Transient cryoglobulinaemic vasculitis following ChAdOx1 nCoV-19 vaccine.

Cryoglobulinaemic vasculitis is an immune-complex-mediated, systemic inflammatory syndrome usually involving small-to-medium vessels due to precipitation of cryoglobulins at <37°C. It can involve any organ but most commonly affects the skin. Associated conditions include infections (hepatitis C and HIV), haematological disorders (chronic lymphocytic lymphoma, monoclonal gammopathy of uncertain significance and multiple myeloma), autoimmune conditions (systemic lupus erythematosus and Sjogren syndrome) or as a complication following vaccination (influenza, pneumococcal and hepatitis B vaccines). Biochemical hallmarks include detection of serum cryoglobulin with low C4 levels. We describe a case of previous healthy patient with transient cryoglobulinaemic vasculitis after first dose of ChAdOx1 nCoV-19 vaccine (AstraZeneca/Oxford).

Immunogenicity and safety of COVID-19 vaccination in patients with primary Sjögren's syndrome.

OBJECTIVES: To evaluate humoral and cellular immune responses and adverse events (AEs) after COVID-19 vaccination in patients with primary Sjögren's syndrome (pSS) compared to healthy controls (HC), and disease activity following vaccination in patients with pSS. METHODS: 67 patients with pSS and 33 HC (ratio 2:1) received COVID-19 vaccinations following the Dutch vaccination programme. Patients with pSS did not use immunomodulatory drugs, except hydroxychloroquine. Anti-spike 1 receptor binding domain IgG serum antibody levels were measured 28 days after complete vaccination. AEs were collected 7 days after vaccination. In a subgroup, salivary anti-SARS-CoV-2 antibodies and T-cell response by interferon-γ enzyme-linked immune absorbent spot was measured. RESULTS: 47 patients with pSS (70%) and 14 HC (42%) received BNT162b2 (Pfizer-BioNtech), 13 (19%) and 5 (15%) received ChAdOx1 nCoV-19 (AstraZeneca), 6 (9%) and 8 (24%) received mRNA-1273 (Moderna), and 1 (1%) and 6 (18%) received Ad.26.COV2.S (Janssen). All participants had positive anti-SARS-CoV-2 antibody levels (>2500 AU/mL) postvaccination. No differences in anti-SARS-CoV-2 antibody levels were observed between patients with pSS and HC, for each vaccine type. Salivary anti-SARS-CoV-2 IgG antibodies also increased, and a T-cell response was observed in patients with pSS and HC. Frequencies of systemic AEs were comparable between patients with pSS and HC (first vaccination: 34/67 (51%) vs 16/33 (48%), p=0.83; second: 41/66 (62%) vs 14/25 (56%), p=0.59). No significant worsening was observed in patient-reported and systemic disease activity, including auto-antibodies. CONCLUSIONS: Patients with pSS had similar humoral and cellular immune responses as HC, suggesting COVID-19 vaccination is effective in patients with pSS. AEs were also comparable, and no increase in disease activity was seen in patients with pSS.

Sjögren-related cardiomyopathy presenting with cardiogenic shock.

Previous reports have described non-ischaemic cardiomyopathy related to a variety of autoimmune diseases. However, very few case reports describe Sjögren disease as a contributing factor to cardiomyopathy. We report the case of a 69-year-old woman with a history of Sjögren disease who presented with cardiogenic shock. Laboratory testing and cardiac MRI revealing apical septal late gadolinium enhancement were consistent with an autoimmune aetiology. After ruling out ischaemic, infectious and other possible causes, the patient's clinical presentation was thought to be related to underlying Sjögren disease. She was treated with intravenous steroids and evidence-based heart failure therapy, but she eventually died after having declined heart transplantation. Given the rarity of Sjögren disease, no diagnostic criteria or standard treatment has been established for cardiomyopathy related to this disease. Diagnosis should be considered in patients who show evidence of autoimmune processes after other possible causes are ruled out.

Immediate Amelioration of Severe Respiratory Distress in Sjögren's Syndrome with COVID-19 Treated with a Single Dose of Off-label Tocilizumab.

The coronavirus disease 2019 (COVID-19) pandemic has become an urgent global health issue. An older age and underlying conditions, such as diabetes, have been reported as risk factors, but whether or not autoimmune diseases increase the risk remains unknown. An 85-year-old man with Sjögren's syndrome developed a severe COVID-19 infection that required oxygen supplementation. After discussing the goals of care with him and his wife, off-label tocilizumab was given concomitantly, resulting in a rapid improvement in his symptoms and respiratory failure. This patient represents a supplementary case confirming the efficacy and safety of tocilizumab for COVID-19 in elderly patients with autoimmune diseases.

Novel insights on lymphoma and lymphomagenesis in primary Sjögren's Syndrome.

Primary Sjögren's Syndrome (pSS) is a systemic autoimmune disease characterized by a chronic inflammatory process mainly affecting the exocrine glands but also burdened by a wide range of extraglandular manifestations. Non-Hodgkin lymphoma (NHL) is the most severe pSS complication worsening disease prognosis. We summarized original articles published between April 2018 and May 2020 on this topic aiming to highlight novelties on lymphoma and lymphomagenesis. Results have been grouped by epidemiology, etiopathogenesis and predictors of lymphoma. NHL is the most severe complication of pSS and occurs in around 5-10% of patients. Over the last two years, several clinical, serological, and histopathological features have been proposed as predictive for lymphoma in pSS patients, allowing early diagnosis and consequently, better management and prognosis. Individual monitoring for disease activity and possible lymphoma development is a central clue in the evaluation of pSS patients.