Churg-Strauss syndrome, also known as Eosinophilic Granulomatosis with Polyangiitis (EGPA), is a rare autoimmune disorder that causes inflammation of blood vessels throughout the body. This condition affects approximately 1 to 3 people per million annually and typically develops in adults between 30 and 50 years of age. The syndrome is characterized by elevated levels of white blood cells called eosinophils, which can damage various organs and tissues.
The disease typically progresses through three distinct phases: the allergic phase, the eosinophilic phase, and the vasculitic phase. Each phase presents unique symptoms that can affect multiple organ systems, including the respiratory system, skin, heart, nervous system, and gastrointestinal tract. Early recognition of symptoms is crucial for timely diagnosis and management.
Understanding the warning signs of Churg-Strauss syndrome can help patients and healthcare providers identify the condition earlier, potentially preventing serious complications. Below are the seven most common and significant symptoms associated with this condition.
1. Severe Asthma and Respiratory Problems
Asthma is often the first and most prominent symptom of Churg-Strauss syndrome, appearing in approximately 90-95% of patients. This respiratory manifestation typically develops years before other symptoms emerge and tends to be more severe than typical allergic asthma.
Patients may experience:
- Progressive worsening of asthma symptoms despite treatment
- Frequent asthma attacks requiring emergency care
- Persistent wheezing and shortness of breath
- Chest tightness and difficulty breathing, especially at night
- Reduced response to standard asthma medications
- Chronic cough with or without mucus production
The asthma associated with Churg-Strauss syndrome often appears in adults who have never experienced breathing problems before, or it may represent a sudden deterioration in patients with previously well-controlled asthma. This late-onset or suddenly worsening asthma should prompt further investigation, especially when accompanied by other systemic symptoms.
2. Chronic Sinusitis and Nasal Problems
Chronic inflammation of the sinuses and nasal passages is another hallmark symptom of Churg-Strauss syndrome, affecting approximately 70% of patients. This symptom often develops alongside or shortly after the onset of asthma.
Nasal and sinus symptoms include:
- Persistent nasal congestion lasting for months
- Chronic sinus infections that recur frequently
- Nasal polyps (growths in the nasal passages)
- Facial pain and pressure, particularly around the eyes, cheeks, and forehead
- Reduced or complete loss of sense of smell
- Thick nasal discharge that may be yellow or green
- Post-nasal drip causing throat irritation
- Nosebleeds in some cases
The sinusitis associated with Churg-Strauss syndrome tends to be particularly resistant to standard treatments, including antibiotics and nasal corticosteroids. Patients often undergo multiple courses of treatment without significant improvement, which should raise suspicion for an underlying systemic condition.
3. Peripheral Neuropathy and Nerve Damage
Peripheral neuropathy, or damage to the nerves outside the brain and spinal cord, occurs in approximately 50-75% of patients with Churg-Strauss syndrome. This symptom typically appears during the vasculitic phase of the disease when blood vessel inflammation restricts blood flow to the nerves.
Neurological symptoms may include:
- Numbness and tingling in the hands and feet
- Burning or shooting pain in the extremities
- Weakness in the arms or legs
- Foot drop (difficulty lifting the front part of the foot)
- Wrist drop (inability to extend the wrist and fingers)
- Loss of reflexes
- Difficulty with fine motor skills like buttoning shirts or picking up small objects
- Asymmetric nerve involvement (affecting one side more than the other)
The nerve damage in Churg-Strauss syndrome typically follows a pattern called “mononeuritis multiplex,” where multiple individual nerves are affected in a random, asymmetric distribution. This can result in sudden weakness or sensory loss in specific areas of the body. Without proper management, peripheral neuropathy can lead to permanent nerve damage and disability.
4. Skin Rashes and Lesions
Skin manifestations occur in approximately 40-70% of patients with Churg-Strauss syndrome and can vary widely in appearance. These dermatological symptoms result from inflammation of small blood vessels in the skin (cutaneous vasculitis) or infiltration of eosinophils into skin tissue.
Common skin symptoms include:
- Purpura: Small purple or red spots on the skin caused by bleeding under the surface, typically on the lower legs
- Nodules: Firm, raised bumps under the skin, usually on the elbows, scalp, or trunk
- Urticaria-like lesions: Raised, itchy welts resembling hives but lasting longer than typical allergic reactions
- Livedo reticularis: A mottled, net-like pattern on the skin
- Digital ischemia: Poor blood flow to fingers or toes, causing color changes and pain
- Ulcerations: Open sores that may develop on the legs or other areas
- Papules: Small, raised bumps on the skin surface
The appearance and distribution of skin lesions can provide important diagnostic clues. Skin biopsies often reveal characteristic features of vasculitis or eosinophilic infiltration, which can help confirm the diagnosis of Churg-Strauss syndrome.
5. Extreme Fatigue and General Malaise
Profound fatigue and a general feeling of being unwell (malaise) are common constitutional symptoms affecting the majority of patients with Churg-Strauss syndrome. These symptoms often accompany the transition from the allergic phase to the more serious vasculitic phase of the disease.
Patients typically describe:
- Overwhelming exhaustion that doesn’t improve with rest
- Significant decrease in energy levels and stamina
- Difficulty completing daily activities and work tasks
- Feeling generally unwell or “flu-like” symptoms without having an infection
- Muscle aches and body pain
- Low-grade fever (temperature between 99-100.5°F or 37.2-38°C)
- Unintentional weight loss
- Loss of appetite
- Night sweats
The fatigue associated with Churg-Strauss syndrome is typically more severe than simple tiredness and can significantly impact quality of life. This persistent exhaustion often prompts patients to seek medical attention, especially when combined with other unexplained symptoms. The systemic inflammation underlying the disease contributes to these constitutional symptoms as the body’s immune system remains in a heightened state of activation.
6. Gastrointestinal Symptoms
Gastrointestinal involvement occurs in approximately 30-50% of patients with Churg-Strauss syndrome and can range from mild discomfort to life-threatening complications. The digestive system symptoms result from inflammation of blood vessels supplying the intestines (mesenteric vasculitis) or direct eosinophilic infiltration of the gastrointestinal tract.
Digestive system symptoms include:
- Abdominal pain, which may be cramping or severe
- Nausea and vomiting
- Diarrhea, sometimes bloody
- Blood in the stool (either bright red or dark, tarry stools)
- Loss of appetite and early satiety
- Bloating and abdominal distension
- Gastroenteritis-like symptoms
- Weight loss due to poor nutrient absorption
In severe cases, inflammation can lead to serious complications such as bowel perforation (a hole in the intestinal wall), intestinal bleeding, or bowel obstruction. These conditions require immediate medical attention. Patients experiencing severe abdominal pain, especially if accompanied by fever, persistent vomiting, or bloody stools, should seek emergency care promptly.
Gastrointestinal symptoms in Churg-Strauss syndrome can sometimes mimic other conditions like inflammatory bowel disease, making diagnosis challenging without considering the full clinical picture.
7. Cardiac Manifestations
Heart involvement is one of the most serious complications of Churg-Strauss syndrome and represents the leading cause of mortality in this condition. Cardiac symptoms occur in approximately 15-50% of patients and can manifest in various ways depending on which part of the heart is affected.
Cardiac symptoms may include:
- Chest pain or discomfort
- Shortness of breath, especially during physical activity or when lying flat
- Rapid or irregular heartbeat (palpitations or arrhythmias)
- Swelling in the legs, ankles, and feet due to fluid retention
- Persistent cough, sometimes with pink, frothy sputum
- Dizziness or fainting episodes
- Reduced exercise tolerance
- Fatigue disproportionate to activity level
The heart can be affected by Churg-Strauss syndrome through several mechanisms:
- Myocarditis: Inflammation of the heart muscle, which can impair the heart’s ability to pump blood effectively
- Coronary vasculitis: Inflammation of the arteries supplying blood to the heart, potentially leading to heart attacks
- Pericarditis: Inflammation of the sac surrounding the heart
- Heart failure: Reduced pumping capacity due to eosinophilic infiltration or inflammation of heart tissue
- Valvular disease: Damage to heart valves affecting blood flow through the heart
Cardiac involvement in Churg-Strauss syndrome can be life-threatening and may occur suddenly. Any chest pain, severe shortness of breath, or signs of heart failure warrant immediate medical evaluation. Early detection and management of cardiac symptoms are critical for improving outcomes in patients with this condition.
What Causes Churg-Strauss Syndrome?
The exact cause of Churg-Strauss syndrome remains unknown, but researchers believe it results from a combination of genetic predisposition and environmental triggers that lead to abnormal immune system activation. Several factors have been identified that may contribute to the development of this condition:
Genetic Factors: While Churg-Strauss syndrome is not directly inherited, certain genetic variations may increase susceptibility to developing the condition. Research has identified associations with specific HLA (human leukocyte antigen) genes that play a role in immune system regulation.
Allergic Predisposition: Nearly all patients with Churg-Strauss syndrome have a history of allergies, asthma, or allergic rhinitis. This allergic background suggests that chronic allergic inflammation may set the stage for the development of the more serious vasculitic phase of the disease.
Environmental Triggers: Various environmental factors have been proposed as potential triggers, including:
- Allergen exposure (pollen, dust mites, molds)
- Infections, particularly respiratory infections
- Vaccinations in rare cases
- Certain medications, though this association remains controversial
Immune System Dysfunction: Churg-Strauss syndrome is fundamentally an autoimmune disorder where the immune system mistakenly attacks the body’s own blood vessels. The condition is characterized by:
- Excessive production and activation of eosinophils (a type of white blood cell)
- Formation of granulomas (clusters of inflammatory cells)
- Vasculitis (inflammation of blood vessels)
- Production of ANCA (anti-neutrophil cytoplasmic antibodies) in 30-40% of patients
Leukotriene Modifiers: There has been debate about whether certain asthma medications called leukotriene modifiers might trigger Churg-Strauss syndrome. However, most experts now believe these medications don’t cause the disease but rather that reducing corticosteroid doses in asthma patients may unmask underlying Churg-Strauss syndrome that was previously suppressed.
It’s important to understand that Churg-Strauss syndrome is not contagious and cannot be transmitted from person to person. The condition develops due to internal immune system dysfunction rather than external infection.
Frequently Asked Questions
How is Churg-Strauss syndrome diagnosed?
Diagnosis involves a combination of clinical evaluation, blood tests showing elevated eosinophils, imaging studies, and tissue biopsy. The American College of Rheumatology criteria include asthma, eosinophilia, neuropathy, pulmonary infiltrates, sinus abnormalities, and biopsy showing eosinophils outside blood vessels. Meeting four or more criteria suggests Churg-Strauss syndrome.
Is Churg-Strauss syndrome life-threatening?
Yes, Churg-Strauss syndrome can be life-threatening, particularly when it affects the heart, lungs, kidneys, or gastrointestinal system. Cardiac involvement is the leading cause of mortality. However, with prompt diagnosis and appropriate management under medical supervision, many patients can achieve remission and maintain good quality of life.
Can Churg-Strauss syndrome go into remission?
Yes, many patients with Churg-Strauss syndrome can achieve remission with proper medical management. Remission means that symptoms are controlled and disease activity is minimal or absent. However, the condition is chronic and may require long-term monitoring, as relapses can occur even after periods of remission.
What is the difference between Churg-Strauss syndrome and regular asthma?
While both conditions involve airway inflammation and breathing difficulties, Churg-Strauss syndrome is a systemic vasculitis that affects multiple organs beyond the lungs. It features extremely high eosinophil counts, affects blood vessels throughout the body, and includes symptoms like nerve damage, skin rashes, and potentially heart involvement—none of which occur in typical asthma.
How rare is Churg-Strauss syndrome?
Churg-Strauss syndrome is very rare, affecting approximately 1 to 3 people per million each year. It typically develops in adults between ages 30 and 50, though it can occur at any age. Men and women are affected approximately equally, though some studies suggest a slight male predominance.
Can children develop Churg-Strauss syndrome?
While rare, children can develop Churg-Strauss syndrome, though it occurs much more commonly in adults. Pediatric cases may present similarly to adult cases with asthma, eosinophilia, and systemic vasculitis. Any child with severe asthma accompanied by systemic symptoms should be evaluated by a pediatric specialist.
Should I see a doctor if I have asthma and some of these symptoms?
Yes, you should consult a healthcare provider if you have asthma along with other concerning symptoms such as numbness or tingling in your extremities, persistent sinus problems, unexplained rashes, extreme fatigue, or any cardiac or gastrointestinal symptoms. Early evaluation is particularly important if your asthma has suddenly worsened or become difficult to control with standard treatments.
What type of doctor treats Churg-Strauss syndrome?
Churg-Strauss syndrome is typically managed by a rheumatologist (specialist in autoimmune and inflammatory diseases) in collaboration with other specialists depending on organ involvement. This may include pulmonologists for lung issues, cardiologists for heart problems, neurologists for nerve damage, and dermatologists for skin manifestations. A multidisciplinary approach often provides the best outcomes.
References:
- Mayo Clinic – Churg-Strauss Syndrome
- Johns Hopkins Medicine – Eosinophilic Granulomatosis with Polyangiitis
- Vasculitis Foundation – EGPA (Churg-Strauss)
- American College of Rheumatology – EGPA
- National Organization for Rare Disorders – Churg-Strauss Syndrome
The information on this page is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always consult a qualified healthcare provider before making decisions related to your health.
Read the full Disclaimer here →