A rare autoimmune disease destroyed the cartilage and bones of a woman’s nose, causing her nose to collapse and sink into her face.
When a 34-year-old woman checked in to a facial plastic surgery clinic, her nose bride completely collapsed, causing her tip. nose Withdraw according to a report released on April 5 New England Journal of Medicine (NEJM). The malformation appeared during the seven years.
Women showed a variety of related symptoms, including persistence inflammation In the inner layers of her nasal passages and sinuses, a condition called “chronic sinusitis”, she also showed fleshy growth in her nasal passages known as “polyps”. Thin mucus leaked from her nose, and dry blood mucus lining her nasal passages.
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Physical examination revealed “almost complete loss” of the woman’s nose Skeleton, On both sides of the pons, severe deterioration of the septal cartilage. In addition, computed tomography (CT) scans showed that a large hole was formed in the septum.
To investigate the cause of the prevailing inflammation, doctors performed tests to identify antibody Circulates female blood. They have discovered high levels of antibodies that target proteinase 3 (PR3), a protein found in certain white blood cells. According to the Mayo Clinic Institute.. Antibodies that target PR3 are a type of autoantibody that can attack healthy human cells and cause inflammation. Blood vessels Causes organ damage and in most cases kidney, lung And the airway.
Based on the presence of the woman’s PR3 antibody, doctors diagnosed her as “granulomatosis with polyangiitis” or GPA. According to the Mayo Clinic, almost everyone with a GPA has these autoantibodies.
However, “the exact role of these autoantibodies in the development of granulomatosis with polyangiitis is not fully understood.” According to national organizations for rare disabilities (main). One possibility is that these antibodies can latch on white blood cells that carry PR3, causing them to malfunction in some way and damage healthy tissue.
“Estimates of the frequency of granulomatosis with polyangiitis vary widely depending on the particular population being studied,” says NORD, which often goes unnoticed by physicians. Because of this, it is difficult to estimate how many people develop a disability each year, but it is considered very rare.
According to the NORD, GPAs can appear slowly over months or quickly within a few days. Symptoms vary widely from person to person, but can affect organs throughout the body. For example, in severe cases, if left untreated, it can cause deafness, loss of vision, renal failure, or cranial nerve damage. Autoimmune diseases can also cause persistent runny nose, nasal crusting, sinus inflammation, and perforation (holes) in the septum, as seen in affected women.
To treat a woman’s GPA, her doctor prescribed rituximab. This is an antibody treatment that targets immune cells called B cells to dial down the body’s dysfunctional immune response. According to Drugs.com.. Patients were also given low doses of cyclophosphamide and prednisone, which are other immunosuppressants. Corticosteroid It also weakens immune activity.
According to a NEJM report, “a follow-up visit six months later eliminated the patient’s bloody nasal secretions and scabs.” “The nasal malformation remains the same.” The patient continued to receive rituximab, but the report did not state whether she would undergo nasal surgery.
Originally published in Live Science.
Rare autoimmune disorder caused a woman’s nose to collapse Source link Rare autoimmune disorder caused a woman’s nose to collapse