ANCA-associated vasculitis and severe proximal muscle weakness

Dutcher, Jordan S; Bui, Albert; Ibe, Tochukwu A; Umadat, Goyal; Harper, Eugene P.; Middlebrooks, Erik H.; Mohseni, Michael; Phillips, Michael

doi:10.1080/08998280.2020.1871177

Cited by 2 publications

(3 citation statements)

References 13 publications

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“…Six previous cases of AAV have been reported that manifested weakness predominantly affecting the proximal muscles (Table 3) (7)(8)(9)(10)(11)(12), although these reports did not emphasize that their muscle weakness was localized. Importantly, all four of our patients did not report truncal muscle weakness, highlighting the possibility that muscle involvement in patients with AAV could be underestimated.…”

Section: Discussionmentioning

confidence: 99%

“…Distal sensory disturbances and muscle weakness are common signs/symptoms of patients with AAV and peripheral nerve involvement. However, there have been several case reports of patients with AAV who present with weakness predominantly in the proximal extremities but do not show sensory deficits, which suggests that muscles are being affected (7)(8)(9)(10)(11)(12). To our best knowledge, all of these published reports are of individual cases, and comprehensive details regarding the clinical and laboratory characteristics, response to treatment, and patient outcomes remain unknown.…”

Section: Introductionmentioning

confidence: 99%

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Antineutrophil cytoplasmic antibody-associated vasculitis with predominant truncal muscle weakness: a retrospective case series

Nemoto,

Nishihara,

Yasuhi

et al. 2023

Front. Neurol.

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IntroductionAntineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) frequently leads to mononeuritis multiplex, which are characterized by distal weakness associated with sensory disturbances. Although AAV has also been reported to be associated with myopathy, the pathogenesis and characteristics remain unclear. We aimed to show the clinical and laboratory findings in AAV-associated myopathy.MethodsThis retrospective single-center study included patients with the diagnosis of AAV who had been admitted to the neurology department and had biopsy specimens of muscle and/or nerve tissue.ResultsWe identified four patients with a distinct clinical presentation of muscle weakness in the trunk and proximal limbs. The weakness resembled that of inflammatory muscle disease. These patients denied symptoms associated with neuropathy, and had normal serum creatine kinase (CK) levels. Needle electromyography (needle EMG) showed spontaneous electrical activity at rest, and results of magnetic resonance imaging (MRI) suggested inflammatory myopathy. Muscle biopsy specimens from all four patients revealed vasculitis and inflammation in proximity to the affected vessels, without any discernible characteristics of other myopathies. The patients also complained of symptoms affecting other organs, such as the ears and kidneys, which is typical of AAV cases. Remission induction therapy, such as cyclophosphamide pulse therapy in addition to oral prednisolone, were effective for all four patients. However, relapses occurred in two patients during maintenance therapy and two patients died of aspiration pneumonia.DiscussionThe clinical course of our patients might represent a subtype of AAV that is characterized by muscle weakness of the trunk and proximal extremities and arises from vasculitis within the muscles. The clinical manifestations of our patients were similar to those of patients with inflammatory myopathy with regard to the distribution of muscle weakness, MRI and needle EMG findings. However, there are notable differences between AAV associated myopathy vs. inflammatory myositis like dermatomyositis; (1) the absence of elevated CK levels, (2) the presence of complications in other organs, (3) distinct pathological findings, and (4) severe outcomes. Awareness that AAV patients with muscle involvement could have a subtype of AAV that seriously affects various organs is critical for an accurate diagnosis and effective therapeutic management.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Antineutrophil cytoplasmic antibody-associated vasculitis with predominant truncal muscle weakness: a retrospective case series

Nemoto,

Nishihara,

Yasuhi

et al. 2023

Front. Neurol.

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“…Hastalıkta birlikte immunsupresif kullanımı, özellikle de steroid kullanımı ile beraber dokümante kas güçsüzlüğü yaygın olarak görülmüş ve raporlanmıştır 8 . Ancak inflamatuvar miyozit birlikteliği aynı sıklıkta değildir.…”

Section: Discussionunclassified

Granülomatöz Polianjitis ve İnflamatuvar Miyozit Birlikteliği: Bir Olgu Sunumu

et al. 2023

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Granülomatöz polianjitis (GPA, eski adıyla Wegener granülomatozisi), en sık üst ve alt solunum yolları ile böbrekleri etkileyen bir hastalıktır. Orta ve küçük çaplı damarlarda nekrotizan vaskülit ve granülomatöz inflamasyon ile karakterizedir. GPA seyri esnasında birçok organda granülomlar veya vaskülit görülebilir, hastalığın tuttuğu organa özgü semptomlar ortaya çıkar. İdiyopatik inflamatuvar miyopatiler (IIM) tanısı geleneksel olarak iskelet kası zayıflığı kliniği ile elektromiyografide spesifik bulgular (spontan fibrilasyonlar, keskin pozitif dalgalar, düşük güçlü/kısa süreli polifazik motor üniteler), kas enzimlerinin yüksek serum seviyeleri ve kas biyopsisinde karakteristik histopatolojik değişikliklerle konulan inflamatuvar hastalıklardır. Miyozit ile ilişkili ve miyozite spesifik otoantikorlar ve manyetik rezonans görüntülemesi (MRI), tanıyı desteklemek için giderek daha fazla kullanılmaktadır. IIM'nin alt türleri şunları kapsar: polimiyozit (PM), dermatomiyozit (DM), inklüzyon cisimciği miyoziti (IBM) ve immün aracılı nekrotizanmiyopati (IMNM). Romatolojik hastalıklarda ortak otoimmün yolakların bulunması sebebiyle ko-insidansa sık rastlanılmaktadır. Ancak küçük damar vasküliti ile inflamatuvar miyozit birlikteliği sık rastlanan bir durum değildir. Bu yazıda, kliniğimizde takip edilen granülomatöz polianjitis ve sonradan gelişen inflamatuvar miyozit birlikteliği olgusu sunulmuştur.

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ANCA-associated vasculitis and severe proximal muscle weakness

Cited by 2 publications

References 13 publications

Antineutrophil cytoplasmic antibody-associated vasculitis with predominant truncal muscle weakness: a retrospective case series

Antineutrophil cytoplasmic antibody-associated vasculitis with predominant truncal muscle weakness: a retrospective case series

Granülomatöz Polianjitis ve İnflamatuvar Miyozit Birlikteliği: Bir Olgu Sunumu

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