14 years old girl is admitted due to persistent microscopic hematuria and proteinuria. She is already diagnosed with Crohn disease. Due to she receive Imuran 2x50 mg, and Humira 40 mg. Last week were performed tru cut renal biopsy witch shows signs of Ig A nephropathy and 1 from 7 glomeruluses were sclerotic. There aren't any other pathological signs, girl is with normal BP and renal function.
What are the treatment possibilities for nephropathy and should we treat it?
IgA nephropathy (Berger's Disease) is the most common cause of primary glomerulonephritis. It often remains asymptomatic. In this case, your patient is 14 years old, and is exhibiting hematuria - the most common, gross sign of IgA nephropathy. Your ultimate goal is to save this child's kidneys! And to prevent her from one day needing dialysis, or in worst case scenario, a kidney transplant. Your patient currently maintains a normal blood pressure and normal renal function - which indicates a decent prognosis. Often, patients with IgA nephropathy are treated with anti-hypertensive drugs; however, this is not (yet) needed in this case given this child has a blood pressure within a normal systolic/diastolic range. Note: It has been reported that some patients with IgA nephropathy (of all ages, both children and adults), have entered a state of remission, while others' conditions worsen in severity 15-20 years after manifestation. One suggestion, is omega-3-fatty acids. Why? Because it is known to decrease inflammation of the glomeruli. Other treatments include drugs that help lower cholesterol levels, as it may slow the progression of kidney damage - which is what this disease is notorious for. Now, I highly suspect that your patient was suffering from an infection (possibly respiratory), or a cold, when her hematuria began. To simplify, the IgA is depositing and accumulating in excessive amounts in this child's kidneys, which causes the hematuria and proteinuria. This child's blood pressure should be monitored constantly. I would also suggest, that any sign of swelling in your patient's extremities (hands and feet), fevers, frequent and recurring respiratory tract infections, be noted, as these are further complications and cannot be ignored. Now, this child has Crohn's, which I suspect, predisposed her to the development of the IgA nephropathy, as most inflammatory GI conditions do. One suggestion is to, (if she has not already), make some alterations to her diet, in an attempt to lessen the severity of her symptoms. Perhaps implementing a low-fiber, low-sodium diet would be beneficial. Given this patient is 14 years of age, it can be assumed that her menstrual cycle has commenced - which causes cramps and discomfort. Often, NSAID's (non-steroidal anti-inflammatory drugs) are taken to alleviate those cramps and discomfort; however, given she has Crohn's disease, she should be advised not to take them, as they may worsen her symptoms. Also, this child's blood should be monitored also, as many patients with Crohn's develop anemia. Perhaps iron and B12 supplements should be implemented in her diet, as they help prevent anemia. (Unless, she has any form of abnormal absorption). Best of luck!
Athena, thank you for your detailed answer. I don't think that our patient has respiratory tract infection. Her CD are enough to ensure antigen passage trough gut mucosa. The main question is whether we treat Ig glomerulonephritis (most of us think we must treat) and a second side of our treatment dilemma is should we change adalimumab to steroids. But this will be very difficult due to excellent therapeutic answer of CD to biologic.
Since the child has no further complications of IgA nephropathy, I would wait and carry out a specific treatment only as part of a controlled trial.
Thank you Dr Evers. What is your recommendation about exact timing to start treatment?
It depends on signs of progressive disease as heavy proteinuria, declining creatinine clearance, and/or hypertension.
For the nephropathy part, Josef and Athena's answers are good. But what about Crohn,s disease? Does it really need Imuran and Humira according to its severity and if so can the doses be reduced considering her renal disease which can be aggravated from a stable position by both these drugs. I would first consider other alternatives like Budesonide and Pentasa which has low gut absorption and in case of severe disease start Imuran in lower dose after discussing the matter with patient and her relatives.
treatment of Chron disease and kidney protection: If the patient is proteinuric or hypertensive treatment of the IgA GN shoul be with renin-angiotensin inhibition. If he only presents hematuria, follow-up for the onset of those markers. If there is increase of serum creatinine renal or proteinuria in nephrotic range, biopsy will dictate other more agressive treatments
Gautam Ray, girl receive adalimumab + Imuran due to refractory perianal disease. Now she show good clinical remission but with renal impairment.
Dr.Ivan, this is exactly what happens. The worse part is that adalimumab and AZA once started has to be continued to maintain the benefit which further the chance of kidney impairment. You must continue treatment in consulation with a nephrologist.
Dr. Ray, that is what we have done, but there are two opinions according to nephrologist consultations (two different independent consultants) - that girl is with preserved renal function (normal BP, normal creatinine clearance), so we can wait and just follow up her, and the second point of view is that sclerotic glomerulus in renal biopsy shows beginning of renal complication and we should treat.
Dr.Yankov, being a gastroenterologist, I can only advise to keep in touch with them at close intervals and monitor your patient very closely.
It makes no sense to treat 1 slerotic glomerulus of 7 as a slerotic glomerulus is irreversibly damaged and can not be cured by specific therapy.
I insist in that the decision should be based on the onset of either proteinuria, hypertension or increase in creatinine level
The commonest form of renal involvement in Crohn's Disease is Amyloidosis secondary to chronic inflammation. IgA nephropathy is unusual as a renal manifestation of Crohn's Disease. Therefore differentials for renal involvement in this patient should also include IgA dominant PIGN (especially in the context of hematuria) as well as secondary amyloidosis. An electron microscopic examination of renal biopsy is recommended for accurate diagnosis and further management should be based on the EM report
Meanwhile, there is a paper published in the NEJM which should be available here.
http://www.nejm.org/doi/pdf/10.1056/NEJMoa1415463
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