Henoch Schonlein Purpura IGA Vasculitis Adult - Others out there?

Welcome, @junetooth. I'm tagging fellow members like @radicchi and @gelysaleigh to join this discussion about adult IgA vasculitis.

June, you may also have some knowledge to add to this related discussion:
- Damage to legs from Henoch-Schonlein purpura? Stumped the doctors: https://connect.mayoclinic.org/discussion/teenager-damage-to-legs-from-hsp-stumped-the-doctors/

How long have you been living with this condition? How is it affecting your kidneys and GI tract?

@colleenyoung
@radicchi
@gelysaleigh
@junetooth

Henoch Schonlein Purpura Purpura IGA Vasculitus Adult

Diagnosed Sept. 2021 via skin lesion biopsy sent to Mayo from hospital admission at Maine Medical Center.

Presented first as skin lesions Purpura, red dots, legs, across belly, tops of feet (extremely painful), inside of arms.

First ER hospitalization included extreme malaise, unresponsiveness/passing out during blood draw. Sick. Unwell.
Treated with iv prednisone. Discharged home.

Skin flares occur about every 21 days. I feel sick and then they start to dissipate. Purpura, bright red in the beginning. Like a child put dots on my body with a sharpie. Over progression dots have progressed in size from dots to eraser sized, to raised lesions, to purple lesions, and now full on open sores during this active flare on buttocks and thighs. ER started on 50 mg. PCP increased to 60 MG prednisone for 10 days. Currently on 40 mg. With new regimen of 21 day taper from here.

Current lesions have subsided I believe in response to prednisone.

2 weeks after my diagnosis, my 25 year old son was diagnosed with metastatic testicular cancer.

I departed ME and flew to meet him in CO for urological surgery then on to TX to MD Anderson Cancer Center.

While in Houston had 2 more hospitalizations.

Severe pain with a flare in November, 21. Then in January 21 hospitalized with GI involvement. Pain scale did not describe. Worse than childbirth. IV morphine did not lessen the pain.

ER staff treated for idiopathic GI blockage. Oral GI drink and then literally watched and felt my GI system flip and move from right side to left. I suspect GI interseption (sp). Pain dissipated after “GI” large intestine, upper abdomen, “moved” internally. Was not a stool issue, little was discharged. GI seemed crimped.

4th ER hospitalization on Aug. 25, 2022 severe aggressive skin lesions on buttocks and thighs, open wounds ranging in size from quarters to lemons. Also maroon purple sized open wounds on legs. Red dot Purpura, typical presentation, on belly and underside of arms.

Most serious new complication is kidney involvement. Blood and protein in urine. Severe back pain mostly left side. Tylenol 500-1000 mg. Mostly bed ridden since 25 Aug.

Son was declared in remission on 5 Aug 2022. Praise God after
Testicular cancer, metastatic to abdomen, spleen, back and bi lateral lungs; pulmonary embolism; pneumonia and blood clot to jugular vein - he is alive. He rang bell and they believe he is in remission.

My body is telling me I must now attend to my own health.

Am building care team in Maine. Met with nephrologist 9/7/22. He has diagnosed progression to IGA nethropathy. Nephrologist is taking over prednisone dosing. Starting me on glucometer to watch sugar levels. Advised short term diabetes likely with high dose prednisone. Prescribing antibiotic to counteract prednisone induce pneumonia. Is triaging with dermatologist who wanted to add dapsone. The two are contraindicated.

Nephrologist will triage with dermatologist today to assess which drug to start with first. Nephrologist believes advancement to chronic kidney disease is the greatest concern.

IGA Vasculitis (diagnosed Sept. 2021) has advanced to diagnosis of IGA Nethropothy as of 9/6/22.

Need quick education on IGA Nethropothy. Treatment options, what to watch for, who to add to care team.

Nephrologist is monitoring proteinuria, microscopic blood in urine, high leukocytes, high immature platelets being released into blood. Creatinine is okay but shows kidneys are not working as they should.

Building care team: Have Nephrologist (kidney monitoring, prednisone dosing, antibiotic to prevent prednisone pneumonia); dermatologist (lesions, dapson); PCP (omaprazal, high dose calcium, B3).
Adding Rheunatologist to care team on Friday.

All outpatient.

Sick and bed bound. Have struggled with extreme malaise, feel unwell, loss of appetite, lower back pain. Please share tips, suggestions, resources on IGA Nethropothy.

A family member (47 years old) was diagnosed with henoch-schonlein purpura.
Although lasts 6 weeks, he’s entering the 3rd month. Taking prednisone for 2 weeks now, eating healthy; But protein and salt values still high.
Has Zero energy (can’t work, little exercise), pain in lower back, shaky hands and voice, incredibly sensitive (sad and depressed and ultra negative)
Not sure if it is the right diagnosis.
Feeling confused. How can I help this member? Who should I search for help?

@saraga welcome to Mayo Clinic Connect. Your family member must be very uncomfortable and concerned. Has he given any thought to getting a second opinion? You can look for a large medical center or a university teaching hospital.
If he is interested in going to the Mayo Clinic, here is a link
https://MayoClinicConnect.in/1mtmR63
The Mayo Clinic is not able to see all patients, but they do have a care network of hospitals:
https://mayoclinic.org/about-mayo-clinic/care-network/members
I hope this will help

@saraga, this must be so hard to watch your family member suffer. I moved your message to this existing discussion:
- Henoch Schonlein Purpura IGA Vasculitis Adult - Others out there?: https://connect.mayoclinic.org/discussion/henoch-schonlein-purpura-iga-vasculitis-adult-others-out-there/

I did this so you can connect with other members like @radicchi @gelysaleigh @junetooth who can share their experiences.

Was your family member diagnosed by a rheumatologist? Rheumatology typically diagnoses and treats Henoch-Schonlein purpura (also known as IgA vasculitis). Is his doctor thinking he may not have HSP?

Saraga, Henoch Schonlein Purpura IGA Vasculitis in Adults is quite rare.
Was your family member diagnosed by a skin biopsy of a lesion?
What were the symptoms that manifested first? Mine was skin lesions, then GI and now kidney. Lungs can also be impacted, mine to date, are holding fine.
I want to help you as those of us who have this are few. I have resources to share.
Namely: if presented as skin lesion you need to get a biopsy taken and prepared in manner Mayo in Rochester needs it so they can diagnose one. Second, prednisone is often used and is one of the few aids. But it is a vicious drug and will cause many side effects. Shaky hands, mental/mood malabity.

What you describe sounds like the HSP IGA Vasculitis has progressed to IGA Nethropothy (sp) that is the IGA attcking the kidney.
The back pain and protein are symptoms I share.

My team includes:
Rheunatologist (auto immune), nephrologist (kidney), dermatologist (kidney), gastroenterologist (gi. My weakest link so far, still building my team), primary care doctor.

I am on a drug trial with Mayo in Florida (third patient with this disorder) to be treated in this trial. Early on the trial but my painful skin lesions have been in remission since I have been on the trial.

If your family members diagnosis is indeed HSP IGA Vasculitis. We need to connect him to Dr. Megan Sullivan, Rheumatologist (one of several Mayo team members) working my case.
Also you can reach me junetoothaker@gmail.com
Let’s try to get him to help. Or at least less impact.
June

Thank you Colleeen, Appreciate the efforts.
He was diagnosed but not by a rheumatologist.
I will follow the existing discussion.
THanks again

Hi June,
Thank you for sharing your journey and the information, particularly the team of doctors working your case. We are now with a nefrologist and he is getting a kidney biopsy in a week. The skin biobpsy had in the bast confirmed the sickness.
How long have you been going through this? Is your test treatment working for you? Prednisone is a nasty drug and we are already halving it due to its side effects. Is there an end to this?
I wish you the best of luck. Once we get results of the biopsy I will be in touch again.
Thank you

Thank you. Looking for second opinion at the moment. Will check your links. Thank you very much for sharing