcSSc) — involves skin thickening mainly on the face and extremities.
2. Diffuse cutaneous systemic sclerosis (dcSSc) — more aggressive, with widespread skin involvement and early internal organ fibrosis.

The pathogenesis involves a triad of vascular damage, immune system activation, and fibroblast dysfunction, resulting in abnormal collagen accumulation.

Key Facts

1. Age of onset: Typically between 30 and 50 years.
   
2. Prognosis: Varies by subtype; diffuse form generally has a poorer prognosis due to internal organ involvement.

Causes and Pathogenesis

The exact cause of systemic sclerosis remains unclear but involves complex interactions between genetic susceptibility, environmental exposures, and immune dysfunction.

1. Genetic Factors: Certain HLA class II alleles (e.g., HLA-DQ, HLA-DR) increase susceptibility. Family history is rare but can be present.
   
2. Environmental Triggers: Exposure to silica dust, organic solvents, and certain drugs have been implicated.
   
3. Immune System Abnormalities: Autoantibodies such as anti-centromere, anti-topoisomerase I (Scl-70), and anti-RNA polymerase III are characteristic.
   
4. Vascular Injury: Early endothelial damage leads to intimal proliferation, narrowing of vessels, and reduced blood flow.
   
5. Fibroblast Activation: Dysregulated fibroblasts produce excessive collagen and extracellular matrix components causing fibrosis.
   

Signs and Symptoms

Cutaneous/Skin Manifestations

1. Skin thickening and hardening.
2. Raynaud’s Phenomenon
3. Telangiectasias
4. Calcinosis cutis
5. Digital ulcers

Systemic/Bodily Manifestations

1. Musculoskeletal: Joint stiffness, muscle weakness.
2. Gastrointestinal: Esophageal dysmotility, acid reflux, malabsorption, constipation or diarrhea.
3. Pulmonary: Interstitial lung disease, pulmonary arterial hypertension (PAH) leading to breathlessness.
4. Renal: Scleroderma renal crisis characterized by sudden hypertension and kidney failure.
5. Cardiac: Arrhythmias, myocarditis, pericarditis.

Investigations

1. Autoantibody profile
2. Elevated inflammatory markers (ESR, CRP)
3. Skin Biopsy
4. Nailfold Capillaroscopy:
5. Pulmonary Function Tests
6. CT Chest
7. Echocardiography
8. Renal function tests
9. Esophageal motility studies
   

Management

Systemic sclerosis management requires a multidisciplinary approach focusing on symptom control, preventing complications, and improving quality of life. There is no cure as per modern medicinal system, so treatment is largely supportive and aimed at controlling fibrosis, vascular problems, and immune dysfunction.

Pharmacological Treatment

1. Vasodilators
2. Corticosteroids
3. ACE Inhibitors
4. Proton Pump Inhibitors
5. Immunosuppressants

Non-Pharmacological Measures

1. Skin Care
2. Physical Therapy
3. Nutritional Support
4. Psychological Support

Ayurvedic Insights on Systemic Sclerosis (Scleroderma)

• In Ayurveda, diseases characterized by hardening, fibrosis, and systemic involvement can be correlated with conditions involving Srotodushti (channel obstruction), Dhatu Kshaya (tissue depletion) due to Ama (toxins) accumulation in the minute channels of the body, affecting the all the dosha is vata pitta and kapha doshas and disturbing their normal functioning.
• Faulty dietary habits lead to improper digestion and metabolism which results in producing ama, which blocks microchannels (Srotas). When these micro channels are blocked, normal functioning of the body gets hampered and dosha according to the diet gets vitiated. Improper digestion results in Rasa (plasma) and Rakta (blood) dushti, as these are the primary tissues that are affected, leading to poor nourishment and oxygenation of skin and organs, contributing to fibrosis and chronic inflammation. Rakta Dhatu also gets vitiated in this condition, causing daha (burning sensation), raga (redness), Twak Kandu (Pruritis).
• As these things remain in action in the body for a longer run express the condition of scleroderma. There is involvement of all the doshas.  Furthermore, when Vata Dosha gets vitiated it leads to impaired circulation, dryness, stiffness, and fibrosis—hallmarks of scleroderma such as skin tightening and joint contractures and causes depletion of normal Locomotory functions. Kapha aggravation causes excessive accumulation of meda (fat) and Mamsa (muscle) tissues along with mucus and toxins, leading to thickening and obstruction in channels (Srotas). The hardening and fibrosis of skin resemble Sopha (swelling) and Shosha (atrophy) with vitiated Vata and Kapha. Raynaud’s phenomenon can be seen as a manifestation of Vata blockage causing impaired blood flow. Internal organ involvement corresponds to Srotorodha (channel blockage) and dhatu (tissue) vitiation.

Ayurvedic Management

• Ayurveda emphasizes restoring balance by treating the root cause—removing toxins, improving digestion, balancing doshas, and rejuvenating tissues (Rasayana therapy). Since systemic sclerosis is chronic, a long-term individualized treatment plan focusing on strengthening the immune system and preventing complications is vital.
• First of all the digestion of the patient is taken care of along with dosha balancing by focusing on pacifying vata, pitta and kapha through diet, lifestyle, and herbs. And avoiding cold, dry, and heavy foods which aggravate Vata and Kapha. Further detoxification therapies (Shodhana) such as therapies like Virechana (therapeutic purgation) and Basti (medicated enemas) help remove ama and restore Srotas function. Gentle Swedana (sudation) helps reduce stiffness and improve circulation.
• Furthermore, herbs such as ashwagandha (Withania somnifera) pacifies vata, rejuvenates tissues, improves immunity, Guduchi (Tinospora cordifolia) detoxifies and immune modulator, Shatavari (Asparagus racemosus) nourishes dhatus, reduces inflammation, Guggulu (Commiphora mukul) acts as anti-inflammatory, improves circulation, Manjishtha (Rubia cordifolia) purifies blood and reduces fibrosis and  various other herbs are used.
• Moreover, Abhyanga (oil massage) with warming oils like Mahanarayan oil or Dashamoola oil to relieve stiffness and improve skin flexibility. And Udwartana (herbal powder massage) stimulates circulation and breaks down fibrotic tissue. In diet, warm, cooked, and easily digestible meals are advised  and avoiding cold, raw foods and excessive physical or mental stress is to be taken into consideration.

Herbal Remedies for Scleroderma by Planet Ayurveda

Planet Ayurveda is a globally recognized Ayurvedic wellness brand dedicated to promoting holistic healing through the ancient science of Ayurveda. Based in India, it offers a wide range of herbal products that are formulated using traditional Ayurvedic principles and prepared from pure, natural ingredients. The brand emphasizes quality, authenticity, and safety by ensuring that its formulations are free from chemicals, additives, and preservatives. Planet Ayurveda supports overall well-being through herbal supplements, classical formulations, and personalized health solutions. It is trusted by people worldwide for its commitment to restoring balance in the body, mind, and spirit through natural and time-tested Ayurvedic practices. Planet Ayurveda is pleased to present its Sclero care pack that has been a magnificent pack for the management of Scleroderma optimally. The products enlisted in the pack are as follows:

1. Ashwagandha capsules
2. Boswellia Curcumin
3. Gotukola capsules
4. Sanjivani vati
5. Gandhak Rasayan
6. Sinhnaad Guggul

Product Description

1. Ashwagandha Capsules

Ashwagandha Capsules are a product of Planet Ayurveda. Ashwagandha (Withania somnifera) plays a supportive role in the management of scleroderma due to its potent anti-inflammatory and anti-fibrotic properties. It contains a key bioactive compound, Withaferin A, which helps inhibit fibrosis by regulating pathways involved in excessive collagen production and immune response. It modulates inflammatory cytokines and reduces oxidative stress, both of which contribute to the progression of scleroderma. Additionally, Ashwagandha supports adrenal function and helps reduce stress, which indirectly triggers autoimmune conditions. These combined effects make Ashwagandha a valuable herb in Ayurvedic management for reducing symptoms and improving quality of life in scleroderma patients.

Dosage : One capsule twice daily with warm water.

2. Boswellia Curcumin

Boswellia Curcumin is a patent product of Planet Ayurveda. The capsules are composed of standardized extracts of Shallaki (Boswellia serrata) and haridra (Curcuma longa), and provide potential benefits in managing scleroderma through their anti-inflammatory and antioxidant properties. Boswellia serrata is known to reduce inflammation by inhibiting pro-inflammatory enzymes like 5-lipoxygenase, while Curcumin modulates inflammatory cytokines and oxidative stress pathways. These combined actions really help alleviate symptoms associated with scleroderma, such as skin stiffness and joint discomfort. The individual components have been studied for their effects on inflammation and immune modulation and have potential supportive roles in managing autoimmune conditions. Therefore, Boswellia Curcumin is indeed a must product for the management of Scleroderma

Dosage : Two capsules twice daily with warm water.

3. Gotukola capsules

Gotu Kola Capsules are a product of Planet Ayurveda. Gotu Kola (Centella asiatica) is a great remedy in the management of scleroderma due to its anti-inflammatory, antifibrotic, and circulation enhancing properties. The herb contains active compounds like asiaticoside and madecassoside, which inhibit collagen synthesis and reduce fibrosis, addressing the excessive collagen production characteristic of scleroderma. Additionally, Gotu Kola's ability to improve microcirculation alleviates vascular symptoms associated with the condition. It has been traditionally used for skin related problems and pitta kapha dosha vitiation. Thus, Gotu Kola is a valuable addition in the holistic management of scleroderma.

Dosage : Two capsules twice daily with warm water.

4. Sanjivani vati

Sanjivani Vati is a classical Ayurvedic formulation which offers supportive benefits in managing scleroderma through its detoxifying, anti-inflammatory, and immunomodulatory properties. It is comprised of potent herbs like Guduchi (Tinospora cordifolia), Pippali (Piper longum), Haritaki (Terminalia chebula), and Shuddha Bhallataka (Semecarpus anacardium), it aids in digesting "Ama" (toxic metabolic waste), a major cause in autoimmune conditions. By enhancing Agni (digestive fire), it reduces toxin accumulation with aampachan, thereby alleviating symptoms such as skin stiffness and joint discomfort. Its anti-inflammatory effects help mitigate tissue inflammation, while immunomodulatory actions support balanced immune responses. Additionally, Sanjivani Vati promotes liver function and improves digestion, contributing to overall systemic balance. These combined actions make it a valuable remedy in the management of scleroderma.

Dosage :  Two tablets twice daily with warm water.

5. Gandhak Rasayan

Gandhak Rasayan is a classical Ayurvedic formulation made from purified sulfur (Shuddha Gandhak) which offers supportive benefits in managing scleroderma with its anti-inflammatory, antimicrobial, and immunomodulatory properties. It aids in detoxifying the body, enhancing immune function, and improving skin health. By promoting the removal of toxins and reducing inflammation. Gandhak Rasayan helps alleviate symptoms such as skin stiffness, discoloration, and discomfort associated with scleroderma. It balances the body's doshas and supports overall well-being in individuals with autoimmune conditions like scleroderma as well as other skin conditions. Being Rasayan, it is of great help in the management of Scleroderma and its associated symptoms.

Dosage :  Two tablets twice daily with warm water.

6. Sinhnaad Guggul

Sinhnaad Guggul is a classical Ayurvedic formulation and is utilized in managing scleroderma due to its detoxifying, anti-inflammatory, and immune-modulating properties. Comprising ingredients like Triphala, Gandhaka (purified sulfur), and castor oil, it is helpful in  ama pachana through enhancing digestive fire that removes toxins and clearing out microchannels. As a result of this, symptoms such as skin stiffness and joint discomfort etc are alleviated. Its anti-inflammatory effects help mitigate tissue inflammation, while immunomodulatory actions support balanced immune responses. These combined actions make it a valuable product in the management of scleroderma.

Dosage : Two tablets twice daily with warm water.

Conclusion

Systemic sclerosis is a complex autoimmune disease with variable presentation and prognosis. Advances in understanding its pathophysiology have improved diagnosis and management, yet challenges remain. Early recognition, comprehensive care, and patient education are key to enhancing quality of life and reducing complications. Ongoing research continues to explore targeted therapies aiming to halt or reverse fibrosis and vascular damage in the modern medicinal system and yet their treatment is based on managing the symptoms and there is no cure of scleroderma. However, the Indian traditional medicinal system of Ayurveda deals with this condition very nicely and provides best results by fixing the very root of the disease and the provided Sclero Care Pack is a  perfect blend of herbal products that are very effective in the condition of Scleroderma