Myopathy and Neuropathy with Pipestem Capillaries and Vascular Activated Complement Deposition

Reimann, Jens; Kornblum, Cornelia; Tolksdorf, Karen; Brück, W.; Landeghem, Frank K.H. van

doi:10.1212/wnl.0b013e3182267bb1

Cited by 9 publications

(11 citation statements)

References 4 publications

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“…In the case report by Riemann et al, sural nerve biopsy had shown inflammatory infiltrates in the vessel wall with complement deposition, findings that were different from our case. [5] Skin biopsy in our case showed superficial dermal perivascular mononuclear cell infiltrates. In the case report by Authier et al skin biopsy had showed mild fibrosis and perivascular lymphocytic infiltrates in superficial dermis, similar to our case.…”

Section: Discussionsupporting

confidence: 46%

“…In other case reports, similar features were seen. [5,6] Electromyogram in our case had shown spontaneous activity with small amplitude MUPs and increased interference pattern. In a case report by Riemann et al, electromyogram had shown spontaneous activity with only a few recordable MUPs from distal muscles and normal findings in the proximal muscles.…”

Section: Discussionmentioning

confidence: 46%

“…[1][2][3] It presents in the 5-7 th decade with subacute proximal weakness or episodic weakness/myalgias after exercise with or without rash. [1,[4][5][6] Our case was a young female, with progressive weakness, skin hyperpigmentation, and Reynaud's phenomenon. CPK level is our case was 3990 u/L, similar to the high values reported (15-50 times normal).…”

Section: Discussionmentioning

confidence: 83%

“…In a case report by Riemann et al, electromyogram had shown spontaneous activity with only a few recordable MUPs from distal muscles and normal findings in the proximal muscles. [5] In the cases from literature, muscle biopsy had shown necrotizing myopathy, minimal cellular infiltration, and microangiopathy with pipestem vessels (enlarged, thickened, hyalinized capillaries/arterioles with deposition of amorphous PAS positive material), MAC deposition, capillary depletion, and Major Histocompatibility Complex (MHC-I) expression. [1,[4][5][6] The patients described by Engel lacked endothelial microtubular inclusions, perifascicular atrophy, and skin rash seen in DM.…”

Section: Discussionmentioning

confidence: 99%

“…[5] In the cases from literature, muscle biopsy had shown necrotizing myopathy, minimal cellular infiltration, and microangiopathy with pipestem vessels (enlarged, thickened, hyalinized capillaries/arterioles with deposition of amorphous PAS positive material), MAC deposition, capillary depletion, and Major Histocompatibility Complex (MHC-I) expression. [1,[4][5][6] The patients described by Engel lacked endothelial microtubular inclusions, perifascicular atrophy, and skin rash seen in DM. [1] Muscle biopsy in our case had shown necrotic fibers, focal sparse perivascular inflammation, markedly thickened walls of endomysial and epimysial capillaries/arterioles (microangiopathy similar to that described in the previous reports), without PAS-positive material.…”

Section: Discussionmentioning

confidence: 99%

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An unusual case of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries

et al. 2015

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Necrotizing myopathy with pipestem capillaries is a form of chronic inflammatory myopathy, with histopathology showing necrotizing myopathy, minimal cellular infiltration, and microangiopathy. A 30-year-old female presented with progressive limb weakness of 6 months, with skin pigmentation and Raynaud's phenomenon. Serum creatine phosphokinase was 3990 u/L. Muscle biopsy showed necrotic fibers, focal sparse perivascular inflammation/perifascicular atrophy, endomysial/epimysial vessel wall thickening with luminal narrowing. The features were of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries/microangiopathy. She was pulsed with intravenous immunoglobulin, methylprednisolone, and cyclophosphamide and showed a good improvement. In the absence of widespread inflammatory response and classical histopathology findings, it is important to diagnose this condition as it shows a good response to aggressive and prolonged immunotherapy.

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Section: Discussionsupporting

confidence: 46%

Section: Discussionmentioning

confidence: 46%

Section: Discussionmentioning

confidence: 83%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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An unusual case of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries

et al. 2015

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Pipestem capillaries in necrotizing myopathy revisited

Schröder

Goebel

Brandis

et al. 2013

Neuromuscular Disorders

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The nonsystemic vasculitic neuropathies

2017

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The vasculitides are a clinically diverse group of diseases with the histopathological signature of blood vessel-centred inflammation that results in vascular damage and ischaemic injury to the affected tissues 1 . Vasculitis can be caused by drugs, infections and cancers, but diagnostic workup reveals no trigger in most patients. Autoimmune mechanisms are active in all vasculitides, except those caused by direct infection of vessel walls. Most vasculitides are systemic and involve multiple organs and tissues. Those affecting small-to medium-sized vessels often manifest with a vasculitic neuropathy. For example, neuropathies occur in 60-70% of patients with polyarteritis nodosa, eosinophilic granulomatosis with polyangiitis and cryoglobulinaemic vasculitis, and 40-50% of those with microscopic polyangiitis and rheumatoid vasculitis 2 .Vasculitic neuropathy can also occur without systemic vasculitis. This single-organ vasculitis of the PNS has commonly been referred to as nonsystemic vasculitic neuropathy (NSVN), but other forms of clinically isolated PNS vasculitis are now recognized and can be considered variants of NSVN 3 . Although NSVN is classically an acute, relapsing, multifocal neuropathy, it can present as a slowly progressive neuropathy without distinct asymmetries. As such, it should be considered as a possible cause of any progressive axonal neur opathy. NSVN can be diagnosed only with a nerve biopsy, with or without a concomitant muscle or skin biopsy; as nerve biopsies are seldom performed, the condition is under-recognized. The disorder must be distinguished from many other causes of multifocal neuropathy, the definition of which is itself not standardized and requires clarification.A Peripheral Nerve Society guideline group reviewed the literature on vasculitic neuropathy and published consensus recommendations on the classification, diagnosis and immunosuppressive treatment of NSVN in 2010 (REF. 4) Abstract | Nonsystemic vasculitic neuropathy (NSVN) is an under-recognized single-organ vasculitis of peripheral nerves that can only be diagnosed with a nerve biopsy. A Peripheral Nerve Society guideline group published consensus recommendations on the classification, diagnosis and treatment of NSVN in 2010, and new diagnostic criteria for vasculitic neuropathy were developed by the Brighton Collaboration in 2015. In this Review, we provide an update on the classification, diagnosis and treatment of NSVN. NSVN subtypes include Wartenberg migratory sensory neuropathy and postsurgical inflammatory neuropathy. Variants include diabetic radiculoplexus neuropathy and -arguably -neuralgic amyotrophy. NSVN with proximal involvement is sometimes termed nondiabetic lumbosacral radiculoplexus neuropathy. Cutaneous polyarteritis nodosa and other skin-nerve vasculitides overlap with NSVN clinically. Three patterns of involvement in NSVN have been identified: multifocal neuropathy, distal symmetric polyneuropathy, and overlapping multifocal neuropathy (asymmetric polyneuropathy). These patterns lack standard defi...

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Myopathy and Neuropathy with Pipestem Capillaries and Vascular Activated Complement Deposition

Cited by 9 publications

References 4 publications

An unusual case of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries

An unusual case of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries

Pipestem capillaries in necrotizing myopathy revisited

The nonsystemic vasculitic neuropathies

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