Pipestem capillaries in necrotizing myopathy revisited

Schröder, Nicolas W.J.; Goebel, Hans‐Hilmar; Brandis, Almuth; Ladhoff, Axel; Heppner, Frank L.; Stenzel, Werner

doi:10.1016/j.nmd.2012.09.001

Cited by 17 publications

(9 citation statements)

References 7 publications

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“…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%

“…[4] Associated disorders in these patients include cerebral infarctions due to vasculitis, neoplasms (bladder transitional cell), and connective tissue disorders. [1,[4][5][6] Our case had overlap syndrome in view of muscle, nerve, skin, and renal involvement, positive ANA and RNP. Overall, our case was similar to the cases reported with regards to clinical presentation, CPK elevation, muscle biopsy showing microangiopathy with sparse inflammation, and being an overlap syndrome.…”

Section: Discussionmentioning

confidence: 74%

“…[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: 85%

“…[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: Discussionmentioning

confidence: 46%

Section: Discussionmentioning

confidence: 74%

Section: Discussionmentioning

confidence: 85%

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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“…Complement binding is present in capillaries and on the surface of muscle fibers. On electron microscopy, pipestem capillaries may occur [ 37 ], but no tubureticular inclusions in endothelial cells (which are present in DM).…”

Section: Clinical Presentation Auto-antibodies and Muscle Histopathomentioning

confidence: 99%

Current Classification and Management of Inflammatory Myopathies

Schmidt

2018

JND

229

199

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Inflammatory disorders of the skeletal muscle include polymyositis (PM), dermatomyositis (DM), (immune mediated) necrotizing myopathy (NM), overlap syndrome with myositis (overlap myositis, OM) including anti-synthetase syndrome (ASS), and inclusion body myositis (IBM). Whereas DM occurs in children and adults, all other forms of myositis mostly develop in middle aged individuals. Apart from a slowly progressive, chronic disease course in IBM, patients with myositis typically present with a subacute onset of weakness of arms and legs, often associated with pain and clearly elevated creatine kinase in the serum. PM, DM and most patients with NM and OM usually respond to immunosuppressive therapy, whereas IBM is largely refractory to treatment. The diagnosis of myositis requires careful and combinatorial assessment of (1) clinical symptoms including pattern of weakness and paraclinical tests such as MRI of the muscle and electromyography (EMG), (2) broad analysis of auto-antibodies associated with myositis, and (3) detailed histopathological work-up of a skeletal muscle biopsy. This review provides a comprehensive overview of the current classification, diagnostic pathway, treatment regimen and pathomechanistic understanding of myositis.

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Skeletal Muscle Biopsy Evaluation

Burns

2019

A Case-Based Guide to Neuromuscular Pathology

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Pipestem capillaries in necrotizing myopathy revisited

Cited by 17 publications

References 7 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

Current Classification and Management of Inflammatory Myopathies

Skeletal Muscle Biopsy Evaluation

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