My daughter Emma is 4 and had a biopsy last November after months of spilling large protein and mild swelling. We've been trying to get a clear diagnosis ever since.
Her biopsy IF showed IgA (3-4+), IgM(3+), IgG(1+) & C3(2+), only 1 antibody short of a "full house" which usually means Lupus Nephritis. There were also "multiple segmental sclerotic lesions" under microscope and "numerous large electron dense deposits, mainly in paramesangial location".
The pathologist listed ~IgA nephropathy with focal-segmental glomerularsclerosis~ as the probable dx's because of the location of the damage and the lack of C1q. But Focal Sclerosing Lupus Nephritis is listed as the "other diagnostic consideration".
Emma has symptoms of Lupus and is ANA+, (she's been seeing a rheumatologist for arthritis, fevers and rashes since her 2nd birthday), so it's very hard for me to believe that her kidney disease is IgA N or FSGS. It doesn't make sense, but her rheumatologist refuses to make the Lupus diagnosis because of her age and stable labs. None of her doctors have ever seen a child this young with SLE or LN.
They have also discussed the possibility of Henoch-Schönlein purpura, but that seems less likely.
Do these biopsy results sound like anything anyone else has seen with IgA/IgM Nephropathy? Lupus Neph? Anything else?
Thanks in advance for any help anyone has!!
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A VERY confusing biopsy report... dx's ?? Help please!
- By Emmas_mom
- Posted July 1, 2009 at 6:42 pm · 2 replies
- In Children with nephrotic syndrome
- Shared with the public
Explore topics in this discussion:
Celiac disease Nephritis Arthritis Crohn's disease IgA nephropathy Allergies Diabetes Rashes Lupus Prednisone Enalapril Methotrexate
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- By smraftery
- Posted July 2, 2009 at 9:04 am
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Hi Emmas Mom -
I am also the mother of an Emma who was diagnosed with FSGS when she was 3...Emma is 5 now.
Her biopsy had IgA, IgG, IgM, C1, C3, C1q, and i believe something else that i cannot remember at this moment. Emma had a GI bug twice in about 3 months, and the doctors feel that this "virus" attacked her kidneys unfortunately. Because she had sclerosing on several of her glomeruli (they got 12 from 1 million), they feel it is FSGS. We live in Boston, and one RenalPathologist gave several possiblities, one of which was FSGS, and IgA nephropathy, and Henoch Schonlein Purpura....I work at a another Boston Hospital and had a world renowned RenalPathologist review Emma's biopsy slides, and he actually feels she has Collapsing Glomerulopathy - not seen in children, but in many adults.
The major thing to remember is this: Whatever the disease MAY be is one thing, but the treatments do not change regardless. The last R.Pathologist told me that it would all depend on how Emma responds to her steroids in how severe her case is.
The one thing i DID make sure was done for Emma was I took her to many specialists to Rule OUT any underlying issues...so we ruled out Celiac Disease, and any Connective Tissue Diseases, Lupus, and Arthritis. It is important to do that, because if you could treat that problem you would eliminate the nephritis. There is also data to link allergies, and tonsilitis believe it or not.
My Emma is doing well, and currently takes prednisone 5mg every other day, and enalapril twice a day. I will check her again formally next week, and we pray we can lower the prednisone again.
Hang in there...I know where you are and what you are going through.
PS, i also sent Emmas information to CHOP in Philadelphia to a specialist there for another opinion - that was after seeing 4 doctors in Boston.
I wanted to make sure no stone was unturned, and all opinions were gathered before I would put her on such medications.
I wish you and your little Emma the best.
Siobhan
- By Emmas_mom
- Posted July 2, 2009 at 10:41 am
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Thank you so much for your reply Soibhan!
We have not started Emma on pred because she has type 1 diabetes and has been insulin dependent since infancy. It complicates everything significantly.
She is doing well on ace inhibitors for now, and only spilling a trace of protein at this time, but I know that doesn't change the underlying disease.
With my Emma we have ruled out celiac, (for now), but she is antibody positive for Crohn's disease and has JRA. We were treating her arthritis with methotrexate and high doses of NSAIDs when her kidney problems started, so she has been taken of those meds.
I feel like the doctors are just waiting for things to get bad enough to justify the steroids in her case, but I'm not sure what the best option is. I definitely need to seek another opinion from a pedi neph and I would love to send her slides to another pathologist as well.
We are seeing a pedi lupus specialist in Houston next month, so maybe that will help clear up some of the questions about the cause.
I will read about Collapsing Glomerulopathy.
Thanks again!
Amy
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