A rare autoimmune disease has damaged the cartilage and bones in a woman’s nose, causing her nose to collapse and sink into her face.
When the 34-year-old was examined at the Bridal Facial Surgery Clinic, her nose was completely collapsed, causing her tip. nose According to a report published on April 5. New England Journal of Medicine (NEJM) The disorder has occurred over a seven-year period.
The woman had a number of symptoms that were involved, including ongoing symptoms. Inflammation In her nasal cavity and lining of the nasal passages, a symptom called “chronic rhinosinusitis”; and she also had a fleshy growth in the nasal cavity called “polyps.” Thin mucus drained from her nose, and the mucus was dry and bloody crusted inside of the nasal passages. her
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Physical examination revealed The “almost total loss” of a woman’s nose. boneWhich is located on either side of the bridge and severe disintegration of the abdominal wall cartilage In addition, computed tomography (CT) scans revealed that a large hole was formed in the septum.
To determine the cause of the severe inflammation, doctors run tests to identify. antibody Circulate in the blood of women They found high levels of antibodies that target S3 protein (PR3), a protein found in certain white blood cells. According to Mayo Clinic Laboratories.The antibodies that target PR3 are a type of autoantibody, meaning they attack healthy human cells and can trigger inflammation in Blood vessels And organ damage most often occurs with kidney, lung And respiratory tract
From the presence of PR3 antibodies in the woman, doctors diagnosed her as “granulomatosis with polyangiitis,” or GPA. Almost everyone with a GPA has These autoantibodies, according to the Mayo Clinic.
That said, “I still do not understand the exact role of autoantibodies These in the development of granulomatosis with polyangiitis ” According to the National Organization for Rare Disorders (Nord). One possibility is that these antibodies may bind to white blood cells that contain PR3 and cause them to malfunction and damage healthy tissue.
Estimating the frequency of The granulomatosis with polyangiitis varies greatly depending on the specific population studied, ”and often this disorder is not recognized by doctors, according to NORD. For this reason, it is difficult to estimate how many people are. That develop malfunctions each year But it is considered very rare
The GPA can occur slowly over several months or be determined quickly within days, according to the NORD.Symptoms can vary greatly from person to person. But can affect many organs throughout the body For example, severe cases can lead to hearing loss, vision loss, kidney failure, or cranial nerve damage if left untreated. Autoimmune disorders can cause a persistent runny nose, rhinitis, sinusitis, and a septum in the septum, as seen in affected women.
To treat a woman’s GPA, her doctor prescribed rituximab, an antibody treatment that targets immune cells called B cells to reduce the body’s malfunctioning immune response. According to Drugs.comPatients were also given low doses of cyclophosphamide, another immunosuppressant drug, and prednisone. Corticosteroid That also compresses immune activity
“At a follow-up six months later, the patient’s runny nose and crust were corrected,” according to NEJM, “the anomalies of the nostrils did not change.” Specify whether she will undergo surgery for a sunken nose.
Originally published in Live Science.