What is Scleroderma? - Symptoms, Causes & Management

Abstract

Connective tissues, a group of tissues which protect, support, and provide structural integrity to other tissues and organs in the body. They are made up of various cells, fibers, and extracellular matrix. They majorly are adipose tissue, bone, cartilage, blood, and lymph. Connective tissues play vital roles in protecting organs, storing energy, transporting nutrients and gases (blood), and aiding in immune defense. They also help in wound healing and maintaining the body’s structure by supporting and connecting different parts of the body. Their diverse functions are essential for overall body function and homeostasis. One autoimmune disorder that can impair connective tissue health is scleroderma, which disrupts our normal functioning. It starts from skin and then involves other organs too.

Introduction

Scleroderma or systemic sclerosis is a rare and chronic autoimmune disorder of connective tissue. It is characterized by the hardening and tightening of the skin and connective tissues. This rare condition results due to an overproduction and accumulation of collagen, affecting both the skin and internal organs.  Collagen is a protein that helps provide structure and support to the skin and other organs. The excessive collagen starts hardening the connective tissue and then causes deformation of the particular part. This condition can also affect internal organs such as the lungs, heart, kidneys, and gastrointestinal tract, leading to life-threatening complications if left untreated. While there is no 100 % cure for scleroderma , treatments can reduce symptoms, slow its progression and improve the quality of life.

Causes

Since it is an autoimmune condition, therefore there is no specific cause. But few factors can influence its predisposition. They are:

1. Genetic: Few genetic mutations are believes to increase the susceptibility for developing this disease.
2. Environmental triggers: Exposure to certain viruses like EPV [Epstein-Barr virus], Silica dust can be a potential triggering factor.
3. Hormonal factors: Scleroderma is more prevalent in women indicating maybe hormones influence its predisposition.
4.  Immune dysfunction: Here, the immune system triggers overproduction of collagen which accumulates in skin and other organs causing stiffness and scarring. Also in people whose immune system is already compromised because of other pertaining auto-immune conditions like RA, SLE etc.
5. Other factors like smoking, stress, and poor nutrition can aggravate the condition, making the body more vulnerable to collagen overproduction and immune dysfunction.

TYPES

1. Localized / Limited Scleroderma

It affects only a specific part of the body and is mostly superficial. It is commonly seen in children and adults. It is a less severe type.

2. Systemic / Diffuse Scleroderma
It is also called systemic sclerosis. It affects skin, blood vessels, lungs and other various organs. It is a serious condition and can lead to life-threatening complications.

Symptoms

Presentation of symptoms varies in accordance to the involvement of various body organs. They can be

Skin-related Symptoms

1. Hardening and tightening of skin, often beginning in fingers, hands &face.
2.  Skin becomes shiny, hard and tight.
3. Pigment changes in skin [darkening and lightening of skin]
4. Sclerodactyly: claw-hand appearance due to excessive skin tightening and fingers extend inwards. There is significant loss of hand function and mobility.
5. Formation of ulcers on fingertips.
6. Raynaud’s phenomenon can develop – In this there is discoloration of toes or fingertips due to stress or cold.

Digestion-related Symptoms

1. Bloating
2. Mal-absorption
3. Dysphagia - Difficulty in swallowing
4. Acid reflux

Cardiovascular Symptoms

1. Irregular heart rhythms
2. High blood pressure
3. Heart failure

Renal Involvement

• Scleroderma renal crisis- in this there is sudden onset of severe hypertension and progressively leading renal failure

Musculoskeletal Involvement

1. Joint pain
2. Muscle weakness
3. Stiffness
4. Limited range of motion

Pulmonary Symptoms:

1. Cough
2. Shortness of breath
3. Scarring of lung parenchyma (fibrosis)

Generalized Symptoms:

1. Malnutrition
2. Fatigue
3.  Weight loss
4. Anemia
5.  Calcium depositions in connective tissues
6. Organ failure in cases of severe scarring and thickening

Grading

In Scleroderma, a modified Rodnan skin score (MRSS) is used to analyze the extent and severity of skin thickening. This scale has a 0-3 score which is decided on the basis of skin of 17 body areas. It is important to monitor your MRSS score to know the prognosis of the disease.

Diagnosis

Physical examination is of prime importance for diagnosing scleroderma. Along with it few blood tests are also recommended for confirmatory diagnosis and to rule out any associated manifestations.

1.  ANA (Antinuclear Antibodies): positive result indicates auto-immune scleroderma.
2. Specific Auto-antibodies tests like Anti-Scl-70; Anti-centromere, Anti-RNA polymerase III for specific diagnosis of scleroderma are done.
3. CBC: for anemia
4. X-Rays, CT scan, MRI for assessing internal organ involvement.
5. Pulmonary Function Tests: To evaluate lung function and detect pulmonary fibrosis or restrictive lung disease.
6. Endoscopy or other tests are done if needed on the basis of organ involvement.

Treatment & Lifestyle Adjustments

 Currently, there is no cure for scleroderma, and treatment focuses on managing symptoms and improving the patient's quality of life. The main goals of treatment are to control inflammation, slow the progression of fibrosis, and address organ-specific complications.

1. Medications

Symptomatic medications are given like Immuno-suppressants, Corticosteroids, Proton-pump inhibitors [to manage gastric symptoms], and high BP medications.

2. Physical Therapy

Regular stretching and exercise can help maintain flexibility, improve blood flow, and reduce stiffness in the skin and joints.

3. Quit Smoking

Smoking can exacerbate symptoms, particularly Raynaud's phenomenon and lung involvement.

4. Stress Management

Reducing stress through techniques like meditation, yoga, or counseling.

5. Organ-Specific Treatment

Depending on the affected organs, treatments might include oxygen therapy for lung problems, dialysis for kidney issues, or heart medications for cardiovascular symptoms.

6. Skin-care

Regular moisturizing to keep the skin hydrated, as well as gentle skincare routines to prevent further irritation.

Ayurvedic Overview

In Ayurveda, there is no direct correlation for Scleroderma but it can be co-related to “Twaka-Gata vata” which means vitiated vata gets lodged in the skin. It is explained in the “Vata-vyadhi Adhyaya” by Acharya Charaka (Father of Medicine in Ayurveda). This disease manifests due to over-consumption of dry food items, physical exhaustion, improper treatments, excessive stress, or due to any trauma the Vata gets aggravated, & gets accumulated in our skin and develops symptoms.

In this verse, the symptoms similar to scleroderma when in skin are seen. It explains when vitiated vata gets lodged into skin it leads to development of dryness, fissures, numbness, thin [emaciated fingers] and blackish pigmentation. It causes pain, reddening of skin, stretching of skin, and pain in distal ends of bones.

Various therapies are advised for balancing vata and strengthening of connective tissue. These are Abhyanga [hot oil massage] followed by Swedana [fomentation], Avgahana [hot immersion bath with vata balancing herbs] are advised. Use of oils like Baladi Tailam, Sahacharadi Tailam, Rasnadi Tailam, Amritadi Tailam, Mahanarayan Tailam, Ksheerabaladi Tailam is indicated to decrease the vitiated vata.

Ayurvedic Herbs Beneficial In Scleroderma

 1. Haridra

Haridra increases our Agni (metabolic fire) because of its ushna veerya [hot potency], Katu [bitter] rasa and Tikta [pungent] rasa. It digests Ama (toxins) and balances tri-doshas. It balances vata due to its Ushna veerya (hot potency); Kapha due to its Rooksha (dry), Katu (bitter), and Tikta [pungent] rasa and also Pitta due to its Tikta (pungent) guna. Haridra removes the blockage in “srotas” [circulatory channels] which are created by vitiated Pitta and Kapha dosha and allows free movement of Vata. Haridra is helpful in immune-modulation by correcting metabolism, enabling free circulation of nutrients & oxygen to every cell due to its antioxidant properties. It is also “Vishothahar” i.e., it is naturally anti-inflammatory. The principle alkaloid “Curcumin” in it stops the growth of any kind of abnormal cells.

2. Ashwagandha

Ashwagandha is enriched with Katu [bitter], Tikta [pungent], Kashaya [astringent] rasa; is Snighda [oily] yet Laghu [light] and of Ushna veerya [hot potency]. Ashwagandha is Kapha & Vata hara [pacifies vitiated kapha and vata dosha]. It is also a Rasayana [improves life expectancy], Balya [improves strength], anti-inflammatory and anti-bacterial.

3. Manjishtha

Manjistha is a potent herb known for its blood purifying properties as it is a “Raktashodhak”. It is helpful in removing toxins from our blood circulation. It also works on hyperpigmentation by reducing melanin production, thus evens out the skin tone. It is a powerful antioxidant which nourishes our skin. It is Kapha and Pittashamak that balances the vitiated kapha and pitta doshas. Acharya Charaka has mentioned it as “varnya” because of its various skin benefits. It is helpful in relieving itching and promotes skin healing. This herb is rich in bioactive constituents like purpurin, munjistin, xanthopurpurin, pseudo purpurin.

4. Mandukparni

It is commonly called “Gotu-kola”. This herb is mentioned under 4 Medhya Rasayana by Acharya Charaka [Father of Medicine in Ayurveda]. This herb is rich in Tikta rasa [bitter] thus, pacifies aggravated Kapha and Pitta dosha and also improves Agni [digestive fire] by “Ama-pachana” [digestion of toxins].  This herb's dry powder is used for “Udvartana” i.e., dry powder massage which is helpful in treating obesity, improves muscle tone and complexion and also improves blood circulation. This herb is said to be “Shothhar” [anti-inflammatory], “Balya” [strengthening] and “Rasayana” [rejuvenates our immune system]. It is also helpful in relieving venous insufficiency to improve blood circulation.

Herbal Remedies For Scleroderma By Planet Ayurveda

 Planet Ayurveda provides a combination of ayurvedic herbs for the management of Scleroderma. With the use of above-mentioned herbs, planet Ayurveda has compiled the “Sclero Care Pack”. This pack includes Ashwagandha capsules, Boswellia-Curcumin, Gotu-Kola capsules, Sanjeevani vati, Gandhak Rasayan and Sihnaad guggul. All the Planet Ayurveda medicines are 100 % natural, vegetarian, additive free, chemical & preservative free and without any side effects. With ayurvedic intervention, the Scleroderma can be managed efficiently and thus, increasing the quality of life.

Product Description

1. Ashwagandha Capsules

These capsules are prepared from the standardized extract derived from the root of Ashwagandha [Withania somnifera]. Ashwagandha has a great effect on the immune system, thus helping in this auto-immune condition. It also purifies blood and reduces edema. Ashwagandha is “Balya” thus helps in strengthening of joints and relieves pain.

DOSAGE: 1 capsule twice daily with warm water.

2. Boswellia-Curcumin

These capsules are prepared from the standardized extract of Shallaki [Boswellia serrata] and Haridra [Curcuma longa]. The “Frankenstein oil” in Shallaki and the major alkaloid Curcumin present in Haridra are both potent anti-inflammatory, therefore, helpful in reducing joint pain and increasing joint mobility. The Curcumin also stops the growth of any unwanted or abnormal cells in the body, thus preventing overproduction of collagen here. 

DOSAGE:  2 capsules twice daily with warm water.

3. Gotu Kola Capsules

These capsules are prepared from the standardized extract derived from the herb Mandukaparni [Centella asiatica]. This herb is rich in Tikta rasa [bitter] thus, pacifies aggravated Kapha and Pitta dosha and also improves Agni [digestive fire] by “Ama-pachana” [digestion of toxins].  This herb improves muscle tone, and complexion. It is also helpful in relieving venous insufficiency to improve blood circulation.This herb is said to be “Shothhar” [anti-inflammatory], “Balya” [strengthening] and “Rasayana” [rejuvenates our immune system].

DOSAGE: 2 capsules twice daily with warm water.

4. Sanjivani Vati

These tablets are prepared from the standardized extract of various efficient herbs like Shunthi [Zingiber officinalis], Haritaki [Terminalia chebula], Amalaki [Emblica officinalis], Vibhitaki [Terminalia bellerica], Pippali [Piper longum], Guduchi [Tinospora cordifolia], Vidanga [Embelia ribes], Vacha [Acorus calamus], Shudha Bhallatak [Semecarpus anacardium] and Shudha Vatsnabh [Aconitum ferox]. These tablets have Kapha balancing properties, thus helpful in respiratory issues. Also it has Deepana [ignites digestive fire]; Pachana [digestive] and Jwar Ghana [antipyretic] properties. It is also well used in inflammatory conditions as it is an “Aam-pachak” [digests toxins in the body].

DOSAGE: 2 tablets twice daily with warm water.

5. Gandhak Rasayan

These tablets are classical herbomineral preparations which are prepared from the purified Sulphur which is purified after following proper Shodhana [purification] procedures with the help of Cow’s milk. Along with this standardized extract from the various herbs like Chaturjaka [Twak (Cinnamomum zeylanicum) + Ela (Elettaria cardamomum) + Patra (Cinnamomum tamala) + Nagkesar (Mesua ferrea)]; Triphala [Amlaki (Emblica officinalis) + Vibhitaki (Terminalia bellerica) + Haritaki (Terminalia chebula)]; Guduchi (Tinospora cordifolia); Shunthi (Zingiber officinalis) and Bhringraj (Eclipta alba). This preparation has anti-microbial, and rasayanic properties which helps in skin conditions like psoriasis, eczema. It helps relieve itching, redness and reduces flakiness of skin. The benefits of sulphur for skin have dated back to ancient times where bathing in sulphur rich water was recommended to alleviate any skin related issues.

DOSAGE:  2 tablets twice daily with warm water.

6. Sinhnaad Guggul

These classical herbo-mineral capsules are prepared from the standardized extract of Amalaki [Emblica officinalis], Haritki [Terminalia chebula], Vibhitaki [Terminalia chebula], Guggul [Commiphora mukul], purified Gandhak [sulphur] and Eranda Tail [Oil from the seeds of Ricinus communis]. Gandhak is purified after following proper ayurvedic Shodhana and Marana purification procedures.  This formulation is a potent Vata dosha pacifier. It is useful in management of conditions like osteoarthritis, joint pains, rheumatoid arthritis, stiffness, etc. It is an excellent anti-inflammatory.

DOSAGE: 2 tablets twice daily with warm water.

Conclusion

Scleroderma is a complex, multifaceted disease that requires a personalized and multidisciplinary approach to treatment for every patient. Early diagnosis and intervention are crucial in preventing irreversible damage to internal organs. The condition can be challenging to manage due to its progressive nature, but with appropriate medical care, lifestyle adjustments, and support, patients can often lead fulfilling lives. A holistic treatment approach, including Ayurvedic interventions, can provide complementary benefits in managing symptoms and improving overall well-being. While this disease is not curable but with the ayurvedic herbs and therapies, it can be managed easily resulting in improved quality of life of the patient.