Retroperitoneal Fibrosis

What is Retroperitoneal Fibrosis?

Retroperitoneal fibrosis is a rare immune-mediated condition in which an inflammatory, fibrous process forms around the aorta in the space behind the abdominal cavity.^{[1, 2]} The tissue can compress or encase structures such as the ureters, the aorta and the inferior vena cava.^[1]

It needs care from specialists such as a nephrologist, rheumatologist or urologist.

Common symptoms

It may cause no symptoms, but it typically causes dull pain or discomfort in the abdomen, back, flank or groin.^[1] Other common but nonspecific signs are high blood pressure and some degree of kidney function change.^[1]

Complications can include acute or chronic kidney injury, blockage of the vena cava and deep vein thrombosis.^[2] The condition is often found by chance on a scan done for another reason.^[1]

Causes and risk factors

Most cases are idiopathic, meaning no cause is found. Idiopathic retroperitoneal fibrosis can occur alone or as part of IgG4-related disease, and it often arises in people who have other autoimmune conditions.^{[2, 3]} Genetic, environmental and lifestyle factors all contribute.^[2]

Secondary forms follow other causes, such as medicines, malignancies, infections and histiocytic disorders.^{[1, 2]}

How it is diagnosed and treated conventionally

Diagnosis is based on CT or MRI scans, and PET scanning can help in staging and follow-up. A tissue biopsy is sometimes needed.^{[1, 3]}

Medical therapy is the cornerstone of treatment. Glucocorticoids are effective, B-cell-targeting drugs are increasingly used, and relapses are frequent after treatment stops.^[2] In an open-label randomized trial of 60 adults with idiopathic disease, low-dose prednisone plus methotrexate was non-inferior to standard-dose prednisone for remission at 9 months, with a lower total steroid dose.^[4] Stents, drainage or surgery may be needed to relieve a blocked ureter.^[2]

Why Retroperitoneal Fibrosis calls for a whole-person approach

Retroperitoneal fibrosis involves the immune system, the kidneys and blood vessels, so no single factor explains how a person feels. The disease and its treatments, which are often long, can each add symptoms.^[2]

Supportive care addresses the effects that remain, in coordination with your specialists.

Immune-mediated inflammation

The disease is driven by an interplay of B and T lymphocytes, along with macrophages, eosinophils and mast cells, which eventually promote fibroblast activation and scarring.^{[2, 3]} This is why treatment relies on immune-suppressing drugs.

Kidney involvement

Blocked ureters are the most common complication and can lead to kidney failure.^[3] Kidney health shapes which herbs and medicines are safe, and it is the main reason for caution with herbal products on this page.^[5]

Pain and discomfort

Dull pain in the back, abdomen or flank is typical.^[1] Pain is one of the symptoms for which supportive therapies are commonly sought.

Long-term treatment and stress

Relapses are frequent after treatment ends, so many people live with long-term monitoring and medicines.^[2] The strain of this, along with the effects of steroids, can affect sleep and mood.

Acupuncture as supportive care in Retroperitoneal Fibrosis

What the research shows

No clinical trials of acupuncture for retroperitoneal fibrosis were found. The evidence for related symptoms in other conditions is limited and uneven.

For pain, a pooled analysis of 39 trials with 20,827 patients found acupuncture better than sham and no acupuncture for chronic musculoskeletal pain, osteoarthritis and headache.^[6] A Cochrane review of chronic nonspecific low back pain, which excludes pain with a known cause such as this one, found low-certainty evidence that acupuncture gave only small pain relief compared with sham, but more relief compared with no treatment.^[7] These findings do not apply directly to pain from retroperitoneal fibrosis.

In chronic kidney disease, a Cochrane review of 24 studies found only very low quality evidence for short-term effects of acupressure on fatigue, depression and sleep in people on hemodialysis. The review could not judge safety or the effects of other acupuncture types.^[8]

How acupuncture may work

How acupuncture might act in retroperitoneal fibrosis has not been studied. We do not claim that it affects the inflammation, the fibrous tissue or kidney blockage.

What treatment involves

Treatment uses fine, sterile, single-use needles placed at selected points, usually left in for about 20 to 40 minutes. A course is typically several visits, with reassessment to see whether pain, sleep or fatigue is changing.

In prospective studies, about 9 in 100 patients had a minor reaction such as brief bleeding, soreness or redness 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 or have a clotting problem or deep vein thrombosis, have a ureteral stent or a nephrostomy tube, take steroids or other immune-suppressing medicines, are pregnant, or have a pacemaker (relevant to electroacupuncture). Because immune-suppressing treatment can lower resistance to infection, we ask that your physician knows you are having needle treatment.

Ayurvedic medicine as supportive care in Retroperitoneal Fibrosis

The Ayurvedic view

Ayurveda has no classical name for retroperitoneal fibrosis. Practitioners may describe deep, long-standing inflammation and hardening of tissue in terms of ama (poorly processed material), imbalance of the doshas, and obstruction of the channels, including those that carry urine. These are traditional explanatory frameworks, not biomedical diagnoses.

Therapies that may be used

For supportive care, an Ayurvedic practitioner may suggest:

• Regular meals and a steady daily routine to support sleep and energy
• Gentle breathing practices and relaxation
• Warm oil massage over the back, avoiding any area with a stent or tube
• Individually chosen herbs, only after review of your kidney function and medicines

Panchakarma is not appropriate during pregnancy, acute illness or frailty, and it is not suitable when there is active disease, a blocked ureter, impaired kidney function or high-dose immune suppression.

What the research shows

No clinical studies of Ayurvedic treatment for retroperitoneal fibrosis were found, so there is no clinical evidence. Because the condition is rare and treated with strong prescription drugs, Ayurvedic claims about it should be viewed with caution. Product quality is a real concern: about one fifth of Ayurvedic medicines bought online contained detectable lead, mercury or arsenic.^[10] Heavy metals and other contaminants are among the recognized ways herbal products can injure the kidneys.^[5]

Herbal medicine as supportive care in Retroperitoneal Fibrosis

Herbs and formulas that have been studied

No herbal formula has been tested in a clinical trial for retroperitoneal fibrosis. The only reports are single cases. One case described a man with IgG4-related disease whose scan changes cleared with steroids, who then took the Japanese Kampo formula Hochuekkito as maintenance because of steroid complications, and who had not relapsed after 3 years.^[11]

What the research shows

The evidence is very limited. A single case with no comparison group cannot show that the formula prevented relapse, since the disease had already responded to steroids. We found no controlled studies of any herb or formula, and herbs are not a substitute for steroids or other prescribed medicines.

Safety and herb-drug interactions

Kidney safety is the main concern. Herbal medicines can injure the kidneys through intrinsic toxicity, wrong processing, contamination or adulteration, and interactions with medicines.^{[5, 12]} Aristolochic acid, found in some Chinese herbs, is a known cause of kidney injury and may raise the risk of urinary tract cancers.^[12] Because the kidneys may already be stressed by ureter blockage, we would avoid herbs with known kidney toxicity.

Reviews of herb-drug interactions describe, mostly from case reports, bleeding when herbs such as danshen, ginkgo, dang gui and ginger are combined with warfarin (and ginkgo with aspirin), and St. John's wort lowering levels of immune-suppressing drugs such as ciclosporin and tacrolimus.^{[13, 14]} Herbal and dietary supplements account for about 20% of drug-induced liver injury cases in the United States, which matters for people taking methotrexate or azathioprine.^[15]

Herbs should come from tested sources and be prescribed individually. Tell us if you are pregnant or breastfeeding.

Translating traditional medicine into modern biological language

This is interpretation, not equivalence. Chinese medicine may describe hardened masses and obstruction as blood stasis and phlegm accumulation, and Ayurveda may speak of ama and blocked channels. Researchers describe related ideas such as chronic inflammation, activated fibroblasts and scarring that narrows the ureters.^{[2, 3]} No study has shown that the traditional patterns correspond to these processes.

The realistic overlap is at the symptom level, such as pain, sleep and stress, which are measured with questionnaires in studies of other conditions.

How this care fits with your medical care

Complementary care for retroperitoneal fibrosis is supportive only and begins after the condition is under medical management. 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 steroids, because relapses are frequent after treatment stops.^[2] We do not diagnose or monitor this condition. Please bring your diagnosis, recent scans and kidney tests, a full list of medicines and supplements, and details of any stent, drain or surgery. Keep your follow-up visits with your specialists.

When to seek urgent medical care

Retroperitoneal fibrosis can block the kidneys or blood flow and needs prompt medical attention.^{[2, 3]}

• Little or no urine, or new difficulty passing urine
• New or worsening flank or back pain, especially with fever or chills
• New swelling in one or both legs, or leg pain with redness (possible blood clot)
• Sudden shortness of breath or chest pain
• Nausea, vomiting, confusion or marked fatigue, which can signal kidney failure
• Severe abdominal pain

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