Neurological symptoms may be more common in IgG4-related disease
Single-center study highlights eye and nervous system involvement
Written by |
IgG4-related disease (IgG4-RD) may affect the nervous system more often than has been previously recognized, according to a study from Turkey.
Researchers found that more than one in five patients treated at their clinic had neurological involvement, most commonly manifesting as vision problems.
“Although neurological manifestations of IgG4-RD are often regarded as rare in the literature, our findings may suggest that their frequency may be underrecognized,” the researchers wrote. “These findings underscore the need for heightened awareness among neurologists and call for prospective studies [following patients over time] with long-term follow-up to better characterize disease course and management strategies.”
The study, “Neurological involvement in IgG4-related disease: focus on neuro-ophthalmologic features,” was published in the Journal of Neurology.
How IgG4-related disease affects the body
IgG4-RD is a chronic immune-mediated disorder marked by abnormal clumps of immune cells, including those that produce the IgG4 antibody. These clumps can affect many different organs throughout the body, leading to a wide range of symptoms and clinical profiles.
Previous reports have described IgG4-RD affecting the nervous system, but neurological involvement has generally been considered rare. This new study challenges that view, with the researchers writing that “neurological involvement in IgG4-RD may be more common than previously recognized.”
Researchers in Turkey reviewed clinical data from 77 adults with IgG4-RD treated at their clinic between 2014 and 2023. They found that 17 patients, or about 22%, had some form of neurological involvement.
Participants with and without neurological involvement had similar mean age, as well as comparable proportions of men and women, which “suggests that neurological involvement does not show a clear gender predilection,” the researchers wrote.
The most common form of neurological involvement, seen in 10 patients, was neuro-ophthalmologic disease — meaning vision problems caused by damage to the nerves that connect the eyes to the brain and help process visual information.
The researchers noted that most of these patients were initially misdiagnosed with other eye diseases before being correctly diagnosed with IgG4-RD. Many of the people with neuro-ophthalmologic disease showed a combination of three symptoms: headache, proptosis (bulging eyes), and diplopia (double vision).
Researchers call for greater awareness and follow-up studies
Based on these findings, the researchers suggested that patients with this particular triad of symptoms should be evaluated for IgG4-RD, which could help reduce misdiagnosis. However, they stressed that this symptom combination also occurs in other diseases, and clinicians should interpret it with caution and rely on histopathological confirmation, such as biopsy, whenever possible.
Features of affected neurological tissue in patients with neuro-ophthalmologic disease were characteristic of IgG4-RD and “consistent with those described in other organ systems, and no distinct pattern specific to neurological involvement was identified,” the team wrote.
Among the seven patients with other forms of neurological involvement, six had hypertrophic pachymeningitis, a condition marked by inflammation and thickening in the membrane that surrounds the brain and spinal cord. The seventh had inflammation in the pituitary gland (a small brain structure that helps regulate hormone levels).
Most of the people with hypertrophic pachymeningitis had headache as their main symptom; some also reported vision issues like diplopia.
Most patients who had neuro-ophthalmologic disease (60%) were able to control their IgG4-RD using glucocorticoids alone, with only four of 10 requiring additional immunosuppressive treatment. In contrast, most patients with hypertrophic pachymeningitis (83.3%) required additional immunosuppressive medicines to achieve remission.
“Notably, patients with neurological involvement were diagnosed earlier than those with extra-neurological disease, reflecting the clinical impact of neurological symptoms,” the researchers wrote. “These findings highlight the importance of a multidisciplinary approach, integrating neurologists, rheumatologists, and radiologists to ensure accurate diagnosis and optimal management.”
The researchers emphasized, however, that this study was limited to patients at a single center, so additional studies will be needed to validate and expand upon the results.
