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|Year : 2015 | Volume
| Issue : 1 | Page : 72-76
An unusual case of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries
Anil Kumar B Patil, AT Prabhakar, Ajith Sivadasan, Mathew Alexander, Geeta Chacko
Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India
|Date of Web Publication||4-Mar-2015|
Dr. Mathew Alexander
Prof and Head, Neurology, Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu - 632 004
Source of Support: None, Conflict of Interest: None
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.
Keywords: Dermatomyositis; membrane attack complex; necrotizing myopathy; pipestem capillaries
|How to cite this article:|
Patil AB, Prabhakar A T, Sivadasan A, Alexander M, Chacko G. An unusual case of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries. Neurol India 2015;63:72-6
|How to cite this URL:|
Patil AB, Prabhakar A T, Sivadasan A, Alexander M, Chacko G. An unusual case of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries. Neurol India [serial online] 2015 [cited 2021 May 8];63:72-6. Available from: https://www.neurologyindia.com/text.asp?2015/63/1/72/152642
| » Introduction|| |
Necrotizing myopathy with pipestem capillaries was first described by Emslie and Engel (1991) as necrotizing myopathy with sparse inflammation, pipestem capillaries, membrane attack complex (MAC) deposition, and capillary depletion. These cases had neither cutaneous changes nor typical biopsy findings of dermatomyositis (DM), although MAC deposition with capillary loss was seen, suggestive of inflammatory myopathy.  Since then, it is considered as a distinct immune-mediated microangiopathy, different from DM and polymyositis, with about 10 cases reported till now. Here, we report the clinical, histopathological profile, and treatment outcome in a patient with inflammatory necrotizing myopathy and neuropathy with pipestem capillaries.
| » Case Report|| |
A 30-year-old female patient presented with progressive limb and axial weakness, proximal to distal in progression since 6 months. She also had Reynaud's phenomenon, skin hyperpigmentation, and generalized swelling. She was treated elsewhere as inflammatory myopathy with steroids, without showing any significant improvement. On examination, she had weakness in axial and limb muscles (shoulder-2/5, elbow extensors-3/5 and flexors-2/5, wrist power 3/5, hand muscles-moderate weakness, hip and knee-1/5, and ankle-3/5) with absent reflexes. There was skin hyperpigmentation, a dry toe gangrene, and edema of limbs. An overlap syndrome (muscle, nerve, and peripheral vasculature involvement) was considered, and she was further evaluated. Serum creatine phosphokinase (CPK) was 3990 u/L. Nerve conductions showed asymmetric motorsensory neuropathy. Electromyogram showed positive sharp waves/fibrillations from right tibialis anterior, vastus, and biceps brachii. Motor unit potentials (MUPs) were of small amplitude, short duration, and polyphasic from biceps brachii and vastus with increased interference pattern, suggestive of myopathic process with a membrane irritability. Left biceps brachii biopsy [Figure 1]a and [Figure 1]b showed necrotic fibers, regenerating fibers, foci of myophagocytosis, and focal areas of sparse perivascular inflammation. Some of the endomysial and epimysial capillaries/arterioles had markedly thickened walls with luminal narrowing. Periodic acid schiff (PAS) staining was negative. Left superficial peroneal nerve biopsy [Figure 1]c and [Figure 1]d showed loss of myelinated fibers and markedly thickened epineurial arteriolar walls with luminal narrowing. Skin biopsy showed dermal perivascular mononuclear cell infiltration. Direct Coomb's test, antinuclear antibody (ANA) and anti-RNP were positive. The 24-hour total urine protein was 412 mgs. The overall features were suggestive of inflammatory necrotizing myopathy with minimal inflammation and pipestem capillaries. There was microangiopathy involving nerves, skin, and kidneys also, suggestive of an overlap syndrome. She was treated with intravenous immunoglobulin (120 gms initially, followed by 40 gms every 6 weeks for 6 pulses, followed by 30 gms every 8 weeks for 8 pulses), intravenous cyclophosphamide (12 pulses of 700 mg every 6 weeks), and intravenous methylprednisolone (tapered over 40 months). After completing cyclophosphamide pulses, she was started on mycophenolate. There was gradual improvement in limb power with remission by around 36 months. At 60 months of follow-up, she is in remission.
|Figure 1: Muscle and nerve histopathology Left biceps biopsy (H&E stain, 40×) showing focal areas of sparse inflammation and atrophic fibers (a, thin arrow), thickened walls of endomysial and epimysial capillaries and arterioles (A, thick arrow), and necrotic fibers with a focus of myophagocytosis (b, arrow). Left superficial peroneal nerve biopsy (H&E stain, 10×) showing nerve fiber loss (c) and markedly thickened epineurial arteriolar walls with luminal narrowing (d, arrow)|
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| » Discussion|| |
Necrotizing myopathy presents with acute/subacute muscle weakness with a high CPK. It can be associated with interstitial lung disease, cardiomyopathy, anti-signal recognition protein antibodies, and malignancy. Muscle biopsy shows necrotic fibers invaded by macrophages and sparse T-cell infiltrates. With severe illness, the capillaries may be swollen resembling a pipestem. This spectrum of necrotizing myositis with pipestem capillaries is being described as a distinct entity. ,, It presents in the 5-7 th decade with subacute proximal weakness or episodic weakness/myalgias after exercise with or without rash. ,,, 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).  Nerve conduction in our case had shown asymmetric motor sensory neuropathy. In other case reports, similar features were seen. , 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.  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. ,,, The patients described by Engel lacked endothelial microtubular inclusions, perifascicular atrophy, and skin rash seen in DM.  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. Nerve biopsy in our case had showed loss of myelinated fibers and markedly thickened epineurial arteriolar walls. 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.  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.  Associated disorders in these patients include cerebral infarctions due to vasculitis, neoplasms (bladder transitional cell), and connective tissue disorders. ,,, 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. Our patient was younger, PAS-positive material was not seen in vessels wall and changes in epineurial vessels were different from the earlier reported cases. Our case showed a good response to combination of immunoglobulin, methylprednisolone, and cyclophosphamide. Cases reported by Riemann et al. and Authier et al. had a good response with steroids and cyclophosphamide, whereas Engel's cases had a poor outcome. ,, Summary of the presentation of the previously reported cases has been provided in [Table 1]. From the previous case reports and our case, necrotizing myopathy with pipestem capillaries appears to be a distinct entity with varied clinical presentation and histopathological findings, which has to be differentiated from the other idiopathic inflammatory myopathies. [Table 2].
|Table 1: Summary of the cases of necrotizing myopathy with pipestem capillaries|
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|Table 2: Presentation of idiopathic inflammatory myopathy phenotypes [2,3]|
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| » Conclusion|| |
Our patient had the characteristic features of an overlap syndrome with immune-mediated vasculopathy, with muscle, nerve, renal, and skin involvement that included inflammatory necrotizing myopathy and neuropathy with pipestem capillaries/microangiopathy. This appeared to be a distinct form of inflammatory myopathy with associated widespread vascular pathology in other systems also. It can have a good response to aggressive and prolonged immunotherapy even in the absence of prominent inflammatory response. Hence, it is important to diagnose this condition in patients presenting with atypical features of inflammatory myopathy associated with other systemic disorders.
| » Acknowledgments|| |
We thank Dr. Sanjith Aaron, Dr. Vivek Mathew, Dr. Maya Thomas and Dr. Karthik Muthusamy, for their support during the preparation of the manuscript.
| » References|| |
Emslie-Smith AM, Engel AG. Necrotizing myopathy with pipestem capillaries, microvascular deposition of the complement membrane attack complex (MAC), and minimal cellular infiltration. Neurology 1991;41:936-9.
Dalakas MC. Autoimmune inflammatory myopathies. In: Mastaglia FL, Hilton-Jones D, editors. Hand Book of Clinical Neurology. Myopathies. Vol. 86. Amsterdam: Elsevier; 2007. p. 283.
Kooi AJ, De Visser M. Idiopathic inflammatory myopathies. In: Biller J, Ferro FM, editors. Hand Book of Clinical Neurology: Neurological aspects of systemic disease Part 1. Vol. 119. Amsterdam: Elsevier; 2014. p. 497-506.
Authier FJ, Kondo H, Ghnassia RT, Revuz J, Gherardi RK. Necrotizing myopathy with pipestem capillaries and minimal cellular infiltration: A case associated with cutaneous signs of dermatomyositis. Neurology 1996;46:1448-51.
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Acciavatti A, Avolio T, Rappuoli S, Foderi L, Soldati V, Franchi M, et al
. Paraneoplastic necrotizing myopathy associated with adenocarcinoma of the lung - a rare entity with atypical onset: A case report. J Med Case Rep 2013;7:112.
[Table 1], [Table 2]
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