Overview

An autoimmune condition known as renal vasculitis, or ANCA glomerulonephritis, makes your white blood cells attack the glomeruli, or the small blood arteries that filter blood in your kidneys. Capillaries, which are blood vessels, get swollen and are harmed. Protein and blood seep into the urine when the glomeruli cannot filter the blood, which can cause kidney damage or even kidney failure in extreme cases.

Different Forms of Renal Vasculitis

Renal vasculitis encompasses various forms, each involving inflammation of the blood vessels in the kidneys. Some common forms include -

- Granulomatosis with Polyangiitis (GPA). Previously known as Wegener's granulomatosis, GPA primarily affects small to medium-sized blood vessels and can lead to kidney damage.
- Microscopic Polyangiitis (MPA). MPA involves inflammation of small blood vessels, including those in the kidneys, and often causes kidney problems.
- Eosinophilic Granulomatosis with Polyangiitis (EGPA). EGPA typically affects small to medium-sized vessels and can impact the kidneys, among other organs.
- Polyarteritis Nodosa (PAN). While PAN primarily affects medium-sized arteries, it can lead to renal artery involvement and kidney issues.
- Immune Complex Vasculitis. This category includes conditions such as lupus nephritis, where immune complexes deposit in the kidneys, causing inflammation.
- Antineutrophil Cytoplasmic Antibody (ANCA). Associated Vasculitis: Both GPA and MPA are examples of ANCA-associated vasculitis, where ANCA antibodies target blood vessel walls, including those in the kidneys.

Causes of Renal Vasculitis

- Genetic Predisposition: Some individuals may have a genetic predisposition to autoimmune diseases, making them more susceptible to vasculitis.
- Infections: Certain infections, such as hepatitis B and C, can sometimes trigger vasculitis.
- Environmental Factors: Exposure to environmental toxins or pollutants may play a role in developing vasculitis in genetically susceptible individuals.
- Medications: Some medications have been associated with an increased risk of vasculitis, although this is relatively rare.
- Other Autoimmune Diseases: Individuals with autoimmune conditions such as rheumatoid arthritis, systemic lupus erythematosus, or Sjögren's syndrome may risk developing vasculitis.

Symptoms of Renal Vasculitis

Some people only show signs of the condition once it progresses. When symptoms do show up, they include a rash on the buttocks or lower legs, stomach discomfort, and blood in the urine.

Symptoms of renal vasculitis can vary depending on the specific form and severity of the condition. Common signs and symptoms of renal vasculitis may include

- Hematuria: Blood in the urine is a hallmark symptom, often appearing as pink, red, or brown urine.
- Reduced Urine Output: Decreased urine output or oliguria can occur due to kidney dysfunction.
- Proteinuria: Excess protein in the urine may lead to foamy or frothy urine.
- High Blood Pressure: Hypertension is common in renal vasculitis and may be difficult to control.
- Fatigue: Generalised weakness and fatigue are common, often due to kidney dysfunction and anaemia.
- Joint Pain: Some individuals may experience joint pain and stiffness, especially in forms such as GPA and MPA.
- Swelling (Edema): Edema may develop in the legs, ankles, or face due to fluid retention.
- Skin Rash: Skin rashes or sores may develop in certain types of vasculitis.
- Abdominal Pain: Abdominal pain or discomfort can occur in certain forms, such as polyarteritis nodosa (PAN).
- Fever: A low-grade fever may be present in some cases.
- Nerve Problems: In some cases, vasculitis can affect nerves, leading to symptoms such as numbness, tingling, or weakness.

Treatment Approaches to Renal Vasculitis

A prolonged "maintenance phase, or relapse prevention phase," which begins once remission has been reached, follows the 3-6 month "induction phase" in treating kidney vasculitis to reduce inflammation and avoid long-term organ damage quickly. High steroids are the first induction therapy with cyclophosphamide and rituximab, two immunosuppressive medications.

If the level of kidney function is abysmal (creatinine > 500 mol/l), a different therapy termed plasma exchange is frequently employed. Once remission is attained, less aggressive therapy is utilised, consisting of low-dosage steroids, occasional rituximab infusions, and either azathioprine or rituximab if a patient is at high risk of recurrence. This therapy may last up to two years.

- Medicines - Your doctor may provide immune system suppressants such as steroids, rituximab and cyclophosphamide to lower your immune system activity and lessen vasculitis' swelling and damage.
- Exchange of total plasma (TPE) - The blood-cleansing procedure known as total plasma exchange, or plasmapheresis, extracts your blood using a tiny catheter (thin, flexible tube) or needle. Your body receives the cleansed, separated plasma. TPE is utilised in conjunction with other therapies to treat glomerular disorders.
- Renal Replacement Therapy - This treatment is temporary and even may need permanent dialysis. The patients will benefit from renal transplant.

When to consult a Doctor

You can also get in touch with the expert Nephrology doctors at Narayana Healthcare based in your city to get immediate attention and medical support during injuries, health disorders or any other health concern.

Conclusion

The kidneys are highly specialised organs that continually filter and regulate the components of your blood. They include millions of small blood capillaries. Unfortunately, these blood vessels are highly susceptible to attack with ANCA vasculitis. The key elements to enhancing outcomes are prompt diagnosis and early therapy. Though most individuals do not develop end-stage kidney disease, those who do can sustain their kidney function by dialysis. Renal transplantation is an effective therapy option for those who are healthy enough. Overall, prognosis depends on time of presentation, early consultation improves outcomes.