Churg–Strauss syndrome complicated by central retinal artery occlusion: case report and a review of the literature

Asako, Kurumi; Takayama, Maki; Kono, Hajime; Kikuchi, Hirotoshi

doi:10.3109/s10165-011-0422-y

Cited by 8 publications

(3 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…As known, systemic vasculitis can present with a variety of clinical features, also including ocular changes [ 47 – 49 ]. In AAV, the increased morbidity and mortality is related to the multi-organ involvement secondary to inflammation and necrosis of the small blood vessels: the early detection of organ damages is, thus, the key challenge in the management of these diseases [ 14 , 15 , 26 , 49 ]. However, the detection of ocular involvement in AAV, unless dramatic, often recognizes a relevant diagnostic delay that leads to a worse prognosis for patients [ 50 ].…”

Section: Discussionmentioning

confidence: 99%

“…BVAS is a comprehensive multisystem clinical assessment in AAV and includes eyes in terms of scleritis/episcleritis, conjunctivitis/blepharitis/keratitis, and uveitis. Also, retinal changes are analyzed in BVAS as vasculitis, thrombosis, exudate, hemorrhages: however, all these abnormalities in pre-symptomatic phases can be undiagnosed and, thus, can lead to subclinical chronic damages, at the same time [ 22 , 25 , 26 ]. In accordance with our findings, the OCT-A can be considered as a key diagnostic tool in the early detection of possible subclinical retinal changes in AAV by adding information on disease-damage and activity in pre-symptomatic patients.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Subclinical microvascular changes in ANCA-vasculitides: the role of optical coherence tomography angiography and nailfold capillaroscopy in the detection of disease-related damage

Triggianese

D’Antonio

Nesi

et al. 2023

Orphanet J Rare Dis

View full text Add to dashboard Cite

Background Both cardiovascular and complement-mediated disorders might lead to microvascular damages in anti-neutrophil cytoplasm autoantibodies (ANCA)-associated vasculitides (AAV). We aimed at investigating, for the first time, subclinical microvascular abnormalities with non-invasive techniques in AAV patients by analyzing both retinal and nailfold capillary changes. Retinal plexi were investigated using optical coherence tomography angiography (OCT-A), while nailfold capillary changes by video-capillaroscopy (NVC). Potential correlations between microvessels’ abnormalities and disease damage were also explored. Methods An observational study was conducted on consecutive patients who met the inclusion criteria of defined diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA), granulomatosis with polyangiitis (GPA), and microscopic polyangiitis (MPA), age ≥ 18 ≤ 75 yrs, and no ophthalmological disorders. Disease activity was assessed by Birmingham Vasculitis Activity Score (BVAS), damage by Vasculitis Damage Index (VDI), and poorer prognosis by the Five Factor Score (FFS). Quantitative analysis of vessel density (VD) was performed by OCT-A in both superficial and deep capillary plexi. Figures and detailed analysis from NVC were performed for all subjects in the study. Results Included AAV patients (n = 23) were compared with 20 age/sex-matched healthy controls (HC). Retinal VD in superficial whole and parafoveal plexi resulted significantly decreased in AAV compared to HC (P = 0.02 and P = 0.01, respectively). Furthermore, deep whole and parafoveal vessel density was strongly reduced in AAV than HC (P ≤ 0.0001 for both). In AAV patients, significant inverse correlations occurred between VDI and OCTA-VD in both superficial (parafoveal, P = 0.03) and deep plexi (whole, P = 0.003, and parafoveal P = 0.02). Non-specific NVC pattern abnormalities occurred in 82% of AAV patients with a similar prevalence (75%) in HC. In AAV, common abnormalities were edema and tortuosity in a comparable distribution with HC. Correlations between NVC changes and OCT-A abnormalities have not been described. Conclusion Subclinical microvascular retinal changes occur in patients with AAV and correlate with the disease-related damage. In this context, the OCT-A can represent a useful tool in the early detection of vascular damage. AAV patients present microvascular abnormalities at NVC, whose clinical relevance requires further studies.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Subclinical microvascular changes in ANCA-vasculitides: the role of optical coherence tomography angiography and nailfold capillaroscopy in the detection of disease-related damage

Triggianese

D’Antonio

Nesi

et al. 2023

Orphanet J Rare Dis

View full text Add to dashboard Cite

show abstract

“…Besides, although the vasculitis in EGPA can involve small and medium-sized muscular arteries, capillaries, veins and venules, the stenosis and blockage of medium-sized arteries is only described in several case reports [7–20]. Artery occlusion of extremities is extremely rare and only five cases have been documented in adults [10, 13–16].…”

Section: Introductionmentioning

confidence: 99%

A delayed diagnosis of eosinophilic granulomatosis with polyangiitis complicated with extensive artery occlusion of lower extremities in children: case report and literature review

et al. 2019

View full text Add to dashboard Cite

Background Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis in children. A delayed or missed diagnosis of pediatric EGPA is common, owing to the atypical clinical manifestation and limited recognition of this disorder. The vasculitis in EGPA typically involves small to medium size vessels. Extensive occlusion of arteries in the extremities was being extremely rare and has never been reported in children. Case presentation A 10-year and 10-month-old girl with recurrent wheezing and breathlessness during exercise, was initially diagnosed with asthma at the age of five years. Despite unexplained manifestations, including intermittent remarkably increased eosinophilia, uncontrolled allergic rhinitis, and recurrent petechia, from the onset of asthma through to its remission, the consideration of EGPA was completely ignored until the patient presented with aggravated petechia and severe ulceration of the lower extremities, associated with extensive stenosis and/or occlusion of the arteries of the shank and foot. Given her history of asthma, eosinophilia, allergic rhinitis, mononeuropathy, pulmonary infiltrates, and vasculitis confirmed by the skin biopsy, the diagnosis of EGPA was ultimately confirmed. Regrettably, the initial inappropriate and irrational use of corticosteroid failed to relieve the symptoms until more aggressive treatment with intravenous methylprednisolone was started. This was followed by methotrexate treatment, with tapering of prednisone, without relapse over a six-month follow-up. Conclusions Pediatric rheumatologists should be alert to the possibility of EGPA in children with refractory asthma associated with unexplained manifestations, and should be aware of the thromboembolic complications as vascular sequelae of EGPA.

show abstract

Central Retinal Artery Occlusion

Hayreh¹

2014

Ocular Vascular Occlusive Disorders

View full text Add to dashboard Cite

Churg–Strauss syndrome complicated by central retinal artery occlusion: case report and a review of the literature

Cited by 8 publications

References 10 publications

Subclinical microvascular changes in ANCA-vasculitides: the role of optical coherence tomography angiography and nailfold capillaroscopy in the detection of disease-related damage

Subclinical microvascular changes in ANCA-vasculitides: the role of optical coherence tomography angiography and nailfold capillaroscopy in the detection of disease-related damage

A delayed diagnosis of eosinophilic granulomatosis with polyangiitis complicated with extensive artery occlusion of lower extremities in children: case report and literature review

Central Retinal Artery Occlusion

Contact Info

Product

Resources

About