Damage associated with disease-specific events and diagnostic or therapeutic procedures is highly prevalent in IgG4-related disease
Abstract format and assignment number: Poster 0286
Presenting author: J. Stone (US)
Date: Saturday, 16th November 2024
IgG4-related disease often requires invasive diagnostic and/or therapeutic procedures. Among 191 patients from Massachusetts General Hospital, 92% had any evidence of organ damage. Most of this damage was due to organ enlargement or dysfunction itself, especially of the pancreas, salivary glands and kidneys. Damage due to invasive diagnostic or therapeutic procedures was registered in up to 12% of patients. Most of this damage affected the salivary or lacrimal glands and pancreas. These numbers let us realize that our procedures are not always without consequences.
Hypersensitivity pneumonitis with autoimmune features: a new entity?
Abstract format and assignment number: Poster 0288
Presenting author: P. Maymó (Spain)
Date: Saturday, 16th November 2024
Hypersensitivity pneumonitis, which is caused by lymphocytic inflammation, can concur with autoimmune features. The authors described 28 cases of hypersensitivity with autoimmune features (HPAF). Half of them were diagnosed with connective tissue disease, half of whom after the onset of the hypersensitivity pneumonitis. All cases were more often treated with immunosuppressants and had a better prognosis as compared with hypersensitivity pneumonitis without autoimmune features. It is unfortunate that the authors did not provide a definition of HPAF. Yet, their suggestion that HPAF is a distinct disease entity, similar to interstitial pneumonia with autoimmune features (IPAF), is interesting.
Characteristics of adult-onset Still’s disease skin eruption in individuals of Sub-Saharan ancestry
Abstract format and assignment number: Poster 0297
Presenting author: B. Suzon (Martinique)
Date: Saturday, 16th November 2024
The authors showed that rashes in patients with adult-onset Still’s disease (AOSD) of Sub-Saharan ancestry are mostly atypical. Among 33 of such patients with a rash, 21 had an atypical rash, being persistent, pruritic, urticarial, eczematous and/or consisting of plaques, patches, bullae, erythroderma, erosion, scales, purpura or necrosis. Those with an atypical rash more often had systemic polycyclic disease than those with a typical rash. Three patients with macrophage activating syndrome and two deaths occurred exclusively among those with an atypical rash.
The performance of Behçet’s disease clinical diagnostic criteria in a low prevalence region: a retrospective single center cohort study
Abstract format and assignment number: Poster 1118
Presenting author: M. Shamim (US)
Date: Sunday, 17th November 2024
The authors reviewed records of 59 patients diagnosed with Behçet’s disease in Ohio. Of these, 53% fulfilled the ISG 1990 classification criteria, 81% fulfilled the ICBD 2014 classification criteria and 5% fulfilled the Japanese Society classification criteria.
The development of the EULAR Score for the Definition of Disease Activity in Adult-onset Still’s Disease: the “DAVID” Project
Abstract format and assignment number: Poster 2030
Presenting author: P. Ruscitti (Italy)
Date: Monday, 18th November 2024
To develop a disease activity score for adult-onset Still’s disease (AOSD), the authors performed a systematic literature review and Delphi discussion to identify relevant features and applied them to an Italian cohort. The clinician’s judgment was used as gold standard. The final score incorporated fever, skin rash, arthritis, patient global assessment and CRP. Active disease was defined as having fever with one other feature or as having no fever with at least three of the other features.
A systematic review of treatment strategies in VEXAS syndrome
Abstract format and assignment number: Poster 2032
Presenting author: A.V. Prakash (US)
Date: Monday, 18th November 2024
The authors performed a systematic literature review of case reports and case series published between 2020 and 2024 to evaluate treatment effects for VEXAS syndrome. Based on 131 patients in 111 articles, treatments with proportions of complete response (CR) and partial response (PR) included, in order from most to least often used: IL-6 inhibitors (CR 50%, PR 38%), azacytidine (CR 73%, PR 27%), IL-1 inhibitors (CR 42%, PR 33%), JAK inhibitors (CR 17%, PR 50%), methotrexate (CR 0%, PR 80%) and TNF inhibitors (CR 0%, PR 100%).
Dr Jacob J.E. Koopman, MD PhD
Jacob is a clinical immunologist who was trained at Erasmus Medical Center in Rotterdam, Netherlands. He has worked at University Medical Center Utrecht. His clinical practice and research focus mainly on systemic lupus erythematosus, but additionally he has interests in inflammatory myopathies, vasculitis, and rare immunological diseases. He is a member of EMEUNET’s Newsletter Subcommittee.