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    Weigh the risk vs benefit of vaccines in autoinflammatory disease

    New study investigates the incidence of vaccine reaction in patients with cryopyrin-associated periodic syndromes (CAPS), revealing frequency of local and systemic reactions in many.

    Physicians should be cautious when administering vaccines, particularly pneumococcal vaccines, to patients with autoinflammatory disorders, according to a new study.

    The study, published in Rheumatology, investigated the prevalence and severity of reactions in patients with cryopyrin-associated periodic syndromes (CAPS) and found that many, but not all, have moderate to severe reactions that warrant caution and additional research.

    Although the Advisory Committee on Immunization Practices (ACIP) recommends that patients with CAPS—particularly familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and neonatal onset multisystem inflammatory disease (NOMID)—receive vaccinations against pneumococcal disease, tetanus, and influenza when treated with immunosuppressive medications, the study investigated the safety of these vaccines in the particular patient population. Patients with CAPS may have an increased susceptibility to pneumococcal pneumonia, similar to other patients with immune-related disorders, according to the report.

    Previous research revealed unusually severe local and systemic reactions in CAPS patients related to vaccination that led to this study. Researchers first analyzed case studies of 7 CAPS patients vaccinated with the pneumococcal series. Six of those patients were also being following in the B-CONFIDENT (Clinical Outcomes and Safety: A Registry Study of Ilaris [Canakinumab] Patients) registry, a long-term prospective observational study of patients treated with canakinumab. This involvement led the study authors to expand their study to investigate the safety of pneumococcal and other vaccines within the entire B-CONFIDENT registry.

    The registry followed 285 patients, 68 of whom fit the CAPS criteria desired by the study authors. Those 68 CAPS patients received a total of 159 vaccine injections across 9 countries—81% influenza, 26% pneumococcal, 18% tetanus and diphtheria, and 16% against other unspecified pathogens.

    Over the study period, the researchers noted that 43 CAPS patients received more than 1 vaccination, and that 22 injections in 18 CAPS patients resulted in at least 1 vaccine reaction.

    Most of the vaccine reactions were in 12 CAPS patients who received pneumococcal vaccines. In total, 80% of the pneumococcal vaccines given to CAPS patients caused a reaction in comparison with just 7% of tetanus/diphtheria vaccines, according to the report.

    The most common vaccine reaction was fever, which occurred in about half of all pneumococcal injections. Additionally, reactions caused by the pneumococcal vaccine occurred very rapidly and symptoms lasted longer than those caused by influenza, tetanus, or diphtheria vaccinations.

    Five of the CAPS patients in the study developed severe reactions, 2 of whom required hospitalization related to cellulitis and meningitis. Additionally, researchers found that pneumococcal vaccines in CAPS patients triggered early systemic inflammation and even CAPS flares.

    “One hypothesis that could explain the adverse events following pneumococcal vaccination is that pneumococcal antigens contain TLR2 and TLR4 ligands that trigger the rapid onset and systemic symptoms in patients who are genetically prone to inflammasome overactivation,” the study notes. “The observation that patients with the more severe CAPS phenotype (NOMID) appeared to have more frequent and more severe events than CAPS patients with the less severe phenotype (FCAS) also supports a role of inflammaso-hyperactivation in this adverse reaction.”

    Although the patients in the study were treated with canakinumab, the study authors did not observe a relationship between canakinumab dose or timing and vaccination reactions, according to the report.

    Hal M. Hoffman, MD, is a professor of Pediatrics and Medicine and chief of the Division of Allergy, Immunology, and Rheumatology in the Department of Pediatrics at the University of California (UC) San Diego/Rady Children’s Hospital-San Diego. One of the study authors, Hoffman’s lab at UC San Diego was the first to identify the genetic basis of 4 human diseases, including the genetic basis for CAPS—NLRP3.

    Although many patients with conditions like CAPS might not be managed by general practice pediatricians, Hoffman says they are often the first to see those patients prior to confirmation of a diagnosis.

    “CAPS is an ultra-rare autoinflammatory disease characterized by rash, fever, and musculoskeletal symptoms. Prevalence is approximately 1 to 2 per million, but patients often first present to pediatricians. Many are referred to and managed by specialists including immunologists and rheumatologists,” Hoffman says.

    Once diagnosed, treatment of CAPS can affect vaccine recommendation for those patients, he adds.

    “Many CAPS patients are treated with IL-1 inhibitors [that] may predispose the patients to common bacterial infections, specifically streptococcal infections, and therefore it is recommended to vaccinate or give booster prior to starting medicines,” Hoffman says. “Most vaccines are given by the pediatricians, although Pneumovax is not a common vaccine given by pediatricians.”

    Hoffman says the study demonstrates that pediatricians should consider carefully the risk versus benefit of specific vaccines in patients with rare inherited inflammatory disorders such as CAPS.

    “Physicians should weigh risks and benefits of vaccines in patients with immune disorders,” Hoffman says. “It is widely known that patients with some forms of immune deficiencies should avoid live viral vaccines. This is another consideration.”


    Rachael Zimlich, RN
    Rachael Zimlich is a freelance writer in Cleveland, Ohio. She writes regularly for Contemporary Pediatrics, Managed Healthcare ...

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