Q&A: COVID-19 vaccination may trigger systemic capillary leak syndrome flares

Three adults experienced severe flares related to systemic capillary leak syndrome immediately after receiving a COVID-19 vaccine, researchers wrote in Annals of Internal Medicine.

Since systemic capillary leak syndrome was first described in 1960, there have been fewer than 500 reports of patients with the disease in medical literature, according to the National Organization for Rare Disorders.

WHO classified the three recent cases of systemic capillary leak syndrome flares as “non–dose-related, unexpected and serious adverse events,” wrote Meghan Matheny, BS, an intramural research training award fellow within the National Institute of Allergy and Infectious Diseases, and colleagues. The researchers also noted that the European Medicines Agency has recommended against the use of the Johnson & Johnson or AstraZeneca COVID‐19 vaccines in patients with the condition.

All three patients in the case series — a white woman aged 68 years who received the Johnson & Johnson vaccine, a white woman aged 46 years who received the Moderna vaccine and a white man aged 36 years who received the Pfizer/BioNTech vaccine — each presented to a hospital with hypotension and tachycardia and tested negative for SARS-COV-2, according to the researchers. The first patient was nauseous and vomiting upon hospital arrival, the second patient presented with influenza-type symptoms and the first and third patients had syncope symptoms upon hospital admission. The first patient died 7 days after hospital admittance, and the second and third patients were discharged 7 and 10 days after being admitted, respectively.

The case series adds to the list of potential effects of COVID-19 vaccination. Earlier this week, the FDA said that Johnson & Johnson’s vaccine may lead to an increased risk for Guillain-Barré syndrome. There have also been reports of tinnitus, facial swelling in patients who have previously received facial fillers and anaphylactic reactions in limited numbers of COVID-19 vaccine recipients.

To learn more about the link between COVID-19 vaccination and systemic capillary leak syndrome, we asked Kirk M. Druey, MD, coauthor of the report and the chief of the lung and vascular inflammation section in the NIAID laboratory of allergic diseases, to discuss the implications of the report for health care professionals.

Healio Primary Care: What is the likelihood that patients with a history of systemic capillary leak syndrome will experience a flare after COVID-19 vaccination? What else can trigger flares?

Druey: Because systemic capillary leak syndrome is such a rare disease, it is difficult to predict whether or not patients will experience a flare after COVID-19 vaccination. However, two out of three patients we describe had no prior diagnosis of systemic capillary leak syndrome. Flares associated with this disease are typically triggered by minor infections such as viral upper respiratory infections and have also been described in association with COVID-19 itself.

Healio Primary Care: Has systemic capillary leak syndrome been documented with other vaccines?

Druey: Not to our knowledge.

Healio Primary Care: Should patients with systemic capillary leak syndrome or suspected systemic capillary leak syndrome receive the COVID-19 vaccine?

Druey: Yes, but we recommend that patients be formally evaluated and started on monthly prophylaxis with intravenous immunoglobulin prior to vaccination.

Healio Primary Care: What should physicians watch for in these patients after vaccination? How can they help prevent serious complications and mortality?

Druey: Physicians should watch for signs and symptoms of hypotension and intravascular volume depletion, such as dizziness, fainting, increased thirst or decreased urination. Patients displaying these signs should be evaluated in a medical setting as soon as possible.

Healio Primary Care: What is the take-home message for primary care physicians?

Druey: We recommend that patients with a diagnosis or a suspected diagnosis of systemic capillary leak syndrome should receive intravenous immunoglobulin prophylaxis before vaccination.

References:

Matheny M, et al. Ann Intern Med. 2021;doi:https://www.acpjournals.org/doi/10.7326/L21-0250.

National Organization for Rare Disorders. Systemic Capillary Leak Syndrome. https://rarediseases.org/rare-diseases/systemic-capillary-leak-syndrome/. Accessed July 13, 2021.