A rare autoimmune disorder decimates cartilage and bone in a woman’s nose, causing her nose to collapse and sink into her face.
When the 34-year-old woman checked into a plastic surgery clinic on her face, her nose fracture had completely collapsed, causing the tip of her nose according to a report published on April 5 in New England Journal of Medicine (NEJM). The deformity had occurred within seven years.
The woman had a number of related symptoms, including persistent inflammation in her nasal cavity and the lining of her sinuses, a condition called “chronic rhinosinusitis”
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A physical examination revealed an “almost total loss” of the woman’s nose bones, located on either side of the bridge, and severe degradation of her septal cartilage. In addition, a computed tomography (CT) scan showed that a large hole had formed in the septum.
To investigate the cause of the violent inflammation, doctors ran a test to identify antibodies circulating in the woman’s blood. They found high levels of antibodies targeted to proteinase 3 (PR3), a protein found in certain white blood cells. according to Mayo Clinic Laboratories. Antibodies targeted to PR3 are a type of autoantibody, meaning that they attack healthy human cells and they can trigger inflammation in veins and causes organ damage, often on kidneys, lungs and the respiratory tract.
Based on the presence of PR3 antibodies in the woman, the doctor diagnosed her with “granulomatosis with polyangiitis” or GPA. Almost all people with GPA carry these autoantibodies, according to the Mayo Clinic.
That being said, “the exact role that these autoantibodies play in the development of granulomatosis with polyangiitis is not understood,” according to the National Organization for Rare Disorders (NERD). One possibility is that these antibodies can be locked onto white blood cells that carry PR3 and somehow cause them to function and damage healthy tissues.
“Estimates of the incidence of granulomatosis with polyangiitis vary widely depending on the specific population being studied,” and often the disease is not recognized by physicians, according to NORD. Because of this, it is difficult to estimate how many people develop the disease each year, but it is considered very rare.
GPA can occur slowly over many months or go away quickly in a few days, according to NORD. The symptoms vary greatly from person to person, but can affect organs throughout the body. For example, severe cases can lead to hearing loss, vision loss, kidney failure or cranial nerve damage if left untreated. The autoimmune disorder can also cause a persistent runny nose, runny nose, sinusitis and perforations – holes – in the septum, as seen in the affected woman.
To treat the woman’s GPA, her doctors prescribed rituximab, an antibody treatment targeted at immune cells called B cells, to slow down the body’s failing immune response. according to Drugs.com. The patient also received low dose cyclophosphamide, another immunosuppressive agent, and prednisone, a corticosteroid which also suppresses immune activity.
“At a follow-up visit 6 months later, the patient’s bloody nasal discharge and crusting were resolved,” according to the NEJM report. “The nasal deformity is unchanged.” The patient continues to receive rituximab, but the report did not note whether she will have surgery for her sunken nose.
Originally published on WordsSideKick.com.
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