Churg-Strauss Syndrome Or Allergic Granulomatosis

Churg-Strauss syndrome is a rare disease characterized by inflammation of small blood vessels (vasculitis). It is also known by the name of allergic granulomatosis and, although it can be treated, there is currently no cure.

The MSD Manual indicates that “it is a type of systematic necrotizing vasculitis of small and medium vessels, characterized by extravascular granulomas, eosinophils and tissue infiltration by eosinophils”.

A rare disease

Churg-Strauss syndrome is a rare autoimmune disease whose main characteristic is inflammation of the blood vessels due to an alteration of the immune system.

This inflammation can lead to more serious conditions that can lead to necrosis of the blood vessels. Inflammation can also reduce blood flow to vital organs and cause potentially permanent damage such as aneurysms or cell death in affected organs.

The lungs are the main organ affected in this pathology. The clinical picture  is characterized by the presence of asthma, rhinitis and sinusitis. However, more serious symptoms can be suffered such as skin, heart, kidney and digestive lesions.

We don’t know exactly what causes Churg-Strauss syndrome. Genetic, immunoallergic, and environmental factors are believed to be involved.

Churg-Strauss syndrome symptoms

The symptoms of this autoimmune disease can be very varied, depending on the person affected. In general, Churg-Strauss syndrome has three phases, although they do not always occur in the same order and may not even go through some of the stages.

Allergic phase

The allergic phase is usually the first stage of Churg-Strauss syndrome. It is characterized by a number of allergic reactions including:

• Asthma: it is usually the first clinical sign that appears in this syndrome, even several years before presenting other types of symptoms. In those cases where the person affected by Churg-Strauss already had asthma, the symptoms of the disease may worsen.

• Allergic rhinitis: this is a type of allergy that causes irritation of the mucous membranes and produces symptoms such as coughing, runny nose or sneezing.
• Sinusitis: the appearance of sinusitis is due to inflammation of the paranasal sinuses. It usually causes a headache, facial pain, and a feeling of congestion.

Eosinophilic phase

Eosinophils are a type of white blood cells (leukocytes) present in the blood that participate in allergic reactions and inflammatory processes. Under normal conditions, these cells are present in small numbers. However, in Churg-Strauss syndrome, the eosinophil population increases uncontrollably  and extravases into the tissues.

The symptoms of this eosinophilic phase can vary depending on the affected organ ; the lungs and digestive organs are often the first to be damaged. Some of the characteristic symptoms of this phase are:

• Fever
• Asthma
• Cough
• Abdominal pain
• Fatigue
• Loss of appetite

Vasculitic phase

Vasculitis is the hallmark of Churg-Strauss syndrome. Inflammation of the small blood vessels can end up necrotizing the tissues and preventing adequate circulation to vital organs.

Some of the typical symptoms of this phase are:

• Muscle tiredness, fatigue, and weakness
• Weightloss
• Skin rashes and ulcers
• Swelling of the lymph nodes
• Joint pain
• Pain and numbness in the hands and feet
• Diarrhea, nausea, and vomiting
• Severe abdominal pain
• Difficulty breathing
• Chest pain
• Arrhythmias

Possible complications

Churg-Strauss syndrome can end up seriously affecting vital organs such as the lungs, kidneys, digestive system, heart, skin, or muscles. Some of the most common complications are:

• Peripheral nerve injuries: the syndrome can end up affecting the peripheral nerves of the feet and hands, due to poor blood supply due to vasculitis.
• Scars on the skin:  Vasculitis can also promote the appearance of ulcers or wounds on the skin.
• Heart disease: when the inflammation typical of Churg-Strauss syndrome affects the heart, more or less serious heart diseases can appear, such as pericarditis or myocarditis, which can lead to heart failure.