How is microscopic polyangiitis diagnosed? How is microscopic polyangiitis diagnosed? Can you die from microscopic polyangiitis? Let’s find out about microscopic polyangiitis symptoms and treatment guidelines!
Microscopic Polyangiitis Definition
Microscopic polyangiitis meaning: What is microscopic polyangiitis? Microscopic polyangiitis is a pauci-immune non-granulomatous necrotizing vasculitis that affects small blood vessels (capillaries, venules, or arterioles), often leads to glomerulonephritis and pulmonary capillaritis, and is often linked to ANCA on immunofluorescence testing (directed against MPO, a constituent of neutrophil granules).
Microscopic Polyangiitis Causes
What causes microscopic polyangiitis? What is the most common cause of microscopic polyangiitis? No one knows what causes MPA. MPA isn’t cancer, and it’s not contagious.
Microscopic polyangiitis is similar to polyarteritis nodosa and granulomatosis with polyangiitis because it can affect both small and large blood vessels and tends to affect capillaries in the lungs and kidneys.
In rare cases, some medications, like propylthiouracil, hydralazine, allopurinol, penicillamine, minocycline, and sulfasalazine, can cause systemic vasculitis with high levels of p-ANCA and signs of microscopic polyangiitis.
Microscopic Polyangiitis Diagnosis
- Necrotizing vasculitis of small and medium-sized arteries and veins.
- The most common cause of pulmonary-renal syndrome (is diffuse alveolar hemorrhage and glomerulonephritis).
- ANCA in 75% of the cases.
Microscopic Polyangiitis Symptoms
Signs and symptoms of microscopic polyangiitis – What are some symptoms of microscopic olyangiitis?
Microscopic polyangiitis can cause a wide range of symptoms that point to vasculitis of small blood vessels. These include purpura that can be touched and other signs of cutaneous vasculitis (ulcers, splinter hemorrhages, vesiculobullous lesions).
The most common cause of pulmonary-renal syndromes is microscopic polyangiitis, which is several times more common than anti-glomerular basement membrane disease. Pulmonary hemorrhage can happen with the typical pathological signs of capillaritis. Interstitial lung fibrosis that looks like usual interstitial pneumonitis may be part of the presenting condition and is a sign of a bad prognosis.
In microscopic polyangiitis, vascular neuropathy (mononeuritis multiplex) is also common.
Laboratory Findings
Three-quarters of people with microscopic polyangiitis have ANCA, usually anti-myeloperoxidase antibodies (MPO-ANCA) that show up as a p-ANCA pattern on immunofluorescence testing. In addition, PR3-ANCA, which works against proteinase-3, can also be seen.
Acute-phase reactants that are high are a sign of active disease. In addition, there may be microscopic amounts of blood, protein, and red blood cells in the urine. The problem with the kidney is segmental necrotizing glomerulonephritis, which is often accompanied by localized intravascular coagulation and intraglomerular thrombi when a kidney biopsy is done.
Differential Diagnosis
It might be hard to tell this disease apart from granulomatosis with polyangiitis. However, microscopic polyangiitis is not related to granulomatosis with polyangiitis, which often has a disease that causes damage to the upper respiratory tract and lasts for a long time.
Also, as was already said, granulomatous inflammation is not present in microscopic polyangiitis, which is a major difference between the two diseases. Microscopic polyangiitis and polyarteritis nodosa must also be told apart because their treatments may differ.
Microscopic Polyangiitis Treatment and Management Guidelines
Treatment of microscopic polyangiitis – What is the best treatment for microscopic polyangiitis?
Microscopic polyangiitis is treated the same way as granulomatosis with polyangiitis: patients with severe disease, which usually includes pulmonary hemorrhage and glomerulonephritis, need urgent induction treatment with corticosteroids and either cyclophosphamide or rituximab.
If cyclophosphamide is chosen, it can be taken orally daily or in short bursts (usually once a month) through an IV. If remission is achieved, treatment should continue with azathioprine, rituximab, or methotrexate (provided the patient has normal kidney function).
In cases of drug-induced MPO-ANCA-associated vasculitis, the offending drug should be stopped. Immunosuppressive therapy is needed when major organs are affected (e.g., pulmonary hemorrhage, glomerulonephritis).
I hope you understand microscopic polyangiitis symptoms and treatment guidelines.