Immunoglobulin A Vasculitis (IgA, Henoch Schonlein Purpura ) is the childhood vasculitis. It can occur in any age and peaks around 4-6 yrs old. It is a non thrombocytopenic, small vessel vasculitis that typically presents acutely . In childhood onset disease 90 % of cases occur under the age of 10 years and often seen in boys.

Taking about this disease it is very little understood about the exact pathophysiology of this condition. It is the condition that involves swelling (inflamation) of small blood vessels. The swollen blood vessels leak into the skin, joints, intestines and kidneys. It is regarded as an autoimmune disorder. This is where the body’s immune system attacks the body’s own cells and organs. With HSP this immune response may be caused by an upper respiratory tract infection and other immune triggers may include an allergic reaction, medicine, injury or being out in cold weather.

Sign/ Symptoms

Each child’s symptoms can vary, Common symptoms are –

• Severe abdominal pain (sdiffuse and colicky)
• Jointpain and swelling (arthritis or athralgia)
• A rash caused by blood leaking into the skin commonly called as Purpura
• Swollen lower Extremities, Skin 
• Renal involvement (proteinuria >0.3g in 24 hrs, hematuria
• Severe Nausea / Vomiting
• Bleeding in the digestive tract which includes mouth, esophagus, stomach and intestine 

It can be further diagnosed by Biopsy, Blood and urine test,  Ultrasound, palpable purpura

Treatment and Management

In regards of the treatment till date there is no form of therapy has been found to shorten the duration of IGA Vasculitis to any significant degree. Therefore treatment remains primarily supportive in most cases. This is consonant with the understanding that HSP is self limited disease. The majority of patient recover within several weeks (6-8 ) whereas some patient may take several months to year inorder to recover.

There is only supportive treatment for this disease which include Hydration, acetaminophen, NSAID for joint pain and fever. However NSAID drugs should be avoided in the presence of GI or Renal manifestation as they have been shown to aggravate these symptoms . However Corticosteroid have been suggested owing to their immunosuppressive properties and their usefulness in treating other childhood vasculitis.

Pediatric therapies for established severe HSP Nephritis includes Corticosteroids, immunosuppressant, angiotensin-converting enzyme inhibitors, plasma exchange.

Complication

37 % cases showed renal involvement and among them 1% cases result in End Stage Renal Disease. 66% of children may experience GI symptoms such as abdominal pain, 22%- intestinal Bleeding, intersusception <3%.

Recurrent HSP

HSP might reoccur upto 33% of affected children . Recurrences appear to be more common in older children >8 yrs of age at onset and in children with renal involvement

My Story 

My younger kid (daughter) was diagnosed with HSP in Feb 25 2022. Back then my family had just arrived in United States in November-2022. I am a Registered Nurse in USA. I was there since 6 months. My daughter had never been admitted in a hospital back in Nepal. She was a healthy kid.

 Actually in the initial stage my daughter showed the sign of knee pain, joint pain. We thought that she might have  got hurt in school while playing; so my husband took her to urgent care for X-Ray. No any abnormalities found. Back then I used to live in different city due to my work  and my husband used to live with his family in Fortworth- Texas. After few days she had vomiting we thought that it was due to food poisoning so we thought it would get better after few days but it reoccurred again with severe abdominal pain. We had doubt that she might have appendicitis, so we decided to take her to the hospital. I had 15 days off inorder to spend some time with my family as I was away from them. So my brother in law drove the car and we went to Cooks Children Hospital – fortworth which was 45 minutes drive from the place we lived. At that time I noticed that my daughter had few dots on her legs which was not that noticeable. So I showed that dots to the doctor after looking he told that it might be HSP, however they were not sure yet. So they did check Blood test, urine test, ultrasound etc and hence after the test and study they were sure it was HSP. So they admitted her. They gave her NS drip for hydration and mild analgesic –acetaminophen (Tylenol). She was admitted for 5 days for the observation.

 The doctors didn’t had definitive answer for its treatment and the prognosis of the disease was unknown. As parents we were devastated at that moment when our healthy kid was diagnosed with some kind of autoimmune disease which don’t have definitive treatment and  time period to be healed. It might take several weeks to months to year to heal and there is also chance of recurrence and it might also affect kidneys. We were terrified by the situation. It was heart wrenching moment for us. No parent would like to see your kid suffer so much. She had flareup every 4-5 days. She used to have severe abdominal pain, vomiting and her rashes started to be prominent, big specially in her lower extremities and her buttocks. She used to cry whole night because of the pain we as a parent used to carry her inorder to comfort her, gave her Tylenol for pain 4 times a day according to doctors prescription but it didn’t help her. Her extremities were swelled up. As a mother I couldn’t control my tears looking the condition of my kid. I think it was the hardest phase of my life. So we had to take her hospital and admit her every couple of days. We were tired and exhausted. Doctors told that it was the severe condition of HSP so they started with Steroid (prednisone) for several weeks, still she had the flare ups then they started IVIG drip still no improvement. We had to take her back and forth to Hospital from home. Same routine. Doctors then gave her immunosuppressant drug cellcept for couple of weeks and they told us that it is a rare case they have seen because usually most kids get healed after steroid but in our daughter’s case it didn’t work. They also made aware that if cellcept didn’t work they have only  had last option that would be chemo therapy. We were shattered at that moment. We didn’t know what to do ? I had lost hope but when I used to  see my daughter’s face  fighting  with this disease in a hope of getting cured it gave me immense  courage. By Gods grace my daughter slowly started to recover. Cellcept medicine worked. Thank God she didn’t had to go through chemo session. What a sigh of relief. Slowly her symptoms subsized. She had continous follow up with Rhemutologist and nephrologist doctors till now. 

The only motive of this article is to create awareness amongst people. Not many people know about HSP because it is quite rare. I would like to raise awareness by sharing my experience to you all. I would be always thankful to the doctors and nurses and all the team of Cooks Children Hospital as well as my families and friends who helped us to overcome this difficult situation.

(Writer Baral is regestered nurse in US)