Do You Need Steroids for Vasculitis in Sjögren's Syndrome? - Internal Medicine

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Is steroid medication necessary for vasculitis?


Two years ago, I experienced difficulty opening my left eye upon waking, and I was diagnosed with dry eye syndrome.
Last year, after a salivary gland biopsy, I was diagnosed with xerostomia, and my white blood cell count dropped to around 3,000, with complement levels also being low.
My physician announced that I have Sjögren's syndrome.
My first question is: my ANA, SSA, and SSB tests are all negative, and lupus anticoagulant and rheumatoid factor are also negative.
Does this mean I am a primary and mild case of Sjögren's syndrome? Is it possible for the negative results to turn positive in the future? My second question is: since May of this year, I have been experiencing recurrent vasculitis in my lower extremities.
The first doctor suggested that, in addition to quinine, I should take corticosteroids and anti-inflammatory pain medication.
I sought a second opinion, and the response was that the vasculitis symptoms are not severe, and I should continue taking quinine.
I am feeling uncertain; what is your opinion?

Jiang Xiaojie, 40~49 year old female. Ask Date: 2017/09/25

Dr. Zeng Guosen reply Internal Medicine


Hello, everyone.

1.
The diagnosis of Sjögren's syndrome primarily involves testing for positive anti-SSA or anti-SSB antibodies, which are present in only 50-60% of patients.
If these tests are negative, a salivary gland biopsy is performed for diagnosis.
If the dry syndrome is associated with autoimmune diseases such as systemic lupus erythematosus or rheumatoid arthritis, it is classified as secondary; if there are no other underlying diseases, it is classified as primary.
Positive results for ANA, RF, anti-SSA, and anti-SSB usually indicate more typical and pronounced symptoms.
Some of these tests may show seroconversion, turning from positive to negative or vice versa, in subsequent follow-ups.
2.
Regarding vasculitis, the use of corticosteroids depends on the specific type of vasculitis, its severity, and whether it involves other organs.
Thank you.

Reply Date: 2017/09/25

More Info


Sjögren's syndrome is a chronic autoimmune disorder characterized primarily by dry eyes and dry mouth due to the destruction of the glands that produce these secretions. It can occur as a primary condition or secondary to other autoimmune diseases such as rheumatoid arthritis or lupus. Your situation, where you have negative results for ANA, SSA, SSB, and other autoimmune markers, suggests that you may have primary Sjögren's syndrome, which can sometimes present with milder symptoms.

Regarding your first question about the implications of negative autoimmune markers: while negative results can indicate a less severe form of the disease, it is essential to understand that autoimmune diseases can be complex. The presence or absence of specific antibodies does not always correlate directly with disease severity or progression. It is possible for autoimmune markers to change over time; thus, your negative results today do not guarantee that they will remain negative in the future. Regular monitoring and follow-up with your healthcare provider are crucial to manage your condition effectively.

Now, concerning your second question about the management of vasculitis in the context of Sjögren's syndrome: the treatment approach can vary significantly based on the severity of the symptoms and the extent of the vasculitis. Vasculitis refers to inflammation of the blood vessels, which can lead to various complications depending on the vessels involved. In mild cases, as suggested by your second physician, treatment may focus on symptomatic relief and monitoring rather than aggressive immunosuppressive therapy. Quinine, which you mentioned, is typically used for muscle cramps and may not directly address the underlying inflammation associated with vasculitis.

On the other hand, if your symptoms of vasculitis are more pronounced or if there is a risk of complications, corticosteroids may be warranted. Corticosteroids are powerful anti-inflammatory medications that can help reduce inflammation and suppress the immune response. The decision to use steroids should be made carefully, weighing the potential benefits against the risks, especially considering your history of infections and the need for careful management of your immune system.

In summary, the management of Sjögren's syndrome and associated vasculitis should be individualized. It is essential to have a thorough discussion with your healthcare provider about your symptoms, treatment options, and the rationale behind each recommendation. If you feel uncertain about the advice given, seeking a third opinion from a specialist in autoimmune diseases or a rheumatologist may provide additional clarity and help you make an informed decision about your treatment plan. Regular follow-ups and monitoring are crucial to adapt your treatment as your condition evolves.

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