Table 2

Characteristics of previously reported cases of AAV with muscle weakness

	Age/ sex	Muscle involvement	CK (U/L)	MRI/PET scan	Histology	Other organ involvement	ANCA	CRP (mg/L)
Our patient	82/M	Severe proximal weakness/myalgia	211	No MRI done, but PET normal	Fibrinoid necrosis of medium vessels	None	MPO	182.2
Oiwa et al 45	76/F	Weakness/myalgia	25	MRI showed muscle oedema	Small vessel vasculitis	Pulmonary fibrosis	MPO	84.52
Birnbaum et al 46	77/F	Proximal weakness/myalgia	Normal	MRI showed muscle oedema	Perimysial vasculitis	Pulmonary fibrosis	MPO	NA
Kim et al 47	71/M	Calf pain	Normal	MRI showed abnormal fat infiltration	Necrotising granulomatous vasculitis	Pulmonary fibrosis and Kidney (FNG)	MPO	95.7
Benz et al Case 38	64/M	Myalgia/proximal weakness	Normal	MRI showed diffuse inflammation	Necrotising vasculitis	None	Negative	330
Benz et al Case 18	80/F	Myalgia/proximal weakness	Eight times the limit	MRI showed muscle oedema	Small vessel vasculitis.	None	Negative	289
Benz et al Case 28	79/F	Myalgia/proximal weakness	4000 U/L	MRI showed lipomatous atrophy/involution	Small vessel vasculitis	None	Negative	380
Ojima et al 23	58/M	Myalgia/Muscle weakness	29 998 U/L	MRI showed muscle oedema	Fibrinoid necrosis of arterioles	None	PR3	305
Akagi et al 48	59/M	Calf pain	237 U/L	MRI showed muscle oedema	Fibrinoid necrosis of arterioles	Kidney involvement	PR3	155

AAV, ANCA-associated vasculitis ; ANCA, antineutrophil cytoplasmic antibodies; CK, creatinine kinase; F, female; FNG, focal necrotising glomerulonephritis; M, male; MPO, myeloperoxidase; NA, not available; PET, positron-emission tomography; PR3, proteinase 3.