Granulomatosis with Polyangiitis

What is Granulomatosis with Polyangiitis?

Granulomatosis with polyangiitis (GPA), formerly called Wegener's granulomatosis, is a necrotizing inflammation of small and medium-sized blood vessels that typically involves the nose and sinuses, lungs and kidneys.^[1] It is one of the ANCA-associated vasculitides, a group of rare autoimmune diseases that also includes microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis.^{[1, 2]}

Common symptoms

Symptoms depend on which organs are affected. GPA commonly involves the upper respiratory tract, lungs, kidneys, skin, eyes and peripheral nerves.^[3]

• Long-lasting nasal congestion, sinus problems and nosebleeds
• Cough, shortness of breath or coughing up blood
• Blood or protein in the urine, or swelling from kidney involvement
• Fever, fatigue, weight loss, joint pain or skin lesions^{[1, 3]}

Causes and risk factors

The exact cause is unknown. Activation of neutrophils and of the complement system, along with inflammatory cells such as monocytes and macrophages, drives vessel damage, and granulomatous inflammation with multinucleated giant cells is a hallmark of GPA.^[3] Genetic susceptibility and environmental triggers such as infections are thought to contribute, although the full cause is not understood, and we do not offer advice on avoiding triggers.

How it is diagnosed and treated conventionally

Diagnosis combines symptoms, ANCA blood tests, kidney and urine tests, imaging and often a biopsy of affected tissue.^[4] Treatment is directed by specialists. For life-threatening or organ-threatening disease, guidelines recommend high-dose glucocorticoids with rituximab or cyclophosphamide, then tapering of the steroid and rituximab, azathioprine or methotrexate to maintain remission.^[4]

Outcomes have improved with earlier diagnosis and targeted biologic drugs, so that attention has shifted to long-term problems such as chronic kidney disease, cardiovascular disease and the side effects of glucocorticoids.^[3]

Why Granulomatosis with Polyangiitis calls for a whole-person approach

GPA is a serious systemic disease that needs a specialist team. Nothing described on this page treats the vasculitis.

The disease and its treatment can affect sleep, energy, mood and comfort, and these are the areas where supportive care may help alongside your medical care.

Immune-driven vessel inflammation

The disease is driven by neutrophil activation, complement and inflammatory cells that damage vessel walls.^[3] It is controlled with immune-suppressing medicines, which is why any supplement or herb that acts on the immune system should be reviewed by your specialist before use.^[4]

Treatment side effects

Reducing glucocorticoid exposure is a main goal, because glucocorticoid toxicity is a major source of long-term harm.^{[3, 4]} Immune-suppressing medicines generally lower resistance to infection, so any therapy that breaks the skin needs sterile technique and your physician's awareness. Ask your rheumatologist whether acupuncture is reasonable at your current level of treatment.

Kidney involvement

The kidneys are commonly affected, and chronic kidney disease is a long-term concern.^[3] Products that can harm the kidneys are therefore a particular worry.^[5]

Fatigue, pain and sleep

Fatigue, joint pain and fever are common general symptoms.^[1] Acupuncture has been studied for fatigue, sleep problems and chronic pain in other groups of patients, which is the basis for considering it as supportive care here.^{[6, 7, 8]}

Acupuncture as supportive care in Granulomatosis with Polyangiitis

What the research shows

We found no studies of acupuncture in GPA, and no evidence that it affects the vasculitis. Any benefit would be for symptoms, based on research in other conditions.

A meta-analysis of 9 trials (809 participants) in cancer survivors found less fatigue with real acupuncture than with sham acupuncture or usual care.^[6] A review of 24 trials in insomnia found that, in the 15 trials comparing acupuncture with medication, sleep quality scores improved more with acupuncture, with benefit appearing after about three weeks, though the studies varied a great deal.^[7] An individual patient data meta-analysis of 39 trials with adequately concealed allocation found acupuncture better than sham or no acupuncture for chronic musculoskeletal pain, osteoarthritis, headache and shoulder pain.^[8] These results come from different patients and may not apply to people with GPA.

How acupuncture may work

No research explains how acupuncture might act in GPA, and we found no evidence that it alters ANCA-driven vasculitis. Any effect on fatigue, sleep or pain would be a symptom-level effect, and the reviews cited above do not settle its mechanism.

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. Because immune-suppressing medicines raise infection risk, we would want your physician to know about acupuncture and would avoid needling over broken, inflamed or infected skin.

In prospective studies, about 9 in 100 patients had a minor reaction such as soreness or bleeding at a needle site, and serious events occurred in about 1 in 10,000 patients.^[9] Tell the practitioner first if you take blood thinners, are pregnant, have low blood counts or a bleeding problem, or have a pacemaker (relevant to electroacupuncture).

Ayurvedic medicine as supportive care in Granulomatosis with Polyangiitis

The Ayurvedic view

Ayurveda has no exact counterpart to GPA. Chronic inflammation of the blood vessels, sinuses and lungs is traditionally described through disturbance of the doshas and of blood tissue, in a way that differs from person to person. These are traditional categories, not biomedical diagnoses.

Therapies that may be used

Care is individualized and, in tradition, includes regular routines, easy-to-digest meals, rest and stress-reducing practices. Herbs, if considered at all, would be chosen one at a time by a qualified practitioner and only with your specialist's approval, from a tested source, because about one fifth of Ayurvedic medicines bought online contained detectable lead, mercury or arsenic.^[10]

Panchakarma is not appropriate during active disease, with immune-suppressing treatment, kidney or lung involvement, acute illness, frailty or pregnancy, and we would not use it for this condition.

What the research shows

There are no clinical studies of Ayurvedic treatment in GPA, and we found none in ANCA-associated vasculitis. Safety is the main practical concern, because contaminated products and herbal supplements can harm the liver and other organs.^{[10, 11]}

Herbal medicine as supportive care in Granulomatosis with Polyangiitis

Herbs and formulas that have been studied

We found no clinical studies of Chinese or other herbal medicine for GPA or ANCA-associated vasculitis. No herb or formula has been shown to treat it, and none is recommended here.

What the research shows

There is no evidence for herbal medicine in GPA, so we cannot say whether any herb helps or harms the disease. The disease is treated with drugs of known effect, and guidelines emphasize shared decisions between physicians and patients.^[4]

Safety and herb-drug interactions

Herbs can interact with medicines, including blood thinners such as warfarin and immune-suppressing drugs such as ciclosporin and tacrolimus.^[12] Herbs can also harm the kidneys, particularly Chinese herbal products containing aristolochic acid, and they can cause liver injury.^{[5, 11]} This matters in GPA, where kidneys are often involved and where immune-suppressing drugs are taken for years.^[3]

We would not offer any herb without your rheumatologist or nephrologist knowing about it. Tell us if you are pregnant or breastfeeding, or have liver or kidney disease.

Translating traditional medicine into modern biological language

The following is interpretation, not equivalence. Chinese medicine may describe a chronic inflammatory vessel disease with sinus and lung involvement in terms such as heat, toxin, phlegm and blood stasis. Researchers study neutrophil activation, complement and inflammatory cells instead, and these ideas are not interchangeable.^[3]

In Ayurveda, dosha disturbance and impaired blood tissue are used to describe chronic inflammation. No study has shown that these traditional patterns correspond to a specific biological process in GPA.

How this care fits with your medical care

Supportive care for GPA is complementary. For a condition like this, 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 medicines, including prednisone, rituximab, methotrexate or azathioprine, without your prescriber.

We do not diagnose GPA, monitor your disease or manage your medicines. Please bring your diagnosis, recent kidney and ANCA test results, a full list of medicines and supplements, and your specialist's contact details. Keep your follow-up appointments with your rheumatologist, and report any new symptoms to them promptly, since the disease can relapse after a period of calm. If a new symptom appears, such as a nosebleed, cough or change in urine, call your specialist before assuming it is a side effect of any complementary therapy. Tell every clinician involved in your care about each therapy you use, including supplements and teas.

When to seek urgent medical care

GPA can damage the lungs and kidneys quickly, and it remains a potentially lethal disease.^[3]

• Coughing up blood, or new or worsening shortness of breath
• Blood in the urine, sharply reduced urine, or new swelling of the face or legs
• New nosebleeds that are heavy or do not stop
• Sudden vision loss, red painful eye or new numbness or weakness
• Fever or signs of infection while on immune-suppressing medicine
• Chest pain, or a rapidly worsening rash or skin ulcer

If any of these occur, call 911 or go to the nearest emergency department.