ABSTRACT
SAVI is a disorder or vasculopathy which begins during the infant period. It is a very rare inflammatory disease when there is inflammation throughout the body especially in skin, blood vessels and lungs. It manifests as systemic inflammation, interstitial lung disease and cutaneous infection. Patients present with recurrent cough, failure to thrive, skin scaling, poly arthritis, interstitial lung disease, and necrosis and auto amputation. SAVI is type1 interferon disorder caused due to mutations in STING1 stimulator of interferon gene protein. Patients carry heterogeneous substitutions in STING1. It is a life threatening condition because there is a gain of function mutations in STING1. Sting regulates various pathways such as functioning of proteins. Lung involvement leads to morbidity and mortality in sting associated vasculopathy. Chest CT and lung biopsy are the measures to diagnose this disorder during infancy. As it is a genetic disorder there is risk of another child being born with a similar problem. In this article we will discuss STING associated with vasculopathy of infancy, its cause, symptoms, diagnosis and its treatment according to classical Ayurveda texts.
INTRODUCTION
SAVI is a genetic disorder which begins in neonates or infants causing systemic illness and vasculopathy of small vessels. Due to excessive inflammation in the body it leads to destruction of the body's own cells and tissues. It is also known as auto inflammatory disease. Most patients have problems with blood vessels and tissues which affects blood circulation. There is particular damage to skin tissues of the face, nose, toes, fingers, etc. Skin lesions appear which develop into wounds. Skin problems may worsen during cold weather and cause skin discoloration. Sometimes it may involve the respiratory system which can be a life threatening condition (affects breathing pattern). The patient may have intermittent low grade fever and may affect lymph nodes. There is quite a therapeutic limitation of sting associated vasculopathy of infancy as supportive treatment can be done. The severity of disease depends on the number of gene mutations taking place. There is association of interferon genes protein characterized by skin and lung symptoms. There is over activation of type1 interferon pathway. STING is a transmembrane protein which plays an essential role to innate immunity against viral and bacterial pathogens.
CAUSES
As we know SAVI is a genetic disorder which onset during infancy and there is disturbance in one or more genes. It can be inherited disorder like autosomal dominant inheritance. There is a mutation in the STING1 gene. This gene helps in functioning of proteins and immune system responses. In this disorder beta interferons are produced which is a kind of protein that promotes inflammation. This inflammatory change leads to tissue damage and leads to development of signs and symptoms.
SYMPTOMS
The sign and symptom of this disease starts appearing during infancy. By growing age, symptoms may differ and they appear at any age. Some of symptoms of SAVI are as following
- Skin lesions
- Ulcers, necrosis (non- healing)
- Skin discoloration ( purplish)
- Low grade fever
- Swollen lymph nodes
- Finger/toe tips white or bluish
- Interstitial lung disease
- Pulmonary fibrosis
- Difficulty in breathing
- Joint stiffness
- Muscle inflammation
- Cardiovascular involvement
- Esophageal reflux
- Tachypnea
- Anemic syndrome
DIAGNOSIS
The diagnosis for SAVI are as following
- Clinical examination
- Medical history
- In Ayurveda darshan, prashan and sparshan is done. (inspection, history and examination)
- CBC (Hb- decreased, ESR- raised)
- Antenatal and postnatal examination
- Genetic metabolic screening/ sequencing
- Imaging like chest x- ray
- Biopsy
- Gene testing
- Duplication analysis
MANAGEMENT
As we know it is a genetic disorder, no specific treatment is available. SAVI is a very challenging condition for managing so supportive treatment is done. Some of the measures are given as following
- Corticosteroids
- Immunosuppressant
- Biologic agents
- Somatic gene therapy
AYURVEDIC POINT OF VIEW REGARDING STING ASSOCIATED VASCULOPATHY IN INFANCY (SAVI)
Ayurveda is a traditional system of medicine which believes in natural herbs (aushadh), lifestyle activities (vihar) and dietary changes (aahar). In classics there is no particular correlation with sting associated vasculopathy of infancy but according to symptoms it can be related according to dosha predominance such as there is predominance of vata and kapha dosha. According to Acharya kashyap in vednaadhya (chapter related to different kinds of disorders in children) it can be related to its signs and symptoms. So, as Ayurveda believes in dosh, dhatu and mala this disease can be treated accordingly.
HERBAL REMEDIES FOR STING ASSOCIATED VASCULOPATHY OF INFANCY BY PLANET AYURVEDA
Planet Ayurveda is a well authorized and GMP, ISO certified manufacturing company. It has been spread throughout the world due to its purity in natural and herbal products. All the formulations prepared here are as discussed in our classical texts and under expert supervision. Products manufactured here are free from any preservative, color, starch, yeast, etc. The best combination of herbal medicines for Sting Associated Vasculopathy of Infancy (SAVI) by Planet Ayurveda are as following
Product List
- Boswellia Curcumin
- Arvindasav
- Syrup Infee
- Manjishtha Capsules
PRODUCT DESCRIPTION
Boswellia curcumin
It is a standardized capsule formulation prepared from the extract of shallaki (Boswellia serrata), haldi (Curcuma longa). These capsules have anti-inflammatory properties and reduce all types of pain. It has antioxidant properties and cures degenerative disorders. It plays an important role in the respiratory system such as in asthma.
Dosage- One capsule twice daily with plain water after meals.
Arvindasav
It is a syrup formulation from our ancient classical texts prepared from extract of kamal phool (Nelumbium speciosum), khus (Vetiveria zizanioides), gambhari (Gmelina arborea), etc. It promotes proper growth and development of children. It increases appetite, digestion and corrects recurrent respiratory infections. It corrects delayed milestones and rickets problems in infants. This syrup improves appetite, strength in children.
Dosage- Two tablespoons mixed with equal amounts of water taken twice daily after meals.
Syrup Infee
This is a syrup formulation prepared from extract of yastimadhu (Glycyrrhiza glabra), guduchi (Tinospora cordifolia), bibhitaki (Terminalia Billerica), etc. It acts as an immunomodulator and protects children from repeated infections. It boosts child immunity and helps ingrowth of the child. It protects children from recurrent respiratory and GI infections. It helps to strengthen weak children and children suffering from cough and bronchitis.
Dosage- Two tablespoons twice daily with plain water after meals.
Manjishtha capsules
It is standardized capsule formulation prepared from extract of manjishtha (Rubia cordifolia). It acts as a blood purifier, anti platelet, anti stress and calcium channel blocker. It is used to treat skin problems (skin rejuvenating, lightens if any spots) and has anticancer properties.
Dosage- One 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 0172-521-4030 (India), +91-172-521-4030 (Outside India) or Whatsapp at (+91) 9915-593-604Conclusion
Sting associated vasculopathy is a very rare, genetic auto inflammatory disease which begins during the infant period. This disorder can affect a child's life as it involves tissue damage of skin, vessels and pulmonary system. Here Planet Ayurveda gives the best combination of medicines for SAVI as they are prepared under expert's supervision. For any severity you may concern the physician and team of planet Ayurveda is always there for any type of guidance.
