Suggest Treatment For Wegener's Granulomatosis

Brief Answer:
Very likely to be adrenal insufficiancy

Detailed Answer:
Hi Dear

Firstly thanks for choosing HCM for you queries
I have noted all your details
As mentioned by you , you are on steroid 5 mg prednisolone daily.
You are worried about the severe fluctuations of blood pressure.,and you are keen to know the modality which can be tried to control it;But before moving on to treatment let me first explain you the cause of this fluctuating blood pressure.
Approximately ,75 percent of individuals with Wegener’s granulomatosis eventually develop kidney disease.Affected individuals may develop high blood pressure (hypertension).The exact cause of Wegener’s granulomatosis is unknown,hence its very difficult for a physcian to control the symptoms as cause is not known.
This explain your high blood pressure ,but does not explain why it fluctuate so much?
Now lets see why it fluctuates in your case so much;

Our kidney on top of them have to small glands called ADRENAL , these glands are responsible for secretion of hormones named ALDOSTERONE ,CORTISOL etc.

Aldosterone along with Cortisol affects the body's ability to regulate blood pressure.It plays a pivotal role in maintaining blood pressure constant through out the day, It sends signal to organs, like the kidney and colon, that can increase the amount of sodium(sodium retains water and can increase blood pressure if there is a drop in blood pressure) the body sends into the bloodstream or the amount of potassium released in the urine.Aldosterone is closely linked to two other hormones: renin and angiotensin, which create the renin-angiotensin-aldosterone system. This system is activated when the body experiences a decrease in blood flow to the kidneys, such as after a drop in blood pressure,and maintains the blood pressure to normal again.

This was a brief explaination for how the system works.

Adrenal supression is one of the most common adverse effect of long term steroid therapy,your adrenal glands naturally produce steroidal hormones like cortisol etc in small quantities daily,but when you start taking external steroids for long term,our brain fails to understand the difference between the externally provided steroids and naturally synthesized steroids,in some of the cases Adrenal gland stop producing the hormones totally, This phenomenon is called Fatigued Adrenals or Adrenal insufficiency.

As you are on steroid the hormone production is either suppresed or is not produced at all, therefore your body finds it difficult to maintain the blood pressure and there are severe fluctuations through out the day.

Long term steroid therapy should always be taken under supervision of a endocrinologist.
My suggestion to you is to get a through check up under a ENDOCRINOLOGIST.
He would prescribe you
A) ACTH(hormone secreted by pituitary that stimulate adrenal gland to produce hormone) stimulation test- commonly used test for diagnosing adrenal insufficiency.You would be given an intravenous injection of synthetic ACTH, and samples of blood, urine, or both are taken before and after the injection. The cortisol levels in the blood and urine samples are measured in a lab.

B)CRH stimulation test-When the response to the ACTH test is abnormal, a CRH stimulation test can help determine the cause of adrenal insufficiency. In this you would be given an IV injection of synthetic CRH, and blood is taken before and 30, 60, 90, and 120 minutes after the injection. The cortisol levels in the blood samples are measured in a lab.

C) serum renin and aldosterone levels will be done.

D) A 2d echo should be done to see the effect of high blood pressures on heart, severe hypertension can lead to left ventricle hypertrophy , coroary artery disease etc.

What i am trying to explain you is that if level of these hormones are not normal in your body ,because of long term steroid therapy,it will be very dificult to control b.p through anti hypertensive therapy only.

So my advice is to find out the root cause of labile blood pressure and treat it.

As per your query regarding anti hypertensive that can be tried , i would say that you are already on betablockers,calcium channel blockers and ace inhibitors.
Diuretics can,t be tried as you have CKD.
He can consider adding nitrates, Nitrates are venodilater ,and reduces the blood pressure with in minutes of being administered,usually indicated in angina patients but are very effective in refractory hypertension.Long acting nitrates can be given for overall control of B.P, while short acting nitrates can be used when B.P suddenly shoots up.
Nifedipine is a short acting drug ,you can switch over to amlodipine which are long acting and are much safer., you have not mentioned the dose of metoprolol but they can be given in a dose as high as 100 mg once daily .As you have already tried tried most of the antihypertensives , the only option left is to increase their doses.
Salt intake should be restricted, a diet containing maximum 1500 mg of sodium per day should be taken,its very essential to stick to a restricted salt diet.
Avoid alcohol.
Salt restriction should strictly be followed.
Avoid oily food .
Try to exercise 45 minutes every day.
Try to avoid egg yolks ,heavy proteins,instead try to eat a lot of vegetable,fibres and fruits.

But at last i would again say the same thing , get a thorough checkup done by a endocrinologist.
Once we find out the cause of labile blood pressure we can easily treat it, and according to me it will come out to be adrenal insufficiency in your case.

If you have any other query ,feel free to ask ,i would be happy to help you!
In case you need more details feel free to ask!

Thank you,
Dr Rishu Saxena
Cardiologist and Physician

---

Brief Answer:
Firstly get your tests done,yes you have to follow DASH diet.

Detailed Answer:
Hi Again,

See if you are stable on prednisolone 5mg and all the tests which i have suggested comes up normal;then you can continue with prednisolone, But in case the tests are suggestive of adrenal crisis ;in that case you can switch over to methotrexate or cyclophosphamide ;as decided by your nephrologist.
Both methotrexate and cyclophosphamide are immunosupressive drugs and require continues monitoring.
Trial shows that discontinuation rates of methotrexate are as high as 16% due to adverse effects which become apparent in 12 to 56 weeks of treatment.

Hence there are pros and cons of each and every drug.

Yes i have already suggested you a Dash diet.
All the below mentioned points comes under DASH diet .

Salt intake should be restricted, a diet containing maximum 1500 mg of sodium per day should be taken,its very essential to stick to a restricted salt diet.
Avoid alcohol.
Salt restriction should strictly be followed.
Avoid oily food .
Try to avoid egg yolks ,heavy proteins,instead try to eat a lot of vegetable,fibres and fruits.

In case you need any other advice ,feel free to ask,i would be happy to help!

Please do rate the answer and write a review .
Thank,
Dr Rishu Saxena

---