Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome)

What is EGPA?

Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss syndrome, is a rare disease with inflammation and damage of small and medium blood vessels. It is one of the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides, and it is set apart from the others by its link to asthma, rhinosinusitis and a high eosinophil count in the blood.^[1]

Eosinophils are a type of white blood cell. In EGPA they build up in the blood and in tissues and are associated with inflammation and organ damage.

Common symptoms

Most people have asthma, often adult-onset and sometimes severe, along with sinus disease and nasal polyps. The classic description is of three overlapping phases: an allergic phase dominated by asthma and sinus symptoms, an eosinophilic phase with lung and tissue involvement, and a vasculitic phase.^[1]

Symptoms depend on which organs are involved:

• Cough, wheeze and shortness of breath, with shifting patches on lung imaging
• Numbness, tingling, pain or weakness from nerve damage, often in a hand or foot
• Skin rashes, purple spots or sores
• Abdominal pain, nausea or vomiting
• Heart involvement, such as heart failure or pericarditis, and, less often, kidney disease^[2]

Causes and risk factors

The cause is not fully understood. It probably involves an interaction between T cells, B cells and eosinophils.^[2] A genetic tendency toward autoimmune disease and environmental triggers have been suggested, but no single trigger can be identified in an individual person. Two broad forms are recognized. One is ANCA-positive, with more vasculitic features such as kidney inflammation, purpura and nerve damage. The other is ANCA-negative, with more eosinophilic features such as lung infiltrates and heart muscle disease.^[2]

How it is diagnosed and treated conventionally

Diagnosis is made by physicians, usually rheumatologists, from the combination of asthma, blood eosinophil count, nasal polyps, nerve involvement, ANCA tests, imaging and sometimes a biopsy. Current classification criteria give points for eosinophil count, obstructive airway disease, nasal polyps, nerve involvement and biopsy findings.^[1] European guidelines stress an integrated, multidisciplinary approach.^[3]

There is no single standard therapy. Corticosteroids are almost universally used, with other immune-suppressing drugs added in many people, and interleukin-5 inhibitors have been a welcome advance.^[4] In a trial of 136 people with relapsing or refractory EGPA, mepolizumab led to more weeks in remission than placebo and allowed lower steroid doses, although only about half of those treated reached remission.^[5] The overall survival is good, but relapses and ongoing symptoms are frequent.^[2]

Why EGPA calls for a whole-person approach

EGPA is an immune-driven, multi-organ disease that must be managed medically, and several things beyond the disease itself shape how a person feels. Asthma, sinus problems, nerve symptoms, sleep, stress and treatment side effects all affect daily life.

This is where supportive care may fit. It does not treat the vasculitis. It may help with comfort while your physicians treat the disease.

Eosinophilic inflammation and the airways

Asthma and sinus disease are core features and usually continue to need treatment.^[1] Because they come from the same eosinophil-driven process, asthma control is reviewed by your specialists as part of EGPA care.

Nerve involvement

Nerve damage is common, and it can cause numbness, tingling and pain.^[2] This damage comes from inflamed vessels supplying the nerves, so it needs prompt medical attention rather than symptom care alone.

Long-term steroid exposure

Most people take corticosteroids for a long time. Long courses of glucocorticoids carry a risk of bone thinning, and guidelines advise assessing fracture risk soon after starting steroids.^[6] Steroid-sparing treatment is one of the main goals of newer drugs.^[5]

Stress, sleep and living with relapses

Because EGPA often relapses and symptoms can persist, many people live with worry, disturbed sleep and fatigue.^[2] Acupuncture and some herbs have been studied for stress and sleep in other groups, as described below.

Acupuncture as supportive care in EGPA

What the research shows

No clinical trials of acupuncture in EGPA were found in PubMed. The evidence below concerns related symptoms in other patient groups and cannot be assumed to apply to EGPA. Acupuncture does not treat the vasculitis.

Asthma is the closest studied condition. A meta-analysis of 16 sham-controlled trials in adults with asthma found acupuncture improved lung function (FEV1%), quality of life and symptom scores compared with sham. The trials varied widely, other lung function and medication-use measures did not change significantly, and the authors called for larger, better-controlled trials.^[7] A meta-analysis of 21 trials of acupuncture added to conventional care found better response rates and lung function than conventional care alone, but the authors noted limitations in the included studies.^[8] Asthma in EGPA has an eosinophilic, vasculitis-linked cause, so these results may not carry over.

For nerve symptoms, the available reviews are about nerve damage from chemotherapy. A network meta-analysis of 33 studies (2,027 participants) found acupuncture-related treatments improved neuropathy symptoms, pain and quality of life in that group.^[9] Nerve damage in EGPA has a different cause and needs medical treatment first.

For sleep, a review of 24 trials in insomnia included a meta-analysis of 15 trials that found acupuncture improved sleep scores compared with medication, with benefit seen after about three weeks.^[10] An individual-patient meta-analysis of 39 trials in musculoskeletal pain, osteoarthritis, headache and shoulder pain found acupuncture better than sham and than no acupuncture for pain.^[11]

How acupuncture may work

A review of laboratory and clinical research proposes that acupuncture acts through the nervous, hormonal and immune systems together. The authors describe this as a hypothesis, and it has not been shown to alter the course of EGPA.^[12]

What treatment involves

Treatment uses fine, sterile, single-use needles, usually left in place for about 20 to 40 minutes, with a short course of visits and reassessment. We would aim treatment at comfort, sleep and stress rather than the disease.

In prospective studies, about 9 in 100 patients had a minor reaction such as soreness, bleeding or bruising at a needle site, and serious events occurred in about 1 in 10,000 patients.^[13] Tell the practitioner first if you take immune-suppressing medicine, since infection risk matters, and if you have skin lesions or purpura, a bleeding disorder, take blood thinners, are pregnant, or have a pacemaker.

Ayurvedic medicine as supportive care in EGPA

The Ayurvedic view

Ayurveda has no classical name for EGPA. Wheezing breathlessness is traditionally linked to Kapha and Vata in the chest, and skin and blood inflammation to Pitta and disturbed blood tissue (rakta). These are traditional categories, not biomedical diagnoses.

Therapies that may be used

In a supportive role, care is chosen for the individual and may include:

• Diet and daily routine suited to the person's constitution, with regular sleep hours
• Breathing and relaxation practices, including gentle yoga where your physicians agree
• Herbs such as ashwagandha or turmeric, prescribed individually after review of your medicines

Panchakarma cleansing procedures are not appropriate during pregnancy, acute illness or frailty. They are also generally unsuitable during disease flares, with heart or lung involvement, or while taking immune-suppressing medicine.

What the research shows

No Ayurvedic trials in EGPA were found, so there is no evidence of benefit for the disease. Research exists for some related symptoms. A Cochrane review of 15 trials (1,048 people) in asthma found moderate-quality evidence that yoga probably leads to small improvements in quality of life and symptoms. Lung function was not significantly changed, and most trials were in people with mild to moderate asthma.^[14]

For stress, a meta-analysis of 9 trials (558 people) found ashwagandha extracts lowered stress and anxiety scores and blood cortisol compared with placebo. Four trials reported mild to moderate adverse events, and long-term safety is not established.^[15] These trials were not in people with EGPA.

Herbal medicine as supportive care in EGPA

Herbs and formulas that have been studied

The only EGPA-specific report is a 2010 case report of two people, one with Churg-Strauss syndrome and one with a different vasculitis, treated with Kampo medicines selected under Chinese medicine theory after conventional treatment had worked poorly. The authors reported improved blood tests and quality of life.^[16]

For nerve symptoms, a review of 22 clinical studies of plant-derived medicines found the most evidence for cannabis, linseed oil, capsaicin and a Japanese formula, and only one trial each for chamomile, turmeric and colocynth. These studies were in diabetic, chemotherapy-related and other neuropathies, not EGPA.^[17]

What the research shows

The evidence for herbs in EGPA is very limited: two uncontrolled case reports, which cannot show that a treatment caused any change.^[16] The evidence for nerve symptoms comes from other causes and needs more studies.^[17]

Our role is limited to supportive care. We do not suggest any herb as a way to control eosinophils or the vasculitis.

Safety and herb-drug interactions

This is the most important section for this condition, because most people with EGPA take immune-suppressing drugs. Herbs and prescription drugs can interact. Published cases include St John's wort lowering blood levels of cyclosporin and tacrolimus, with organ rejection reported when cyclosporin levels fell.^[18] Herbs described as immune-stimulating may also work against the purpose of your treatment, so each herb must be reviewed with your prescriber.

Herbal and dietary supplements account for about 20% of drug-induced liver injury cases in the United States, which matters if you take methotrexate, azathioprine or other drugs that affect the liver.^[19] In one study, about one fifth of Ayurvedic medicines bought online contained detectable lead, mercury or arsenic.^[20] Use only tested products prescribed individually, and tell us if you are pregnant or breastfeeding or have liver or kidney disease.

Translating traditional medicine into modern biological language

This is interpretation, not equivalence. Chinese medicine may describe EGPA-type illness through patterns such as blood stagnation, damp-heat or yang deficiency, which are the patterns the Kampo case report used to choose its medicines.^[16] Researchers instead study eosinophils, immune signaling and inflammation, and some propose that acupuncture acts on the nervous, hormonal and immune systems.^[12]

In Ayurveda, aggravated Kapha, Pitta and disturbed rakta describe congestion, heat and blood-vessel inflammation. They might loosely parallel airway and vascular inflammation, but no study has shown that a dosha corresponds to a biological process or that either framework changes the immune process in EGPA.

How this care fits with your medical care

Supportive care here is complementary. EGPA needs specialist treatment, and we ask that you are under a physician's care for it and that your treating team knows you are receiving complementary care. Do not stop or change prescribed steroids, immune-suppressing drugs, biologic injections or inhalers without your prescriber.

We do not diagnose or manage the disease. Please bring your diagnosis, ANCA and eosinophil results, a full list of medicines and supplements, and the names of your specialists. Report new or worsening symptoms to your physicians rather than waiting for a visit with us.

When to seek urgent medical care

EGPA can damage the heart, nerves, lungs and kidneys, so some changes need prompt medical assessment.^[2]

• Chest pain, a racing or irregular heartbeat, or new swelling of the legs
• Worsening shortness of breath, wheeze not relieved by your usual inhaler, or coughing blood
• New weakness, a foot or hand that drags or goes limp, or sudden numbness
• Blood in the urine, or severe abdominal pain or bloody stools
• New purple spots, skin sores or fever with a flare of symptoms
• Sudden vision changes or severe headache

For severe breathing difficulty, chest pain or any emergency, call 911 or go to the nearest emergency department.