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Linda’s Loop

contributed by Brenda Shilling

I have always known that chronic and life-threatening diseases can have a devastating effect on people’s lives. I’ve seen it happen to friends’ families and heard of it happening to friends of friends. But until 2002, the realities of such challenges were quite remote to me, as I had never felt a loved one struggle with a serious condition.

Linda Gray and her twin sister, Brenda Shilling.

In the spring of 2002, my identical twin, Linda, was diagnosed with neuropsychiatry lupus and central nervous system vasculitis. It was a scary time for all of us, particularly for Linda and her family. Like many lupus patients, Linda had been ill often and had many problems in the years leading up to her diagnosis. We were extremely thankful that The Johns Hopkins Vasculitis Center quickly identified what was wrong and started Linda on a treatment program.

In the months that followed, I watched my sister experience a multitude of emotions, including devastation over her diagnosis, fear for her future, and relief that she had finally found help. Although I know that not all lupus patients fare well, I learned that the treatment of lupus has come a long way over the years and I prayed that Linda would benefit from these advances. Her treatment was extremely challenging. However, during the hard realities of chemotherapy and steroids on Linda’s body, her spirit was amazing.

Despite knowing that family is extremely important during times like this and that frequent calls, letters, and visits to Linda were supportive, I slowly began to feel helpless. This was an unexpected emotion. I wanted to do more. I needed to do more. My sister was going through the most difficult time of her life, and I had do something more.

One morning in the late spring of 2003 I considered coordinating a bicycle ride to raise funds for The Johns Hopkins Vasculitis Center. Our sister, Liz, thought it was wonderful idea and wanted to help. We were driven by love for our sister and that was all the motivation we needed!

Linda Gray and her sister, Liz Adams.

We decided on a 50-mile ride – short enough to manage but long enough to sound good! We then mailed over 200 letters to friends and family asking for a financial gift to The Johns Hopkins Vasculitis Center in honor of our sister and others with her disease. We also distributed the letter to social and religious groups in which we are involved. Four of our friends asked if they could join us on the bike ride in a generous gesture of support. We even had t-shirts made!

Participants in Linda’s Loop:

Left to Right: (Front Row, kneeling) Georgann Pattillo, Jan Rowe, Bill Schilling ; (Middle Row) Kevin Adams, Linda Moore, Liz Adams, Sue Schilling, Brenda Schilling, Betty Lamey, Patrick Pattillo, Leroy Lamey ; (Back Row) Chandler Burroughs, Steven Rowe, and Bill Schilling, Sr.

The night before the ride, we finished packing up drinks, snacks, and lunches and then headed home to get some sleep. Liz told me that she didn’t sleep a wink that night – neither did I! We were too excited.

The day finally arrived and everything went so well. It was such a wonderful day that I simply didn’t want it to end! Our family and friends came out to cheer us along. It felt great to have them there. But wow! The miles were more difficult than I expected. Although three riders finished far ahead, the remaining three of us dragged ourselves over the finish line some time later. We were quite the motley crew!

Hot, sweaty, hungry, but happy!

Response to our effort was overwhelming. My husband Bill teases that this was the only time I actually picked up the mail! Liz and I were moved by all the contributions. Some were from people who don’t know Linda but had read about the ride in the local paper. When we started planning we weren’t sure how much money we could raise. It was wonderful to have received just over $8,600 in contributions to vasculitis research!

Linda told me how much love she felt because of what we did for The Johns Hopkins Vasculitis Center. What more could I have asked of myself? We wanted to show Linda how proud we were of all she accomplished during her difficult treatment. We also wanted to say thank you to The Johns Hopkins Vasculitis Center for the wonderful work they do in caring for their patients everyday.

Hopefully we accomplished both.

All information contained within the Johns Hopkins Vasculitis Center website is intended for educational purposes only. Visitors are encouraged to consult other sources and confirm the information contained within this site. Consumers should never disregard medical advice or delay in seeking it because of something they may have read on this website.

Causes of Vasculitis

There are many different types of vasculitis, some with different causes than others.

Certain forms of vasculitis that can be due to infection where the microbe directly invades the vessel wall. Syphilis is one example of vasculitis that can be caused by infection in the blood vessel. Treating the infection is the main goal in managing this sort of vasculitis, which is not an autoimmune disease, but rather an infection.

Other infections can provoke the immune system into causing damage in blood vessels. Here, the infection is the trigger, but the immune system is the cause of the vascular damage. Viral hepatitis (B and C) are examples of this sort: some patients with Hepatitis B may develop polyarteritis nodosa, while some patients with Hepatitis C may develop cryoglobulinemic vasculitis.

Other types of vasculitis may be due to an ‘allergic‘-type reaction to medications. For example, certain blood pressure medications (hydralazine) or thyroid medications (propylthiouracil) can trigger ANCA associated vasculitis in some patients. Cocaine is an illicit drug that is linked to vasculitis and vascular damage.

However, the causes of most vasculitides are currently unknown. While we can identify some risk factors (such as older age in giant cell arteritis), we do not know the specific causes of these diseases. These forms of vasculitis of unknown cause are considered autoimmune diseases.

Under normal circumstances, our immune system serves to defend us from infection and other threats, such as cancers. In autoimmune diseases, the immune system generates a response not against a foreign threat, but against normal “self” tissues. This abnormal immune response against “self” tissues can result in a wide array of autoimmune diseases, including relatively common diseases (such as psoriasis or thyroid disease) as well as rare conditions (such as vasculitis).

In most cases, autoimmune diseases are believed to be due to an abnormal immune response that is generated in a susceptible person, and eventually leads to a cycle of ongoing inflammation in otherwise normal tissues where no infection or other identifiable threat is present. Some interaction between the immune system and the environment is thought necessary for this to occur, and a person’s genetic background likely places some individuals at higher risk than others.

A better understanding of the specific causes of these diseases would lead to improved means of diagnosing, treating, and even preventing these conditions. Uncovering the causes of vasculitis is a major goal of vasculitis research.

While we may not know the specific causes of the vasculitidies, we do have a basic understanding of the way that the immune system causes organ damage in these conditions. In all forms of vasculitis, activation of the immune system leads to the deposition of inflammatory cells and proteins in the walls of blood vessels. As this inflammation in blood vessels continues, the vessels become damaged and no longer serve their normal function of delivering blood to the organs that they supply. Consequently, the tissues downstream of these inflamed vessels are starved of oxygen and nutrients needed for normal function. At a basic level, this is a process similar to what occurs in a heart attack or a stroke – but instead of the cholesterol plaque that blocks a coronary artery in a heart attack, the immune system is responsible for blockage of blood vessels in vasculitis.

Henoch-Schönlein Purpura

Fast Facts
First Description
Who gets Henoch-Schönlein Purpura (the “typical” patients)?
What causes Henoch-Schönlein Purpura?
How is Henoch-Schönlein Purpura diagnosed?
Treatment and Course of Henoch-Schönlein Purpura
Living with Henoch-Schönlein Purpura

Fast Facts

HSP is usually self-limited. Therefore, treatment is not indicated in all cases, and full recovery is the rule.
HSP is more common in children than adults, but has a tendency to be more severe when it occurs in adults.
In a small minority of cases, HSP can cause severe kidney or bowel disease.

First Description

Dr. William Heberden, a London physician, described the first cases of Henoch-Schönlein purpura (HSP) in 1801. In describing HSP, Heberden wrote of a 5-year old boy who “…was seized with pains and swellings in various parts…He sometimes had pains in his belly with vomiting…and the urine was tinged with blood. Presently, the skin of his leg was all over full of bloody points” (purpura). The young boy suffered all four disease hallmarks of HSP: arthritis, gastrointestinal involvement, kidney inflammation, and purpura. Johann Schönlein (1837) and Edouard Henoch (1874) reported additional cases decades after Heberden. They recognized that the disorder often followed upper respiratory tract infections and was not always self-limited, sometimes progressing to serious kidney involvement.

Who gets Henoch-Schönlein Purpura (the “typical” patient)?

Usually, HSP affects a child shortly after an upper respiratory infection has resolved.

HSP is the most common form of vasculitis in children, with an annual incidence on the order of 140 cases/million persons. The mean age of patients with HSP is 5.9 years.

What causes Henoch-Schönlein Purpura?

In two-thirds of the cases, the disease follows an upper respiratory tract infection, with onset an average of ten days after the start of respiratory symptoms. Despite this association, no single microorganism or environmental exposure has been confirmed as an important cause of HSP.

How is Henoch-Schönlein Purpura Diagnosed?

Purpura not due to a low platelet count, caused by inflammation in blood vessels of the skin, is the hallmark of HSP. The tetrad of purpura, arthritis, kidney inflammation, and abdominal pain is often observed. However, all four elements of this tetrad are not required for diagnosis. The microscopic hallmark of HSP is the deposition of IgA (an antibody found in blood, saliva, tears, etc.) in the walls of involved blood vessels.

More than 90% of cases occur in children. The disease usually resolves within a few weeks. However, adult cases are sometimes more difficult. Skin manifestations are more variable in adults, and sometimes symptoms in adults endure longer [Figure 1, 2].

Figure 1. Pustular lesions. These can occur in HSP, but they are more common with the adult form of HSP.

Figure 2. Vesiculobullous lesions These are also more common with the adult form of HSP.

Adults are more prone to permanent kidney damage. However, patients can take some comfort in knowing that fewer than 5% of patients with HSP develop progressive renal insufficiency.

HSP can be mimicked by other forms of systemic vasculitis that are more often life-threatening. Granulomatosis with polyangiitis and microscopic polyangiitis can also present with purpura, arthritis, and renal inflammation. These disorders both have the potential for serious involvement of other organs (for example, the lungs, eyes, and peripheral nerves) and carry more dire renal prognoses. Therefore, it is very important to distinguish the difference by performing a careful evaluation including bloodwork, urinalysis, chest imaging, and possibly biopsies. HSP may be misdiagnosed as another form of vasculitis – most commonly hypersensitivity vasculitis – because of the frequent failure to perform direct immunofluorescence (DIF) testing on skin biopsy and the consequent failure to detect IgA.

Treatment and Course of Henoch-Schönlein Purpura

NSAIDs may alleviate arthralgias but can aggravate gastrointestinal symptoms, and should be avoided in any patient with renal disease. Dapsone (100 mg/day) may be effective in cases of HSP, perhaps through disrupting the abnormal immune response. Although steroids have not been evaluated rigorously in HSP, they appear to ease joint and gastrointestinal symptoms, in many (but not all) patients. Steroids, however, do not appear to improve the rash; although usually, over weeks to months, the recurrent bouts of purpura usually resolve on their own.

Living With HSP

Supportive care may involve a short course of prednisone or an NSAID, such as naprosyn or ibuprofen, if the kidneys are not involved. Keeping the legs elevated may help prevent purpura during flares of active disease. Additionally, many patients’ purpura will recur after they start to feel better and become more active, inherently increasing their exposures to very minor trauma (e.g. jogging, leg shaving, increasing gravity exposures). Often, the recurring purpura is less prevalent [Figure 3], and additional HSP symptoms are often absent. In many fewer cases, primarily in adults, HSP can progress from hematuria (blood in the urine) to renal insufficiency (decreased kidney function). HSP patients who experience this symptom should be followed more closely, with regular testing of their urine for blood and protein. Recurrences, found in 33% of patients, usually develop within the first few months after resolution of the first bout.

Figure 3. Palpable purpura . Occurring in a more diffuse pattern.

Figure 4. Palpable purpura . Here they are occurring in a very dense pattern with coalescing lesions.

Figure 5. Swelling around the hand and wrist . Although arthralgias are more common in HSP, arthritis can occur as well as periarticular swelling, such as the tenosynovitis shown here.

Figure 6. Swelling around the ankle and foot .

Figure 7. Palpable purpura can appear in many different patterns . This picture shows a denser distribution with a sharp demarcation caused by what is known as Koebner’s Phenomenon (Minor trauma, such as the elastic band in one’s sock, can cause such a pattern). In this case, the “trauma” was caused by the patient’s shaving of her legs, leading to the eruption of purpura in the area of skin where the razor had passed. Sufficient pressure, such as this, causes the rupture of inflamed blood vessels.

Figure 8. CT of abdomen showing bowel edema . This image is of a distended large bowel. The characteristic dips between haustra (bowel sections) are less pronounced because of the swelling / inflammation seen in HSP.

Figure 9. Formally known as DIF (Direct Immunofluorescence) testing. This picture shows immunofluorescence testing of a skin biopsy, IgA positive. Palpable purpura should be biopsied, and two fresh samples should always be sent for testing (an adequate biopsy should be large enough to divide; one for H&E (hematoxylin and eosin) staining, and one for DIF testing.

Figure 10. Arm rash . It is more common to have a purpuric outbreak on the lower extremities. However, an outbreak can occur on the abdomen, chest, or as in the case with this woman, on the upper extremities. Note the hive-like lesions that appear larger than the papules. The rash may also be itchy.

Figure 11. Colonoscopy of HSP-affected bowel . This image shows what the lining of the bowel could look like when it is inflamed and swollen, as in HSP. Looks painful…it is.

Avacopan (Tavneos®)

What is avacopan?

Avacopan is an oral drug used to treat ANCA associated vasculitis. Avacopan was FDA approved in 2021, and we are still learning how best to use it.

How does avacopan work?

Avacopan is thought to work by blocking the activation and movement of neutrophils – a type of white blood cell involved in ANCA associated vasculitis. It was designed to block a specific part of the immune system called C5a (complement component 5a).

How is avacopan given?

Avacopan is currently used to help reduce the amount of prednisone given during the treatment of active ANCA associated vasculitis. Avacopan is taken by mouth at a dose of 30 mg twice per day.

Side effects:

We pay particular attention to liver function tests during avacopan therapy.

All immunosuppressants require regular monitoring in the form of blood tests, in-person assessments, and prompt attention to any signs or symptoms of infection.