STING ASSOCIATED VASCULOPATHY WITH ONSET IN INFANCY (SAVI)
Abstract
SAVI is an abnormal inflammation and a rare condition in which there is a lesion in the interstitial lung disease and skin. In 2014, it was first discovered. It is a hereditary disorder because it is caused by gene mutation. It typically manifests in neonates and has a high mortality rate. In this article, we will read about the sting associated with vasculopathy with onset in infancy in brief.
Introduction
Sting associated vasculopathy with onset in infancy is a genetic condition of abnormal inflammation to the whole body mostly occurring in lungs, blood vessels and skin. It is a rare condition and auto-inflammatory disorder. It starts in infancy. It is mainly characterized by interstitial lung disease and vasculopathy. The Raynaud phenomenon is experienced by the affected person, in which the affected area changes the color white to blue and turns red to exposure with stress and cold. It may be further needed for amputation.
Causes
SAVI is caused by genetic mutations. STING 1 gene mutation takes place which results in excess production of the inflammatory proteins that cause tissue damage and excessive inflammation. This gene is important for producing STING protein which produces cytokines (Beta-interferon) that cause inflammation.
Symptoms
- Low grade fever
- Non healing ulcer
- Joint stiffness
- Muscle inflammation (Myositis)
- Dyspnea
- Swollen lymph node
- Pulmonary lesions
- Failure to thrive
- Coughing
- Hypermobility
- Interstitial lung disorder
- Skin lesions present on toes, soles, nose, ears, fingers and cheek
- Lung fibrosis
Diagnosis
- Magnetic resonance imaging
- Chest X-rays
- Blood culture
- CT scan
- Lung biopsy
- Genetic testing
Treatment
There is no specific treatment for this disease. Only supportive management can be given.
- Corticosteroids
- Biologic agents
- Medications:- JAK inhibitors
Ayurvedic overview
According to shloka given in charaka sharira sthana chapter 3/17:-
This theory holds good for each and every channel (Srotas). Whichever avayava (Gene) of beeja bhaga is impaired by the doshas (Vata, pitta and kapha) the respective gene presents with an illness or disease.
In Ayurveda, we can correlate the sting associated with vasculopathy with onset in infancy with Bija dushti (Abnormal ovum and sperm). It is categorized under hereditary disease (Sahaja or Kulaj roga. Bija bhaga avayava (Gene) is the molecular unit of heredity of a living organism. Genes hold the information to build and maintain an organism’s cells and pass genetic traits to offspring. If both female and male have bija impaired completely then they are unable to produce new progeny but if only get contaminated they will deliver a defective progeny. Abnormal doshas (Vata, Pitta and Kapha) enter the bija bhaga avayava resulting in the development of disease in that area and develop symptoms like dyspnea, growth retardation, skin lesions, joint stiffness and pulmonary lesions.
Treatment
Hereditary or genetic diseases are not curable. There is no treatment available for this disease. We can give only supportive treatment to delay the progression of disease and manage the symptoms. Planet Ayurveda gives some herbal remedies for SAVI for supportive management which are given below.
Herbal remedies for SAVI by Planet Ayurveda
Planet Ayurveda provides various types of herbal remedies for various diseases. Planet Ayurveda follows strictly all the principles given in the ancient Ayurvedic Samhita. These ayurvedic medicines are GMP certified and are safe to consume. These medicines are very effective in nature and have no side effects. These are free from chemicals, yeast, additives and other preservatives. Planet Ayurveda provides herbal remedies for the management of the SAVI which are given below:-
- Boswellia+Curcumin
- Arvindasav
- Infee syrup
- Manjishtha capsule
Boswellia+Curcumin
It is a poly herbal capsule containing shallaki (Boswellia serrata) and haridra (Curcuma longa). Its taste is bitter and hot in effect. It balances all the three doshas in the body-Vata, Pitta and Kapha dosha. It is sharp, unctuous, sticky, dry and light. It contains organic compounds and inorganic ions and also has mineral matter that includes calcium, silicon dioxide, iron, volatile oil, and magnesium. It is a very effective medicine for various types of joint problems such as osteo-arthritis, gout and rheumatoid arthritis. It helps in reducing pain, tenderness and swelling of the inflamed joints. It is also known to stimulate expectoration, immunostimulant and that which promotes menstrual discharge.
Dose:- 1 Capsule twice daily
Arvindasav
It is a classical medicine described in ancient textbook containing majith (Rubia cordifolia), kherati (Sida cordifolia), mulethi (Glycyrrhiza glabra), arjun (Terminalia arjuna), etc. It is sweet in taste, unctuous, heavy and cold in effect. It pacifies all the three dosha (Vata, pitta and kapha). It is an antipyretic, anti-inflammatory and wound healer medicine. It improves the complexion, relieves problems of the eyes, an antacid agent and is also an effective general tonic. It contains calcium, glucose, starch, vitamin B and potassium. It liquifies the mucus and helps in its smooth excitation. It is also indicated in cough, asthma, bronchitis and other chest problems. It improves the voice, promotes body weight, inhibits fatigue and excessive thirst. It acts as a digestive stimulant. It is very good for skin and helps to gain a shining and natural glow to the skin.
Dose:- 2 Tsp twice daily
Infee syrup
It is a herbal syrup containing yashtimadhu (Glycyrrhiza glabra) and guduchi (Tinospora cordifolia). It balances vitiated doshas that are Vata, pitta and kapha. It is also an antipyretic, anti-inflammatory and a good wound healer. It is astringent and bitter in taste and hot, unctuous and heavy in effect. It contains fatty acids, alkaloids, volatile oils and glycocides. It is indicated in rheumatoid arthritis, fever, dyspepsia, gout and urinary diseases. It has antioxidant, immuno-modulator and rejuvenating properties. It lowers uric acid level.
Dose:- 2 Tsp twice daily
Manjishtha capsule
It is a single herbal capsule consisting of manjishtha (Rubia cordifolia). It is a blood purifier, detoxifier, a booster of the hemopoietic system and improves the complexion. It is also an astringent, antiseptic, hemostatic agent, digestive and carminative. It has anti-inflammatory and uterine stimulant properties. It is at the same time bitter, astringent and sweet in taste and hot, dry and heavy in effect but it is a pacifier of kapha and pitta dosha. It treats various diseases like raised uric acid, gouty arthritis, recurrent skin infections and glandular swelling. It is indicated in diarrhea, chronic fever, dysentery and urinary and uterine infections. It removes pimples, acne, blemishes, and provides scar free skin. It also promotes the healing of the skin tissues damaged by infection or injury.
Dose:- 1 Capsule twice daily
Conclusion
In this article, we read about the SAVI which is a genetic disorder in which abnormal inflammation takes place, caused by mutation in genes. In Ayurveda, we can correlate it with Bija dusti or sahaj or kulaj vyadhi that means hereditary disorder. Hereditary diseases are not curable and have no treatment but we can give supportive management to overcome the symptoms and hamper the condition. Herbal medicines like infee syrup, arvindasav, manjishtha capsule etc are very effective in this condition.
Dr. Vikram Chauhan
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