Severe proximal muscle weakness with normal CK as a presenting feature of ANCA-associated vasculitis

Nagiah, Sureshkumar; Saranapala, D.M. Manodhi K.

doi:10.1136/bcr-2019-232854

Cited by 13 publications

(12 citation statements)

References 41 publications

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“…ANCA-associated vasculitis rarely presents with muscle weakness, regardless of the serum CK; Nagiah and Saranapala summarised the handful of similar previously reported cases 3. Although this patient had, in retrospect, many features suggesting ANCA-associated vasculitis—such as chronic cough, nasal symptoms and persistently raised inflammatory markers—it was his exertional myalgia, proximal weakness and reduced mobility that precipitated the hospital admission.…”

Section: Discussionmentioning

confidence: 63%

Myeloperoxidase-positive ANCA-associated vasculitis presenting as myalgia, proximal weakness and a normal CK

et al. 2022

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We describe an unusual presentation of myeloperoxidase positive antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis managed by a multidisciplinary approach. A 75-year-old man gave a 3-week history of proximal lower limb weakness and exertional myalgia. His serum creatine kinase was normal and many of his non-specific symptoms suggested small vessel vasculitis. His investigations for common causes of muscle weakness were normal, and renal biopsy was normal despite haemoproteinuria. CT scan of the chest identified a pulmonary nodule of uncertain significance, not amenable to biopsy. MR scan of the thighs showed muscle oedema, and muscle biopsy confirmed typical features of vasculitis. Following high-dose corticosteroids his exertional myalgia quickly resolved and his normal mobility returned. Early immunosuppression is essential to improving clinical outcomes in ANCA-associated vasculitis, but diagnostic investigations often lack sensitivity.

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Section: Discussionmentioning

confidence: 63%

Myeloperoxidase-positive ANCA-associated vasculitis presenting as myalgia, proximal weakness and a normal CK

et al. 2022

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“…Se ha demostrado cierta predisposición genética de HLA-DP para PR3 y HLA-DQ para MPO, lo que avala que son síndromes autoinmunes distintos y posiblemente gatillados por factores externos 27,28 . Las manifestaciones clínicas son variadas, pudiendo limitarse solo al riñón o comprometer la vía aérea superior, los pulmones, la piel entre otros órganos [29][30][31][32][33][34][35][36][37] . El compromiso renal ocurre en el 70% de los pacientes y se suele manifestar como una glomerulonefritis rápidamente progresiva, pudiendo evolucionar en el 50-66% de los casos a insuficiencia renal crónica con un grado variable de ingreso a hemodiálisis crónica.…”

Section: Discussionunclassified

“…El compromiso pulmonar se observa en aproximadamente la mitad de los pacientes y puede variar desde cuadros clínicos con infiltrados pulmonares o nódulos hasta formas severas como fibrosis y hemorragia pulmonar 8,31,38 . Nuestro grupo ha publicado pacientes con formas de presentación infrecuentes 33,34 . Las formas clínicas de inicio más frecuentes fueron las caracterizadas por compromiso renal, pulmonar y otorrinolaringológico, hecho que coincide con lo comunicado en la literatura 8,[25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41] .…”

Section: Discussionunclassified

“…Nuestro grupo ha publicado pacientes con formas de presentación infrecuentes 33,34 . Las formas clínicas de inicio más frecuentes fueron las caracterizadas por compromiso renal, pulmonar y otorrinolaringológico, hecho que coincide con lo comunicado en la literatura 8,[25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41] . Si bien el compromiso renal en forma global fue cercano al 70%, similar al de otras series, en el análisis de subgrupos evidenciamos menor compromiso a lo reportado en la GPA (54.54%) y mayor compromiso en la MPA (100%).…”

Section: Discussionunclassified

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Vasculitis asociadas a ANCA: análisis de 26 pacientes

Young¹

2022

RFEM

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Las vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos (ANCA) comprenden un subgrupo de afección de pequeños vasos que comparten en forma variable la presencia de ANCA. Se analizaron todos los pacientes con vasculitis asociadas a ANCA que fueron atendidos en el Hospital Británico de Buenos Aires durante los años 2000 a 2016 con el objetivo de describir la casuística de nuestro centro. Se incorporaron un total de 26 pacientes: 11 (42.31%) granulomatosis con poliangeítis (GPA), 6 (23.08%) poliangeítis microscópica (MPA) y vasculitis limitada al riñón (VLR) y 3 (11.54%) granulomatosis eosinofílica con poliangeítis (EGPA). La edad media al diagnóstico fue de 51.73±18.85 años (18-78) con predominio de sexo femenino (57.61%). El compromiso inicial más frecuente fue renal (73.08%), seguido por compromiso pulmonar (42.31%) y rinosinusal (26.92%). Cinco pacientes (19.23%) debutaron con síndrome pulmón-riñón y 6 (23.08%) presentaron afectación de más de dos órganos al inicio. La clasificación clínica más común fue la generalizada (34.62%), seguido por la severa (30.77%), con una media de BVAS de 16.6. El 92.31% presentaron determinaciones de ANCA positivas y 76.92% biopsia diagnóstica positiva. Los 26 pacientes realizaron tratamiento de inducción, la adición de ciclofosfamida a glucocorticoides fue la terapia dual más frecuente. En forma global el 50% de los pacientes que ingresaron al estudio lograron remisión, el 61.53% luego de la inducción inicial y el resto luego del tratamiento de la recaída. La mortalidad global fue de 15.38%, ocurriendo durante la inducción inicial, no reportándose eventos durante la remisión o recaída. Del análisis de nuestra serie se desprende que a pesar del compromiso clínico severo es posible reducir la mortalidad con los nuevos esquemas de tratamiento.

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“…As musculoskeletal symptoms in patients with vasculitis, myalgias present as initial symptoms in 22.6%-48.2% and are relatively prevalent in patients with AAV and PAN. [13][14][15][16][17][18][19][20] In clinical practice, the diagnosis of vasculitis is often delayed when patients present with primary complaints of extremity symptoms (muscular pain, edema, numbness, etc.) without evident organ manifestations of vasculitis, such as skin, kidney, or lung lesions.…”

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confidence: 99%

Comparison of characteristics of muscle magnetic resonance imaging findings in patients with antineutrophilic cytoplasmic antibody‐associated vasculitis and polyarteritis nodosa

Ito,

Yoshida,

Fukuda

et al. 2024

Int J of Rheum Dis

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AimThis study aimed to analyze the muscle magnetic resonance imaging (MRI) findings of patients with antineutrophilic cytoplasmic antibody‐associated vasculitis (AAV) and polyarteritis nodosa (PAN) presenting with clinical symptoms in the extremities.MethodsRetrospective analysis was conducted on short tau inversion recovery MRI findings, with a focus on intramuscular vessels displaying abnormal perivascular signals, in 22 and eight patients with AAV and PAN, respectively. The number per unit area (4 cm2) and diameter of abnormal vessels on muscle MRI were compared between patients with AAV and those with PAN. Cut‐off values, clinical sensitivity, and specificity for these indices were calculated from the receiver operating characteristic curves to distinguish between AAV and PAN, and the relationship between the indices and clinical findings in AAV was analyzed.ResultsThe number of abnormal vessels per unit area was significantly higher in AAV compared to PAN (p < .05). Additionally, the diameter of the abnormal vessels was significantly higher in PAN than in AAV (p < .05). The presence of >6.44 abnormal vessels per unit area or ≤3.61 mm diameter of abnormal vessels was able to predict AAV (sensitivity, 0.955; specificity, 0.625). AAV patients with peripheral neuropathy exhibited a significantly higher number of abnormal vessels per unit area than those without peripheral neuropathy (p < .05).ConclusionsMuscle MRI can detect small‐ to medium‐vessel vasculitis and be a valuable tool for distinguishing between patients with AAV and PAN experiencing clinical symptoms in the extremities.

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Severe proximal muscle weakness with normal CK as a presenting feature of ANCA-associated vasculitis

Cited by 13 publications

References 41 publications

Myeloperoxidase-positive ANCA-associated vasculitis presenting as myalgia, proximal weakness and a normal CK

Myeloperoxidase-positive ANCA-associated vasculitis presenting as myalgia, proximal weakness and a normal CK

Vasculitis asociadas a ANCA: análisis de 26 pacientes

Comparison of characteristics of muscle magnetic resonance imaging findings in patients with antineutrophilic cytoplasmic antibody‐associated vasculitis and polyarteritis nodosa

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