Pemphigus blood test values
Recently diagnosed with pemphigus with intercellular antibody titer of 1:1280.
There are no skin blisters and no biopsy performed.
Currently, the symptoms are concentrated in the oral mucosa with redness and ulceration.
What is the clinical significance of this titer? I have received a biological agent once and will receive another dose in about 2-3 weeks because the doctor prescribed a simple regimen of colchicine and steroids.
Should this titer be considered severe?
kola, 40~49 year old female. Ask Date: 2023/10/25
Dr. Gao Jiankai reply Internal Medicine
Hello, thank you for your question.
Intercellular matrix antibodies are one of the blood tests used to diagnose pemphigus.
A higher value indicates a greater amount of antibodies and is related to disease activity.
However, the values can change with treatment and can serve as an indicator of whether the disease is under control.
If the biologic agent you are receiving is rituximab, a typical treatment course involves two infusions, spaced two weeks or more apart.
This medication is very effective for the treatment of pemphigus, and your condition should be well controlled.
The Allergy, Immunology, and Rheumatology Department of Changhua Hospital, Ministry of Health and Welfare, is concerned about your health.
Reply Date: 2023/10/25
More Info
Pemphigus is an autoimmune blistering disorder characterized by the presence of autoantibodies against desmogleins, which are proteins crucial for cell adhesion in the skin and mucous membranes. The presence of these antibodies leads to the disruption of cell-to-cell adhesion, resulting in the formation of blisters and erosions. The clinical significance of pemphigus antibody levels, particularly the intercellular substance antibody (also known as anti-desmoglein antibody), is paramount in diagnosing and managing the disease.
In your case, the reported antibody level of 1:1280 is notably high. This indicates a significant presence of autoantibodies in your system, which correlates with the severity of the disease. High antibody titers are often associated with more extensive disease involvement and can predict the likelihood of severe manifestations. While you currently do not have skin blisters and your symptoms are localized to the oral mucosa, the elevated antibody levels suggest that the disease is active and could potentially worsen if not adequately managed.
The absence of skin blisters does not rule out the diagnosis of pemphigus, as the disease can manifest primarily in mucosal areas, particularly in the oral cavity. Symptoms such as redness, swelling, and ulceration of the oral mucosa are common presentations in pemphigus vulgaris, which is the most prevalent form of the disease. The clinical picture you describe aligns with this condition, and the high antibody levels further support the diagnosis.
Regarding your treatment regimen, it appears that you are receiving a combination of colchicine and corticosteroids. Colchicine is primarily used for conditions like gout and may not be the first-line treatment for pemphigus. Corticosteroids, on the other hand, are a cornerstone in the management of pemphigus due to their potent anti-inflammatory and immunosuppressive effects. They help reduce the immune response that leads to the production of autoantibodies and the resultant blistering.
The use of biologic agents, which you mentioned receiving, can also be beneficial in managing pemphigus. Biologics such as rituximab target specific components of the immune system and can lead to a more sustained remission by depleting B cells that produce the pathogenic antibodies. The timing of your treatments, approximately every 2-3 weeks, is consistent with standard practice for biologic therapy, allowing for adequate drug levels to manage the disease effectively.
In summary, your elevated pemphigus antibody level of 1:1280 is clinically significant and suggests active disease, despite the absence of skin blisters. The symptoms localized to the oral mucosa are consistent with pemphigus vulgaris. It is crucial to continue monitoring your condition closely and to adhere to the treatment plan prescribed by your healthcare provider. Regular follow-ups are essential to assess the effectiveness of the treatment and to make any necessary adjustments based on your clinical response and antibody levels. If you experience any worsening of symptoms or new manifestations, it is important to communicate these changes to your healthcare team promptly.
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