What is a Churg-Strauss Syndrome?

Churg-Strauss syndrome, often abbreviated to CSS, is a rare medical condition that affects the auto-immune system of sufferers, causing it to overreact with harmful consequences. More specifically, the syndrome, is a form of vasculitis, a condition that causes inflammation of blood vessels. In cases of CSS, it's small to medium-sized blood vessels that are affected, and it can produce any of a variety of symptoms in patients suffering from this disorder. Cases of this form of vasculitis range from mild to severe, and Churg-Strauss syndrome, unfortunately, is not curable at the present time. It's a chronic, lifelong condition, which although life-threatening in severe cases is not necessarily a fatal disease. It can be managed quite effectively with a carefully chosen and followed course of immunosuppressive drugs that can enable patients in many cases to enjoy long periods of remission.

How Churg-Strauss syndrome progresses

Typically, the syndrome progresses in three distinct stages in the order shown below, but there are sometimes exceptions to this order and the stages may also overlap to an extent. Symptoms experienced by patients can also be highly variable depending on which part of the body has been affected by the disease.

Stage 1 - The allergic stage

This stage of Churg-Strauss syndrome is characterised by allergic reactions. Asthma is the most common symptom, and this stage may last between three and nine years before other stages of the disease appear. Those who already have a history of asthmatic problems are very likely to experience a worsening of their condition. Other symptoms such as hay fever and sinusitis are also commonly experienced during this initial phase of Churg-Strauss syndrome. Nasal polyps requiring repeated surgical removal may also form during this stage.

Stage 2 - The eosinophilic stage

For the patient, the second or eosinophilic stage of Churg-Strauss syndrome is characterised by a variety of symptoms depending on which part of the body is affected by the condition. Medically, this stage is characterised by the markedly increased levels in the blood stream of eosinophils, a particular type of white blood cell. Overproduction of eosinophils can cause tissue damage particularly to the lungs and digestive tract where concentrations of them are usually at their highest. Symptoms experienced during the eosinophilic stage may include weight loss, abdominal pain, coughing, malaise and fever. This stage can also last a number of years, and may even intrude into the third and final stage of the disease.

Stage 3 - The vasculitic stage

The vasculitic stage of Churg-Strauss syndrome is the final and permanent stage of the condition, and in the most severe cases, it can be life-threatening. This stage is characterised by inflammation of small to medium-sized blood vessels, which reduces the blood flow to vital organs and tissue. Blood clots may also form in severe cases, and heart disease may also develop during the vasculitic stage. Symptoms experienced by the patient during this stage may include diarrhea, skin rashes, sores, vomiting, coughing up blood as well as the presence of blood in their urine.


The key to effective treatment of Churg-Strauss syndrome is early diagnosis. The earlier the condition is confirmed, the more effectively it can be treated by immunosuppressive drugs, thus avoiding severe and potentially fatal complications that may arise during the later stages of the disease. Unfortunately, it's not all that easy to make an accurate diagnosis during the initial stage of the disease as the symptoms presented can be caused by a large range of disorders, many of which are trivial and temporary, but the current medical advice is that anyone having a runny nose or sinus problems that refuse to clear up, or who have breathing problems, should see their doctor. There are elaborate tests that need to be carried out before a diagnosis of the syndrome can be accurately confirmed, and the doctor can arrange for such tests to be carried out in a timely manner. Tests for this syndrome may include blood and urine analysis, lung biopsy, and an x-ray and chest CT scan may be performed among other procedures. Diagnosis is easier during the second stage of the condition due to being able to detect greatly elevated levels in the blood stream of eosinophils capable of causing damage to surrounding tissue, but it's important to have the condition diagnosed as soon as possible so that treatment can begin before any lasting or permanent damage is done to vital organs or tissue.

Treatment of Churg-Strauss syndrome

Treatment of this syndrome involves the patient regularly taking immunosuppressive drugs such as prednisolone and azathioprine. As the disease can vary hugely in its severity and in its symptoms from person to person, so too do the treatments. Not all confirmed cases of CSS are life-threatening or debilitating. Some cases of the syndrome are so mild that they are no more than an annoying inconvenience to the patient. In such cases, the condition can be managed with minimal treatment. More severe cases may require a more complex course of powerful immunosuppressive drugs.

The History of CSS Syndrome

Churg-Strauss syndrome is named after Jacob Churg and Lotte Strauss, two doctors working as pathologists at New York's prestigious Mount Sinai Hospital. They identified and described the disorder in 1951 and called it allergic granulomatosis with angiitis. It's also known as eosinophilic granulomatosis with polyangiitis, abbreviated to EGPA.