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by Richard Brasington, MD, FACP
Rheumatology Fellowship Program Director
Professor, Department of Medicine
Washington University in St. Louis, St. Louis, Missouri

One of the most important aspects of “preventive health care’ is receiving the appropriate vaccinations. This is particularly important for patients with autoimmune disease with comprised immune function, especially when immunosuppressive medications are used. We can think of vaccinations in three broad categories: 1) those which everyone should receive; 2) those which are particularly appropriate for patients with autoimmune diseases; and 3) vaccines which may be dangerous for such patients and therefore should be avoided.

Updating vaccinations is particularly important before receiving some immunosuppressive medications, because these can seriously blunt a person’s response to appropriate vaccination.

Nowadays, the “standard” vaccinations are administered during the pre-school and elementary school years and include mumps, measles, rubella, tetanus, diphtheria, etc. For purposes of this discussion, we will assume that all patients who are ultimately diagnosed with Sjögren's have received all of the appropriate childhood immunizations. All adults need to remember that at least every ten years they should receive a tetanus and diphtheria “booster.” In reality, this booster is often administered when a question arises as to whether tetanus immunity is current; if there is no documentation of a Td booster in the previous ten years, it is given at that point.

Note that the Varicella vaccine is a live virus vaccine, and should not be given to immunosuppressed persons.

Patients with autoimmune disorders such as Sjögren's are generally considered to have some compromise of the immune system and increased susceptibility to infection. A simple way to think of this is to consider that if the immune system is “misdirected” toward self, it probably is not doing an ideal job of protecting against infectious agents. Obviously, someone with mild Sjögren's who does not have pronounced systemic disease will not be as susceptible to infection as a patient with systemic disease requiring prednisone and/or immunosuppressive agents such as azathioprine, methotrexate, mycophenylate mofetil, rituximab, or cyclophosphamide. Nonetheless, I recommend that all patients with Sjögren's undergo vaccination for influenza, pneumococcal pneumonia, and shingles.

While some patients fear that vaccines can activate the immune system and cause systemic flares, no scientific evidence exists to indicate that this is the case. In fact studies in systemic lupus erythematosus (SLE) do not suggest disease activation with Pneumovax®. Vaccines need only be avoided when a previous reaction has occurred, and a reaction to one vaccination does not mean that all future vaccinations should be avoided.

The annual “flu shot” each fall is familiar to everyone. This vaccine is different each year and must be given every year. The vaccine for a given flu season is developed based on scientists’ best predictions of which strains of influenza virus will be dominant that particular year. Hence, immunity one year does not necessarily carry over until the next year. Even when vaccination does not prevent the occurrence of influenza in those vaccinated, it is likely that the illness will not be as severe in those vaccinated.

The “pneumonia shot” (Pneumovax, or pheumoccal polysaccharide vaccine) specifically protects against one kind of bacterial pneumonia, pneumococcal pneumonia, and covers twenty-three serotypes. Those who are elderly or chronically ill are particularly at risk of developing severe, even fatal, pneumococcal infections. For some persons, a second dose is recommended.

There has been an important advance in vaccination against pheumococcal pneumonia with the advent of Prevnar, or Pneumococcal 13-valent Conjugate Vaccine.  Prevnar provides additional protection, and should also be given. The order in which one receives Pneumovax and Prevnar depends upon a number of issues, so you should consult with your physician about this. Everyone with Sjögren's should receive these vaccines, unless there are specific medical reasons to the contrary.

The third category of vaccines to consider is the “live virus” vaccines. The vaccines discussed above are made of killed viruses or bacteria and pose no risk of infection. However, vaccination with a live virus does pose some risk of infection, and in someone with an autoimmune disease on immunosuppressive medication, this may be quite dangerous. One such live attenuated vaccine is FluMist®, which is administered as a nasal spray. FluMist® should not be given to immunosuppressed patients. The definition of immunosuppressed is open to interpretation but clearly includes patients taking prednisone or immunosuppressive agents such as azathioprine, methotrexate, cyclophosphamide, rituximab, or mycophenylate mofetil.

There has been a major new development in the “shingles vaccine”. Zostavax is an attenuated live virus vaccine which cannot safely be administered to patients who are significantly immunosuppressed. The new vaccine, Shingrix (recombinant zoster vaccine), is a “killed virus” vaccine, and therefore is not as risky to immunosuppressed persons. Furthermore, it appears that it is much more effective than Zosavax in preventing shingles. The Centers for Disease Control recommends Shingrix over Zostavax, and recommends two doses separated by two to six months in “immunocompetent persons” 50 years of age and older, including patients on “low dose immunosuppression.” This latter term is open to interpretation, so ask your doctor which vaccination is right for you.

Gardasil® protects against human papilloma virus infection and the complication of cervical cancer. Experience with this vaccine in young women with Sjögren's is limited; there is too little evidence to recommend its routine use.

For patients on chronic steroids, keep in mind that doses of prednisone higher than 30mg a day may alter antibody production. Ideally, vaccines should be administered at the lowest possible steroid dose. For patients on Rituxan®, vaccines should be given at least three weeks before the infusion in order to optimize antibody production. Similarly, vaccines should be given prior to a course of Cytoxan®, which can also suppress B lymphocyte function. IVIg should not pose a problem and, in fact, may provide what is known as passive immunity to many microbes.

In conclusion, my recommendation is that all patients with Sjögren's should have the pneumococcal pneumonia vaccine, yearly influenza vaccine and a tetanus-diphtheria booster at least every ten years. Vaccination against shingles is now less complicated, and should be strongly considered. Live virus vaccines such as Flumist® and ZostavaxTM should be avoided except in special circumstances to be determined by the physician.

This article was first printed in January 2020 in the Foundation's patient newsletter for members, Conquering Sjögren's. Click here to learn more about becoming a member. 

Click here to learn more about the COVID-19 Vaccine and Sjögren's.