Churg-Strauss syndrome is a blood vessel inflammatory illness. This inflammation can reduce blood supply to organs and tissues, causing them to be irreversibly damaged. Eosinophilic granulomatosis with polyangiitis is another name for this disorder (EGPA). The most prevalent symptom of Churg-Strauss syndrome is adult-onset asthma. There are a variety of probable side effects from the sickness, including stuffy nose, sinus problems, skin eruptions, gastrointestinal bleeding, as well as soreness and numbness in your hands and feet.
Flu-like symptoms such as fever, a general feeling of weakness and exhaustion (malaise), loss of appetite (anorexia), weight loss, and muscle discomfort are common nonspecific findings linked with this syndrome. Depending on which organ systems are impacted, additional symptoms and results may occur. Peripheral nerves (nerves outside the central nervous system), kidneys, and the gastrointestinal tract are frequently affected. Serious organ damage and potentially life-threatening consequences may occur if proper treatment is not received.
What causes Churg-Strauss Syndrome?
Churg-Strauss syndrome is a relatively uncommon condition. The syndrome’s etiology is unknown, although it involves an aberrant immune system over-activation in a person with underlying bronchospastic lung illness (asthma). While certain asthma drugs known as leukotriene modifiers have been linked to Churg-Strauss syndrome, it is unclear whether they cause the disease or whether people who take them have more severe asthma, which makes them more likely to develop this type of syndrome.
This type of syndrome has yet to be identified. The majority of researchers believe that a variety of factors (such as environmental, immunological, and genetic) all have a role in the disorder’s development. The autoimmune condition Churg-Strauss syndrome categorized as. When the body’s natural defenses against “foreign” or invading organisms begin to attack healthy tissue for unclear reasons, autoimmune diseases develop. Researchers aren’t sure what “sets off” or “triggers” the aberrant immune response in Churg-Strauss syndrome patients. Antineutrophil cytoplasmic antibodies (ANCA) found in approximately 39 to 59 percent of Churg-Strauss syndrome (CSS) patients. ANCAs have also found in diseases of the blood vessels (vasculitides), such as Wegener’s granulomatosis. The specific role of these antibodies in this syndrome’s development is uncertain.
Zafirlukast (Accolate), a nonsteroidal drug approved in 1996 for the prevention and treatment of asthma in adults, has associated with Churg-Strauss syndrome. Asthma patients on zafirlukast have suffered Churg-Strauss syndrome, according to the FDA. All patients weaned off steroidal asthma medicines while on zafirlukast. This does not indicate zafirlukast caused the condition. Also, asthma patients should not discontinue taking their medication without first consulting their doctors. It now says doctors should continuously evaluate patients as corticosteroid dosages lowered.
What are the Signs & Symptoms?
Churg-Strauss syndrome symptoms vary greatly from case to case due to the probable involvement of numerous organ systems. The condition divided into three stages: prodromal, eosinophilic, and vasculitic. These stages may or may not occur in that order. Some people who impacted will not go through all three phases. Churg-Strauss syndrome successfully controlled with adequate treatment. Without treatment, the disease may advance to the point of posing a life-threatening threat.
The prodromal (or allergic) phase frequently precedes the other early phases in Churg-Strauss syndrome. Late-onset asthma symptoms include coughing, wheezing, and shortness of breath (dyspnea). Asthma sufferers may have worsening symptoms. When there is persistent nasal inflammation, tiny, harmless growths (polyps) might form (polyposis). These symptoms can be present for months or even years before they diagnosed. In Churg-Strauss syndrome, respiratory symptoms appear six months to two decades before vasculitis manifests.
Churg-Strauss syndrome’s eosinophilic phase marked by eosinophilia in multiple tissues. Eosinophils the name given to these white blood cells. White blood cells known as eosinophils develop as a result of allergies. Churg-Strauss patients are more likely to develop hypereosinophilia, which can affect the lungs, gastrointestinal tract, and even the skin.
The vasculitic phase of Churg-Strauss syndrome causes widespread inflammation of the body’s blood vessels (vasculitis). In patients with chronic vasculitis, blood vessels narrow, reducing blood flow to many organs. Rupture and leakage into surrounding tissue are possible when inflamed blood vessels become thin and brittle (aneurysm).
The symptoms of Churg-Strauss syndrome differ per organ system. Asthma-like symptoms (described above) frequently appear before other symptoms. However, vasculitis symptoms might occur before respiratory symptoms.
The Affected Populations
Males and females equally affected by Churg-Strauss syndrome, while some findings imply that males harmed slightly more frequently. The condition can affect people of any age, with cases ranging from 15 to 70 years old. The majority of instances affect people between the ages of 30 and 50. The annual incidence rate expected to be 2.4 people per million. According to some experts, Churg-Strauss syndrome is underdiagnosed, which makes determining its true prevalence in the general population problematic.
Other Churg-Strauss Syndrome-Related Disorders
The following illnesses have symptoms that are similar to Churg-Strauss syndrome. For a differential diagnosis, comparisons can be informative.
Wegener’s granulomatosis is an uncommon disease that affects numerous organ systems, the most common of which are the lungs and kidneys. Nasal ulcers with secondary bacterial infection, chronic runny nose, sinus pain, and chronic middle ear infection (otitis media) with potential hearing loss are among the symptoms. Renal problems can develop to kidney failure, which is a life-threatening condition. If the lungs affected, a cough, blood expectoration (hemoptysis), and inflammation of the thin membrane lining the outside and inside of the lungs may develop. The cause of Wegener’s granulomatosis is unknown. (For additional information about Wegener’s granulomatosis, go to the Rare Disease Database.)
Polyarteritis nodosa is a rare multisystem condition that causes extensive inflammation, weakness, and degeneration of small and medium-sized arteries. The arteries supplying the kidneys, heart, gut, neurological system, and/or skeletal muscles impacted. Aneurysms (ballooning) of an arterial wall, formation of blood clots (thrombosis), obstruction of blood supply to certain tissues, and/or tissue damage and loss (necrosis) may arise from artery injury. Weight loss, fever, general malaise, weariness, weakness, headache, muscle aches (myalgias), and/or stomach discomfort are common symptoms. Other symptoms and observations may be present depending on the body location affected. Although the actual etiology of polyarteritis nodosa is unknown, many researchers believe it caused by immune system issues. (Search the Rare Disease Database for “polyarteritis nodosa” for more details.)
Churg-Strauss syndrome characterized by eosinophilia, pulmonary infiltrates, and vasculitis. Aspergillosis of the lungs can be caused by eosinophilic infections and malignancies, such as acute eosinophilic pneumonia, hypereosinophilic syndrome, and microscopic angiitis. Churg-Strauss syndrome-like symptoms can occur as a result of certain drug reactions.
Most people with Churg-Strauss syndrome start with corticosteroids like prednisone or methylprednisonone. It may also affect the immune system and reduce inflammation. Corticosteroid dosages are initially high, then gradually reduced as improvement seen (tapered off). Many patients just use corticosteroids to obtain symptom relief (monotherapy).
Patients who do not respond to steroid therapy or who have a severe disease may need treated. With cytotoxic treatments such as cyclophosphamide or azathioprine. Other treatments recommended. Other Churg-Strauss Syndrome treatments are symptomatic and supportive. The FDA approved Nucala (mepolizumab) in 2017 for treating adults with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). GlaxoSmithKline makes Nucala.