Intravenous immunoglobulin therapy in vasculitic ulcers: a case of polyarteritis nodosa

Pego, Petra M.; Câmara, Inês; Andrade, J.P.; Costa, José Jackson do Nascimento

doi:10.1007/s13317-013-0048-5

Cited by 7 publications

(5 citation statements)

References 36 publications

(25 reference statements)

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“…GC with concomitant use of CY or MTX is recommended as a standard treatment for PAN ( 2 ), and various immunosuppressive treatments, including RTX, have been used for refractory cases ( 3 - 5 ).…”

Section: Discussionmentioning

confidence: 99%

The Successful Treatment of Refractory Polyarteritis Nodosa Using Infliximab

et al. 2017

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Polyarteritis nodosa (PAN), characterized by arteritis of medium-sized blood vessels, is usually treated with a combination of glucocorticoids and immunosuppressants; however, some cases are refractory to these treatments. We herein report the case of a man with PAN that was refractory to various immunosuppressive treatments, including cyclophosphamide, methotrexate, and rituximab. After infliximab (IFX) treatment was initiated, his symptoms improved dramatically and remission was maintained. IFX is considered to be an effective alternative treatment for PAN which proves to be refractory to several immunosuppressive treatments.

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Section: Discussionmentioning

confidence: 99%

The Successful Treatment of Refractory Polyarteritis Nodosa Using Infliximab

et al. 2017

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“…On the basis of data from numerous reports, 40-45% of patients develop a lower-limb neuropathy, more commonly a multifocal neuropathy than a distal polyneuropathy 78,[80][81][82][83][84][85] . Reports of nerve biopsies in patients with cutaneous polyarteritis nodosa are rare, but one revealed vasculitis 86 . The incidence of myopathy has not been adequately investigated, but in one study, all five muscle biopsy samples demonstrated necrotizing vasculitis 77 .…”

Section: Diabetic Lumbosacral Radiculoplexus Neuropathymentioning

confidence: 99%

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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“…[20] In patients with resistant or relapsed disease, the role of intravenous immunoglobulin as a successful option for the treatment of skin lesions has been highlighted in 2 case reports. [21,22] Moreover, the effectiveness of vasodilators and antithrombotic agents, such as prostaglandin I2 analogues, has been reported, especially in the treatment of the skin ulcers and gangrene associated with cPAN [23] and other types of vasculitis. [24] Less aggressive treatments may be warranted in cPAN, as it is not usually associated with life-threatening or progressive outcomes.…”

Section: Discussionmentioning

confidence: 99%

Cutaneous polyarteritis nodosa and pulmonary arterial hypertension: An unexpected liaison. A case report

Berardi,

Antonica,

Procaccio

et al. 2023

Medicine

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Background: Cutaneous polyarteritis nodosa (cPAN) is a form of medium-sized vessel necrotizing vasculitis. It is a rare, skin-limited variant of polyarteritis nodosa, characterized by dermal and subcutaneous tissue involvement. The most common findings in cPAN include digital gangrene, livedo reticularis, and tender subcutaneous nodules. However, while limited to the skin, cPAN results in significant morbidity and mortality due to the accompanying skin ischemia and necrosis, such that patients are vulnerable to superinfection. Here, we describe a unique presentation of cPAN associated with pulmonary arterial hypertension (PAH). Methods: A 78-year-old female presented with digital ischemia and leg ulcers associated with PAH. Skin biopsy showed necrotizing fibrinoid necrosis of the small- and middle-sized vessels of the dermis. A diagnosis of cPAN and PAH was made. The patient was treated with glucocorticoids, vasodilators, and cyclophosphamide. Results: She died due to severe sepsis complications. Conclusion: To date, this is the first case report describing the association between cPAN and PAH. In this case, PAH is a complication of the cutaneous vasculitides suggesting that vasculopathy could play a role in the pathophysiology of PAH. However, the underlying pathophysiological mechanisms still have to be firmly established.

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Intravenous immunoglobulin therapy in vasculitic ulcers: a case of polyarteritis nodosa

Cited by 7 publications

References 36 publications

The Successful Treatment of Refractory Polyarteritis Nodosa Using Infliximab

The Successful Treatment of Refractory Polyarteritis Nodosa Using Infliximab

The nonsystemic vasculitic neuropathies

Cutaneous polyarteritis nodosa and pulmonary arterial hypertension: An unexpected liaison. A case report

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