Response to: Correspondence on ‘2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis’ by Joanna C Robson<i>et al</i>and ‘2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis’ by Pimentel-Quiroz<i>et al</i>

Grayson, Peter C.; Robson, Joanna; Suppiah, Ravi; Ponte, Cristina; Watts, Richard A.; Luqmani, Raashid; Merkel, Peter A.

doi:10.1136/annrheumdis-2022-222362

Cited by 21 publications

(23 citation statements)

References 7 publications

(7 reference statements)

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“…According to the 2022 AAV classification criteria (eTable 1), the presence of mononeuritis multiplex and episodes of peripheral eosinophilia qualified the patient for the diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA). 3 Corticosteroid was initiated (80mg/d, i.v. ), resulting in a remarkable improvement in weakness and fever, as well as a reduction of peripheral eosinophils (0.2 x 10 9 /L, 1.4%).…”

Section: Sectionmentioning

confidence: 99%

“…Previously known as Churg-Strauss syndrome, it is an ANCA-associated vasculitis (AAV) subtype histologically defined by eosinophil-rich, necrotizing granulomatous inflammation mainly affecting the small-sized arteries. 3 According to recently updated criteria, 3,4 three core features, i.e. peripheral eosinophilia, obstructive airway disease and nasal polyps, constitute the hallmarks of EGPA diagnosis.…”

Section: Sectionmentioning

confidence: 99%

“…Mononeuritis multiplex and eosinophilic inflammation on biopsy serve as important discriminant classifiers. 3 By contrast, cANCA/anti-PR3 positivity and hematuria are red flags indicative of an alternative diagnosis. 3 Neurological complications of EGPA are common, including PNS involvement in 53-78% of patients.…”

Section: Sectionmentioning

confidence: 99%

“…3 By contrast, cANCA/anti-PR3 positivity and hematuria are red flags indicative of an alternative diagnosis. 3 Neurological complications of EGPA are common, including PNS involvement in 53-78% of patients. 5 Our patient presented with an acute onset mononeuritis multiplex with concomitant systemic findings.…”

Section: Sectionmentioning

confidence: 99%

“…Firstly, EGPA is the AAV subtype characterized by a low prevalence of ANCA positivity (only 40-60%). 3 A negative ANCA testing should not exclude the diagnosis of AAV. Secondly, by the time of evaluation, the confluence of sequential neuropathies may falsely lead to a clinical impression of generalized axonal polyneuropathies.…”

Section: Sectionmentioning

confidence: 99%

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Clinical Reasoning: A 60-Year-Old Man With Asymmetric Weakness and Persistent Fever

et al. 2023

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Peripheral neuropathies, especially those with atypical features, remain a diagnostic challenge. In this case, a 60-year-old patient presented with acute-onset weakness starting in the right hand then sequentially involving the left leg, left hand, and right leg over 5 days. The asymmetric weakness was accompanied by persistent fever and elevated inflammatory markers. Subsequent development of rashes combined with careful review of the history led us to the final diagnosis and targeted treatment. This case highlights clinical pattern recognition with the help of electrophysiological studies in peripheral neuropathies, which provide shortcuts to narrow the differential diagnosis. We also illustrate important pitfalls from history taking to ancillary testing in diagnosing rare but treatable cause of peripheral neuropathy (eFigure 1).

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Section: Sectionmentioning

confidence: 99%

Section: Sectionmentioning

confidence: 99%

Section: Sectionmentioning

confidence: 99%

Section: Sectionmentioning

confidence: 99%

Section: Sectionmentioning

confidence: 99%

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Clinical Reasoning: A 60-Year-Old Man With Asymmetric Weakness and Persistent Fever

et al. 2023

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Granulomatosis With Polyangiitis Presenting With Strawberry Gums

2023

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Takayasu arteritis associated with autoimmune/inflammatory syndrome induced by adjuvants: a case-based review

2023

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Takayasu's arteritis (TA) is a chronic granulomatous vasculitis that predominantly affects the aorta and its major branches. Despite advancements in the understanding of the pathogenic pathways of vascular inflammation, the etiology and predisposing factors of TA remain to be fully understood. In susceptible individuals, exposure to adjuvants may trigger, unlock or unmask an autoimmune disorder, presenting as non-specific constitutional symptoms or a fully developed autoimmune syndrome such as vasculitis. Here, we hypothesize that TA could be triggered by siliconosis, a subtype of the autoimmune/ inflammatory syndrome induced by adjuvants (ASIA). ASIA, also known as Shoenfeld syndrome, encompasses a wide range of autoimmune and immune-mediated diseases resulting from dysregulation of the immune response after exposure to agents with adjuvant activity. This case report describes the development of large artery vasculitis, TA, in an individual one year following the placement of silicone breast implants. The patient initially presented with non-specific symptoms, and multiple imaging methods were employed, including ultrasound diagnostics, CT angiography, and 18-fluorodeoxyglucose positron emission tomography/CT. These techniques revealed vasculitic alterations in the carotid arteries and thoracic aorta. Initial treatment with glucocorticosteroids proved ineffective, prompting the addition of steroid-sparing immunosuppressive agents. Due to the distinct clinical symptoms, disease progression, implant-associated fibrosis, and resistance to therapy, the potential involvement of implants in the development of large-vessel vasculitis was considered, and a potential association with ASIA was postulated. Although there is limited evidence to support a direct link between adjuvants and the pathogenesis of TA, similarities in cellular immunity between the two conditions exist. The diagnosis of this complex and potentially debilitating condition requires a comprehensive clinical examination, laboratory evaluation, and instrumental assessment. This will aid in identifying potential contributing factors and ensuring successful treatment. KeywordsAutoimmune/inflammatory syndrome induced by adjuvants • Autoimmunity • Breast implants • Immunologic adjuvants • Silicone gels • Takayasu arteritis • Large vessel vasculitis • Case report

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Cited by 21 publications

References 7 publications

Clinical Reasoning: A 60-Year-Old Man With Asymmetric Weakness and Persistent Fever

Clinical Reasoning: A 60-Year-Old Man With Asymmetric Weakness and Persistent Fever

Granulomatosis With Polyangiitis Presenting With Strawberry Gums

Takayasu arteritis associated with autoimmune/inflammatory syndrome induced by adjuvants: a case-based review

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