Interstitial Lung Disease May Trigger Microscopic Polyangiitis in Some Patients, Case Report Suggests

Interstitial Lung Disease May Trigger Microscopic Polyangiitis in Some Patients, Case Report Suggests

Interstitial pneumonia may trigger the production of anti-neutrophil cytoplasmic antibodies (ANCA) and lead to a kind of vasculitis called microscopic polyangiitis, a case report shows.

The finding suggests that interstitial pulmonary fibrosis may be a cause, rather than a consequence, of ANCA-associated vasculitis.

The study, “Idiopathic Interstitial Pneumonia as a Possible Cause of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A Case Report,” was published in the Archives of Rheumatology.

Interstitial lung diseases (ILD) — those that cause scarring of lung tissue — often are observed in patients with ANCA-associated vasculitis (AAV). However, studies assessing the relationship between both diseases have produced conflicting results.

While some believe ILD may be the cause of AAV, others suggest that myeloperoxidase (MPO) autoantibodies — a hallmark of AAV — cause the fibrotic tissue to proliferate.

Japanese researchers presented the case of a 72 year-old woman who developed rapidly progressing kidney inflammation (glomerulonephritis) during the course of idiopathic (unexplained) interstitial pneumonia.

Doctors first suspected interstitial pneumonia during a health checkup when she was 69. Additional analysis did not show any cause for the scarring tissue and the patient was diagnosed with idiopathic interstitial pneumonia. The patient was negative for MPO antibodies.

At age 71, the patient complained of joint pain and was suspected of having rheumatoid arthritis, after her rheumatoid factor came up elevated in blood analysis. MPO levels also were high.

While anti-inflammatory and immunosuppressive treatment reduced her symptoms, her condition continued to progress, and she was again admitted to the hospital one year later. At this point, she had swollen legs, but no other noticeable physical changes.

New blood work revealed signs of inflammation and kidney failure. Also, MPO autoantibodies were even higher than in prior analysis. An analysis of the kidneys revealed damaging glomerulonephritis, which supported a final diagnosis of microscopic polyangiitis (MPA).

Meanwhile, researchers reviewed her lung analysis and found that the pulmonary fibrosis had been  progressing slowly for years.

She started treatment with methylprednisolone, followed by high-dose prednisolone and intravenous cyclophosphamide. Four months later, inflammation markers were close to normal and her kidney function had improved.

“Along with progression of idiopathic interstitial pneumonia, the test for MPO-ANCA was converted to be positive, and subsequently ANCA-associated renal vasculitis, namely MPA, developed,” the researchers wrote.

This is the tenth known case of patients who develop MPA disease after an initial idiopathic interstitial pneumonia diagnosis. The team believes that “interstitial pulmonary fibrosis may be a cause, rather than a result, of the production of ANCA, and may even be a cause of MPA.”

One comment

  1. Adriana Carevic says:

    with 2 years I got the first lung inflammation and with 4 others. I was given penicillin. In the course of the treatment, the physician has taken the parents to failure of healing, how much lung inflammation was life-threatening. After that, I began to have problems with hypothermia and this was common throughout my life. Now after extensive blood tests I can see that I’ve had LUPUS activated several times in my life. My life-threatening hypothermia appears to be the result of hypoglycemia due to chronic inflammation of the pancreas. Every time I go on a strict diet, the symptoms of LUPUSA have diminished and become tolerable. RTG lungs show two nodules which are morphologically scarred, a small bronchial deformation in diameter of 8mm and hyperinflation is seen on 09.03.2017. Because of the oscillation of blood glucose because it suddenly falls: for 15 minutes from 6.8 to 5.8 and for 15 minutes from 5.8 at 4.8 and again for 15 minutes from 4.8 to 3.8 that is 1 hour after the blood glucose drops to 3.8. my carotid artery stenosis is diagnosed by 30%. index of arteriosclerosis = 3. reumatoid factor = 411. alkaline phosphatase 56. amylase = 156. osteoporosis 30% .AST = 29, ALT = 37, GGT = 19, (bo) an9i-Ro-52 (TRIM21) antibody = antibodies to SS-A (Ro (antigen 3+, antibodies to SS-B (La) antigen = 1+, antibodies to DFS70 antigen = 2+) I have a low pressure, 100/50.
    My opinion is that LUPUS endangers all organs at the same time but the damage depends on the length of the acute inflammation and on the type of inflammation. In my acute LUPUS 1983. when my monocytes were low and lymphocytes elevated I had problems with the swallowing. I received explicit pigmentation and depigmentation on the face of the year, and since then I have great problems with staying in the sun: I get a strong rash within a couple of minutes and pressure on the patient completely deorient. how I’m sensitive to the sun and the cold and how I have to take meals every hour and how these meals must be freshly prepared, dietetic: I almost do not get out of the house because I feel everyday on several occasions weak. In the CT of my heart I have Pulmonary hypertension and (S) NT-proBNP = 144

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