Sjögren's Syndrome & Vasculitis

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Valculitis attack on your editor  Nov 2919

 

A Hat Trick of Vasculitis attacks in just 3 week’s

 

Regular visitors to the Sjogren’s  Cambs website may well have noticed the absence of new content over recent weeks.  It reflects on the problems that someone with a serious autoimmune condition can face when taking on the role of editing a website

 

This article is being typed in a patient’s bedroom at the Dermatology Clinic at The University Klinikum, Dusseldorf, Germany.  The article will reflect on what occurred related to my own condition, some reflections on the pro’s and cons of being a patient in Germany under the Insurance based scheme introduced by Bismarck comparing it to the NHS system and the free Market world of Commercial For Private Profit Medical Insurance which has been put out in the public arena by Farage and other UK political operators.

 

Personal experience since December 2017

 

During the winter months I experienced much pain undertaking simple movements, on a bad day unable to getup from a chair or even out of bed without help.  As my Secondary Sjogren’s Syndrome has caused severe joint and muscle pain for over twenty years, the initial assumption was that this was related to the Rhumatic  problems related to my illness.  My body was experiencing the usual problems releasing water. Kidney disease is another area which links to the history of my current problems.

 

 

During the excessive heat encountered over here in Germany during  July 2019 the water problem became very acute.  The build up was acute and the body’s way of dealing with this factor was violent and uncontrollable to say the least.

 

During September with such extreme problems something had to give, it always does with an autoimmune illness where the weakest area of the body is subject to an attack and the basic defensive mechanism of the body just break down and assist the attack.

 

When researching the pages we earlier published Vascular conditions were a possibility but I along with many other Sjogren’s Syndrome patients hoped it would not happen to me and had no idea of the intensive and excess pain and speed of onset Vasculitis can have.

 

During late September 2019 I noticed a tiny red mark on my right thigh which itched and the urge to scratch the area was something that common sense had to override short term relief of the intense itching.   A few hours laterI awoke to notice this tiny red mark had grown in size at rapid speed to a size of 50mm x 50mm and appeared a a black bruise.  The pain was very severe an I was barely able to move to to the severity of the pain.  A phone call to my GP resulted in an instant appointment and I was given a referral to the local hospital, Krankenhaus Florence Nightingale in Kaiserswerth.  After extensive tests this hospital could not get to the bottom of the problem.  One staff member said it should go away naturally in about four weeks.  Bull....! Is not only found in the NHS but abroad too.  One doctor did suggest that a dermatologist looked at the condition and immediately arranged an appointment at the Emergency Out of Hours ambulanz at the Specialist Skin Clinic at the regions largest teaching hospital UKD University Klinikum Dusseldorf.

 

During the excessive heat encountered over here in Germany during  July 2019 the water problem became very acute.  The build up was acute and the body’s way of dealing with this factor was violent and uncontrollable to say the least.

 

During September with such extreme problems something had to give, it always does with an autoimmune illness where the weakest area of the body is subject to an attack and the basic defensive mechanism of the body just break down and assist the attack.

 

When researching the pages we earlier published Vascular conditions were a possibility but I along with many other Sjogren’s Syndrome patients hoped it would not happen to me and had no idea of the intensive pain and speed of onset Vasculitis can have.

 

During late September 2019 I noticed a tiny red mark on my right thigh which itched and the urge to scratch the area was something that common sense had to override short term relief of the intense itching.   A few hours laterI awoke to notice this tiny red mark had grown in size at rapid speed to s size of 50mm x 50mm and appeared a a black bruise.  The pain was very severe an I was barely able to move to to the severity of the pain.  A phone call to my GP resulted in an instant appointment and I was given a referral to the local hospital, Krankenhaus Florence Nightingale in Kaiserswerth.  After extensive tests this hospital could not get to the bottom of the problem.  One staff member said it should go away naturally in about four weeks.  Bull....! Is not only found in the NHS but abroad too.  One doctor did suggest that a dermatologist looked at the condition and immediately arranged an appointment at the Emergency Out of Hours ambulanze at the Specialist Skin Clinic at the regions largest teaching hospital UKD University Klinikum Dusseldorf.

 

Readers please note the same words can mean something completely different in different countries.  An Ambulanz in Germany is not a large wan featuring blue lights, special equipment and crewed by paramedics, nor is it a vehicle that ferry’s patients to and from hospital.   Ambulanz in Germany is a hospital out patients department.

 

After a very long, painful day, my wife (careers suffer just as much as patients) and I were starving and the emergency doctor had three more patients to see before our turn.  We were saved by the proverbial bell as the lady who serves the evening meals to patients on the wards offered to rustle up some food and drinks.  The ham and cheese salad samdwiches did the trick and revived us.  I have never been offered sustenance after a long wait in hospital before.  Most places expect you to buy it from the food court areas..

 

The emergency doctor on duty was honest and stated that he had not personally seen anything like my injury before and phoned his boss ( the Oberarzt) and sent her a photo of the injury.

It was decided there and then that I had to report to the Clinic’s Daycare Ambulanz the following morning to be admitted for residential treatment.

 

The next morning I noted that the building where the Dermatology/ skin clinic was based has a sign over the main entrance. “BAU XXXXXl”. No it was not built after the war in 1951 this building was originally constructed in 1851 and is one the the oldest buildings in this vast hospital complex.  UKD Uni-Klinikum  Düsseldorf is a really large complex site and features new state of the art buildings, listed historic buildings which have been upgraded to meet modern needs internally but retaining the historical exterior rebuilt to reflect the building when it was  new.  The present dental clinics and eye clinics are the product of this policy, inside state of the art but outside reflecting the look of the buildings when first opened as a care home complex.  

 

A tour of the complex results in a unique survivor of the blitzing of Germany where many old  buildings   survived and are valued in a country where so much had to be rebuilt very quickly during the late 1940’s and 1950’s.  It also features buildings that represent the ideals in hospital design and planning over 170 years.  The centre piece in the inter denominational chapel which is a real church building featuring bell tower and one awakes to hear churchbells sounding if there is a service being held.  This reflects upon the churches involvement in German Hospitals since the earliest days of the hospital system in nearby Kaiserswerth.  The Church Services are televised on the hospitals internal TV channel to all bedsides.

 

Another unique building is the air raid shelter located behind the chapel, does anyone know of another hospital with it ‘s  own air raid shelter!  Bear in mind Germany was on the front line during the Cold War era (both West and East Germany) lasting until 1989.

 

 Back to my treatment and diagnosis, it was decided that I was suffering from a yet undefined form of Vasculitis and I had to give permission for a surgeon to take a biopsy shortly after arrival on the ward.   This was a different experience as a photographer was awaiting my arrival to take a series of pictures of the injury before the biopsy was taken.   The surgeon’s and staff asked me about my thoughts as an Englishman on Brexit, I replied we call it  Brexsxxt and it reassured the staff that by no means are  the majority of British people in agreement with this crazy loss of influence, power and prestige being inflicted on our nation by a certain privileged group in the U.K., political structure. The truth is that Britain shares a very close culture  with countries such as Germany, The Netherlands and the Baltic and Scandinavian countries.  Languages may differ but the basic culture is so similar.  UKD is now rebuilding and upgrading  a large tower building as residential accommodation for hospital staff being recruited from the NHS.  Better Pay, Working Conditions and Longer Paid Holidays are all part of this attractive package.  While our U.K. government is hell bent on destroying the NHS by removing the right to work and move freely for EC citizens our culturally similar neighbours are now able to take advantage of this pool of trained experienced medical professionals which has been dumped by the U.K. in the persuit of the most stupid course ever taken by any U.K. politicians in history. (Destroy and undermine the NHS to make money for big investors, large scale political donors who want to avoid the new European Tax Directive 2020 which is designed to deal with tax avoidance on a large scale across Europe ( including the U.K. ]

 

Shortly after my biopsy I was wheeled back onto the ward to commence treatment, A huge increase in steroid dosage, treatment of the wound with antisceptics and an array of creams featuring cortisone and a high fat content.  After the morning doctor’s visit my legs were dealt with with cooling  antisceptics, being from the generation where the mere word antisceptics meant being accompanied by a stinging pain, it was a relief to have antisceptics applied which were cooling.  The next stage was to cream the infected area and apply dressings plaster or bandages subject to the rate of healing. This was followed by covering the  creamed legs with stockinette and then binding the legs with compression bandages from the feet to the upper thighs.  The stockinette was doubled over and returned up the legs over the compression bandages.

 

The morning schedule also included blood tests, blood sugar tests and blood pressure tests.

 

A second doctors visit was made in the afternoon and sometimes during the evening.

 

The leg treatment continued during the evenings with the antisceptics and cream treatment but this time the legs were not bound to enable them to breath naturally and dry out overnight

 

The really severe pain  like core was coming away like a thin film, It looked like a scab but no scab had formed like a normal injury.  As the black areas reduced in size they were replaced by areas of bright red skin which identified that the healing process was underway.

 

The slow process appeared to have achieved a cure but still awaited were the complex series of lab reports.  I did not expect this condition to return and not as quickly as was the case 

 

 

The staffing at large teaching hospitals in Germany is strictly regulated. The Clinic Director.has to be qualified as a University Professor and Dr. Med to hold this position,  The Professor male or female leads a team made up of Oberarzt ( senior consultants.) who lead the day to day work

Being a teaching hospital rounds undertaken by the professor and the Oberarzt always feature the doctors who work on the ward (answering questions  from the senior person present about the patients condition and treatment plan.  It is somewhat reminiscent of the old Doctor in the House films from the 1950’s but involving a younger generation.

 

After seven days the wound had healed and stitches were due for removal 12 days after the biopsy operation, this was undertaken at the GP practice (hausartzt) by a doctor. There remained one further hospital appointment in a couple of weeks from the date of discharge to give me the laboratory reports and also to check on the healing process.

 

However during the week prior to the final meeting at the hospital,  I suffered a further attack in the form of an-identical wound forming in a different area of the left leg, this time the area effected was the lower right shin about 100 cm below the knee.  Yet again the onset was rapid and pain levels were extreme.  On this occasion it happened on a Sunday evening when Ruth, my wife, was visiting our children in Britain.   Fortunately I was able to seek assistance from a couple who are Neighbours who work as Senior Surgeon’s at the nearby hospital in Krefeld, who had offered to help at any time.  Having a senior senior trauma surgeon and a senior. Vascular Surgeon next door is a luxury available to few suffers from my condition.  After both inspected the injury and a start was made immediately in treating my damaged right shin.  The pain was just as excessive as on the earlier occasion with a lager area effected.. The Vascular Surgeon promised to undertake a full check of my veins and arteries two days later at his clinic in the Helios Klinikum ( the main hospital in Krefeld,)   All paperwork needs was arranged personally by the surgeon with my GP practice..  The Helios Klinikum is a new hospital complex run by a private company and is situated close to the city centre in a landscaped environment.  Much smaller than Uni-Klinikum Düsseldorf in size, but purpose designed with departments easy to find.  I had to smile at the large model Stork which displayed a sign announcing the latest birth at the maternity wing, or the modern sculpture of a man perched most precariously on top of the Children’s wing of the hospital.  This hospital won the award as Best Hospital in Germany 2019 in a review by the business journal Handelsblatt

 

Passing the receptionist to get to the appointment meant side stepping the formal procedures when an Einweisungschine (specific appointment) could not be produced as per the strict systems applied.  Generally patients are issued with an uberweisungschiene ( an open referral where the patient selects the hospital or specialist who they wish to be seen by.  German burocracy can be very frustrating at times.  After explaining that this had been personally arranged by the Oberarzt in charge of the Ambulanz in the Gefassklinik ( Vascular Surgery).  The receptionist scurried of to seek advice on what to do.  A fete about two minutes she returned with another lady who accompanied me to the Reception area in the Vascular Clinic. Our neighbour met me and apologised that I would have a short wait as he had to deal with another patient first.

 

The tests were carried out to establish the extent of damage to the arteries and veins, to supplement tests carried out on the skin in Düsseldorf,  The results were given to meet the time and a letter from the hospital was delivered within 24 hours of the test.  News was generally better than expected, for my age the arteries are in very good shape. The veins showed some problems but thankfully the inner areas of the legs were not effected by the Vasculitis attacks.  I am very lucky to have some very helpful neighbours.   The Senior Surgeon based in Krefeld visited me each day after work to attend to the wounds.

 

Hat trick - just 4 days on from the second attack a third occurrence took place this time on my left thigh and the Vasculitis injury proved to be much larger than the previous two which were by no means small.  I was scheduled to have a post hospital check at Unit-Klinikum the early the following  morning and took a precautionary bag for a hospital stay. As soon as I was able to speak with the doctor I explained what had happened and  I was wheeled across to the residential ward. Luckily I was given an upgrade to the Private Patient’s station with a single room.

 

The speed of check in was impressive and within minutes I had been seen by a doctor on the station (ward).  First I had to be taken upstairs to the photographic studio  a  series of  full body image pictures were taken[1]  and arrangements made to return a week later for a before and                  after photo shoot.

 

Back to the ward and a detailed investigation commenced, a blood test with at least a dozen different samples were taken for lab analysis.

 

I was immediately taken off the blood thinners prescribed for anti coagulation purposes after a prenomanary embolism. Blood Clots on the left lung which originated in the right lower leg and moved up to the lungs.  This dates back to the mid 2000,s when it was treated at Peterborough City Hospital in Gt, Britain. The  replacement drug Eliquis 5mg to be taken twice per day, but no blood tests are required at The GP practice to monitor blood /water levels.


 

 

The Chapel at UKD      

Air Raid Shelter !!

 

 

Patient education: Vasculitis (Beyond the Basics)

Author

Peter A Merkel, MD, MPH

Section Editor

Eric L Matteson, MD, MPH

Deputy Editor

Monica Ramirez Curtis, MD, MPH

 

INTRODUCTION

 

The word “vasculitis” means inflammation of blood vessels. Blood vessels include the huge network of arteries and veins that deliver blood from the heart to all of the organs and tissues throughout the body and then return the blood back to the heart. Blood vessels range in size from very large (eg, the aorta, the body's largest artery which carries blood from the heart) branching out to very small (eg, capillaries that bring oxygen to the body's tissues).

The inflammation caused by vasculitis can affect the lining of the blood vessels (called the endothelium) or the wall of an artery or vein. This can cause the vessel to become thickened, weakened, narrowed (stenosis), blocked (occlusion), enlarged (aneurysm), or scarred. A damaged vessel may not function normally, which can affect blood flow to the tissues that the vessel normally serves. This can lead to partial or complete organ failure due to lack of blood flow, or bleeding into the skin or other part of the body due to rupture of the blood vessel wall. This damage is sometimes permanent.

Some types of vasculitis resolve without treatment, while others require lifelong treatment with medicine. Fortunately, treatments can usually control or minimize vessel damage in the short term. However, both short- and long-term side effects of these treatments are common. Furthermore, relapses of vasculitis (also known as “flares”) are also common. It is extremely important that patients with vasculitis see their doctor or nurse on a regular basis.

 

VASCULITIS CAUSES

 

In most cases, the cause of vasculitis is unknown. A combination of factors likely sets the inflammatory process in motion.

Vasculitis can occur in conjunction with another illness, such as lupus or rheumatoid arthritis.

 

 

Vasculitis can develop as a reaction to certain drugs or other substances (called drug-induced vasculitis).

 

Vasculitis can also occur as a result of infection such as the following viruses: hepatitis B or C, HIV (the virus that causes AIDS), cytomegalovirus, Epstein-Barr virus, or parvovirus B19. Vasculitis can also occur with some infections caused by bacteria.

 

The group of diseases labeled as “vasculitis” often refers to several types of vasculitis with no obvious cause, also known as “idiopathic” vasculitis; this set of diseases is the focus of this discussion.

 

 

VASCULITIS SYMPTOMS

 

Symptoms of vasculitis vary from one person to another and depend upon the type of vasculitis and location in the body of inflamed vessels. Some common symptoms include:

Fatigue or weakness

Fever

Muscle and joint pain

Rash

Lack of appetite and weight loss

Abdominal pain

Kidney problems (bloody or dark-colored urine)

Nerve problems (numbness, weakness, pain)

 

 

VASCULITIS DIAGNOSIS

 

It can be difficult to diagnose vasculitis because the symptoms are similar to those caused by a number of other illnesses. Your doctor or nurse will talk to you, will perform a physical exam, and may order tests to help establish a diagnosis.

The tests used to diagnose vasculitis depend on the type of vasculitis that is suspected and may include blood or urine tests, imaging tests (like X-ray or magnetic resonance imaging [MRI]), nerve testing, or lung function testing. A biopsy is often required to be sure of the diagnosis before starting treatment.

 

(Editor - There are many types of vasculilis and a specialist referral may be needed to obtain the correct diagnosis - it is a complex condition)

 

VASCULITIS TREATMENT

 

The treatment of vasculitis will depend on the specific type of vasculitis and the areas of the body that are involved.

 

General measures — Treatment may include one or more of the following approaches.

 

Glucocorticoids (also called “steroids”), such as prednisone, can be taken by mouth (as a pill) in most cases; high doses may be given into a vein. Because there are risks when glucocorticoids are taken for long periods of time, the goal is to take them only as long as needed.

 

Some people require treatment with long-term glucocorticoids to control symptoms and prevent worsening of their condition. Close monitoring for possible side effects of glucocorticoids, such as diabetes, weight gain, or osteoporosis (bone thinning), is needed. There are medicines that can reduce the risk of bone fractures for people who take long-term glucocorticoids. (See 

 

Additional treatment (beyond glucocorticoids) that suppresses the immune system might be needed for more serious types of vasculitis. One type of immunosuppressive medication, cyclophosphamide, has dramatically improved the outlook for people with some types of vasculitis.

 

Azathioprine, methotrexate, and mycophenolate are medicines that also suppress the immune system but are not as strong as cyclophosphamide. These treatments can be used for less severe forms of vasculitis and as maintenance therapy after treatment with cyclophosphamide.

 

A newer set of medications, called “biologics,” are increasingly being used to treat some forms of vasculitis. These newer drugs include rituximab, tocilizumab, and anti-tumor necrosis factor (TNF) medications.

 

All immunosuppressive medications have the potential to increase the risk of infections, and each type of medication has other important side effects that must be monitored for by a doctor.