ABSTRACT
It is a rare autoinflammatory disease. SAVI causes severe lung disease and interstitial lung disease. It occurs due to mutations in the STING1 gene which gives the instruction for making STING (stimulator of interferon genes) protein. It involves the abnormal inflammation in the body that is in the skin, lungs, and blood vessels. Inflammation normally occurs when the white blood cells of our body reach the site of injury to fight against bacteria and help in tissue repair. Excessive inflammation damages the tissues and cells of our body. Inflammation occurs due to autoinflammatory disease.
INTRODUCTION
SAVI is an inflammation of the body due to a mutation in the STING1 gene in the body. It starts from the first few months of life. The signs and symptoms are mostly of blood vessels and damage to tissue. As an infant suffers from SAVI the lesions occur on the face, nose, and ears. These lesions start in the form of lesions and turn into ulcers and lastly tissue damage. The skin problems mostly worsen in cold weather. Due to abnormal blood vessels in the skin, the skin turns into purplish discoloration. People affected with SAVI also experience symptoms of Raynaud phenomenon as fingers and toes turn white or blue in colour due to exposure to cold temperatures and any type of stress.
PATHOPHYSIOLOGY
When the protein content STING shifts to its normal position the SAVI disease occurs. Normally, the STING protein is present in the cytoplasm of cells but in this condition, the protein is present in the Golgi apparatus.
CAUSES
The main cause of SAVI is mutation in STING1 genes. There are some risk factors that cause SAVI are described below-
- A positive family history
- Abnormality in the cytoplasm of cells
SYMPTOMS
The SAVI includes the following symptoms
- Interstitial lung disease
- Skin lesions on fingers, cheeks, nose, toes and soles
- Myositis (when the immune system attacks the muscles of the body)
- Joint stiffness
- Rashes on skin
- Blisters on skin
- Ulcer in the mucous membrane
- Ulcers on the skin
- Abdominal pain
- Difficulty in breathing
- Swelling and inflammation in hands and feet
- Vomiting
- Diarrhea
- Fever
- Anemia
- Fatigue
DIAGNOSIS
The SAVI include the following diagnosis
- Clinical examination
- Evaluation of medical history
- Assess the signs and symptoms of patients
- Blood test- ESR (erythrocyte sedimentation rate) and C-reactive protein are elevated
- Skin biopsy- Shows the inflammation of capillaries
- Lung biopsy- Shows the follicular hyperplasia and fibrosing alveolitis
COMPLICATION
The SAVI includes the following complications
- Chronic inflammation
- Scarring of skin
- Disfigurement of skin
- Loss of mobility
- Organ damage mostly involves the lung
- Gangrene forms on the tips of the finger
TREATMENT
The treatment includes the following
- Control the inflammation
- Prevent organ damage
- Supportive care is given to reduce symptoms
- Steroids - To reduce inflammation
- Antibiotics -To prevent infections
- Immunosuppressant - To suppress the immune system
AYURVEDA ASPECT FOR STRING-ASSOCIATED VASCULOPATHY INFANCY DEPENDANT
- As Acharya Charak explained in Sharira sthana च.शा.०३ chapter खुडाक गर्भावकृान्ति शारीर (Khuddika Garbhavkranti Sharira ) explained बीज दुष्टि (defect in ovum and sperm).
- The defect in the ovum and sperm is due to 3 reasons: बीज (sperm and ova), बीज भाग (chromosomes), and बीज भाग अवयव (genes). In च.शा.०४।०४ the 7 भाव (qualities) are described by Acharya Charak that is मातृज भाव (maternal factor), पितृज भाव (paternal factor), आत्मज भाव (soul), सात्मयज भाव (wholesomeness), रसज भाव (nutritional factor), and सत्वज भाव (psych/mind). The disorder is found before the birth so considered in सहज रोग.
- As the dosha is vitiated the signs and symptoms are seen according to the severity of the disease.
HERBAL REMEDIES BY PLANET AYURVEDA FOR STRING-ASSOCIATED VASCULOPATHY INFANCY DEPENDANT
Planet Ayurveda is GMP-certified. Their products are 100% pure, easy to use and free from any type of adulterant, preservatives, fillers, yeast, and chemicals. The products are prepared under the guidance of an M.D. Ayurveda experts. The products are effective and have no side effects. The herbal remedies by planet Ayurveda for string-associated vasculopathy infancy dependant-
Product list
- Boswellia curcumin
- Arvindasav
- Syrup Infee
- Manjishtha capsules
Product Description
1. Boswellia curcumin
The Boswellia capsules contain Curcumin (Curcuma longa), and Shallaki (Boswellia serrata). Inflammation in blood vessels and lungs is associated with SAVI so curcumin helps to reduce inflammation as it has anti-inflammatory properties. Curcumin also reduces muscle weakness, and muscle pain and protects from muscle damage. As skin discoloration and ulcers form on the skin, the shallaki helps to reduce those symptoms.
Dosage - 1 capsule twice daily, with plain water, after meals
2. Arvindasav
The Arvindasav has a mixture of many herbs as it contains Bach (Acorus calamus), Kachoor (Curcuma zedoaria), Amla (Embilica officinalis), Sarva (Hemidesmus indicus), Sarfonka (Tephrosia), water and many more herbs. As a mental weakness, jatamansi helps to improve mental weakness. As skin discoloration is there amla improves skin complexion. As Arjuna has antioxidant properties it prevents the formation of free radicals that cause skin damage. Munakka has anti-microbial and anti-inflammatory properties; it reduces bacterial infections and also reduces inflammation.
Dosage - 2 teaspoons daily after meals
3. Syrup Infee
The syrup contains extract of Yashtimadhu (Glycyrrhiza glabra) and Guduchi (Tinospora cordifolia). Yashtimadhu helps to improve the degeneration process, and skin-related problems, and reduces weakness or fatigue of the body. Guduchi has the property of immunomodulator which helps to improve the immune system of the body.
Dosage - 2 teaspoons twice daily with water
4. Manjishtha capsules
The capsules are made up of a single extraction of Manjistha (Rubia cordifolia).Skin discoloration associated with SAVI Manjistha helps the skin for its natural color, it also reduces the ulcers and blisters present on the skin. Manjistha contains madhura, tikta, and kshaya rasa so pacifying the pitta dosha and helpful in reducing fever. It contains astringent properties so reduces mobility and is helpful to treat diarrhoea. It promotes skin glow in both ways, internally and externally.
Dosage - 1 capsule twice daily, with plain water, after meals.
Contact Planet Ayurveda Support Team to provide you the costing/ordering and delivery information at - [email protected] or Call at 0172-521-4040 (India), +91-172-521-4040 (Outside India) or Whatsapp at (+91) 842-749-4030.
CONCLUSION
In this article, we talk about the introduction, causes, symptoms, diagnosis, and treatment of string-associated vasculopathy infancy dependent. As the doshas are imbalanced there is an imbalance in the functioning of the body. In Ayurveda च.शा.०३ chapter खुडाक गर्भावकृान्ति शारीर tell about the बीज दुष्टि. Where defect in ovum and sperm at the time of fusion of gametes. There are three main reasons for this defect in the ovum and sperm, genes, and chromosomes. As this disease is hereditary it is considered in सहज रोग. The planet Ayurveda provides herbal remedies to treat the string associated with vasculopathy infancy dependent Boswellia curcumin, Arvindasav, Syrup Infee, and Manjishtha capsules.
