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Diseases of the Peripheral Nerve and Mononeuropathies
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Diana Mnatsakanova, Charles K. Abrams
Primary systemic vasculitic neuropathy occurs in the setting of a disorder with mainly vasculitic manifestations: Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome): neuropathy is common, occurring in 65–80% of cases.Microscopic polyangiitis occurs in > 50% of cases.Polyarteritis nodosa: up to 75% of patients.Granulomatosis with angiitis (Wegener's granulomatosis): 14–40% of patients.Secondary systemic vasculitic neuropathy occurs in the setting of a disorder with nonvasculitic manifestations such as systemic lupus erythematosus (SLE), rheumatoid arthritis, and Sjögren's syndrome. Vasculitic neuropathy is uncommon in SLE and rheumatoid arthritis, but is a relatively common occurrence in Sjögren's syndrome.
Unexplained Fever Associated With Hypersensitivity and Auto-Immune Diseases
Published in Benedict Isaac, Serge Kernbaum, Michael Burke, Unexplained Fever, 2019
Classical polyarteritis nodosa is a necrotizing vasculitis of small- and medium-sized muscular arteries with segmental lesions, sometimes leading to aneurysmal dilatation up to 1 cm in size in medium-sized intraabdominal arteries, sparing the lungs.14,30 Recently, some new aspects of polyarteritis nodosa have been outlined. We will only list them in Table 6 as all can be febrile and have to be known.
Immunologically mediated skin disorders
Published in Ronald Marks, Richard Motley, Common Skin Diseases, 2019
Polyarteritis nodosa is a serious, rare inflammatory disorder of large and medium-sized arteries. Inflammation of the vessel wall, which dilates aneurysmally, causes rupture and ischaemic changes. Central nervous system, cardiovascular, gastrointestinal and renal problems may all arise in this potentially fatal disease. In the skin, a livedo reticularis pattern and persistent ulcers are seen.
Rare forms of inflammatory myopathies - part II, localized forms
Published in Expert Review of Clinical Immunology, 2023
Claudio Galluzzo, Ilaria Chiapparoli, Ada Corrado, Francesco Paolo Cantatore, Carlo Salvarani, Nicolò Pipitone
An important differential of FM is vasculitis limited to lower limb muscles, usually the calf muscles, which can be unilateral or bilateral [60,61]. These patients typically present with localized muscle pain, often worsened by exercise, without or with only mild muscle weakness. On examination, the affected muscles show an induration in 72% of cases. Fever is present in 64% of cases, while weight loss and arthralgia (usually of the ankle joints) has been reported in about or less than half of cases. Progression to systemic vasculitis (polyarteritis nodosa) is very rare (4% of cases). Unlike FM, inflammatory markers are often markedly elevated, while muscle enzymes are raised in only 10% of cases. EMG shows a myopathic pattern, but its sensitivity is rather low (25% of cases). MRI usually shows muscle edema on T2 and STIR sequences, but its findings are nonspecific. Histology is the gold standard to secure the diagnosis: the classical picture is of a polyarteritis nodosa-like vascular inflammation, but a pattern of leucocytoclastic vasculitis can also be observed. Treatment with prednisone (0.5–1 mg/kg/day) is usually efficacious, but relapse rates are high (up to 90%) upon tapering of glucocorticoids; methotrexate or azathioprine can be used in relapsing cases. Spontaneous improvement is infrequent [60,61].
A case of multiple aneurysms with stenosis in the coronary and peripheral arteries of a patient with systemic lupus erythematosus
Published in Modern Rheumatology Case Reports, 2018
Ryosuke Hara, Masahiro Yasumura, Takanori Ichikawa, Tomoki Tanaka, Seiichi Ono, Sadahiro Suzuki, Fusazo Urano
The known causes of CAAs include SLE, congenital disorders (e.g. polycystic kidney disease, Ehlers–Danlos syndrome), Kawasaki disease, syphilis, Takayasu arteritis, polyarteritis nodosa, Behçet’s disease, IgG4-related disease, segmental arterial mediolysis and infectious aneurysm. In this case, the patient had no history of congenital disease or Kawasaki disease, and did not present with infectious disease. Takayasu arteritis often causes lesions in the coronary arteries lesions, as well as vascular obstruction in the extremities; however, no characteristic lesions were detected in the aorta or its branches, and the aneurysms and thrombi occurred in smaller arteries. Although CAA and gangrene of the limbs are rare complications of polyarteritis nodosa, the present case did not satisfy the criteria for this classification. We excluded Behçet’s disease because she lacked oral aphthous ulcers, genital ulcers, abnormal skin lesions and ocular lesions. Her normal level of IgG4 suggests that IgG4-related disease was negative. Segmental arterial mediolysis was also negative because abdominal visceral artery aneurysm was not detected. Therefore, we considered that antiphospholipid antibody syndrome might be responsible for thromboembolism in the peripheral artery. However, this would be a case of chronic inflammation associated with vasculitides, as the onset was not acute and the condition was accompanied by aneurysms.
A case report of cutaneous polyarteritis nodosa in siblings
Published in Modern Rheumatology, 2018
Toshitaka Kizawa, Yuko Yoto, Miyako Mizukami, Takeshi Tsugawa, Takako Takeuchi, Hotaka Kamasaki, Yasue Ishii-Osai, Toshiharu Yamashita, Kazushige Nagai, Tsukasa Hori, Hiroyuki Tsutsumi
Subjects with cutaneous polyarteritis nodosa (CPAN) develop chiefly skin lesions and also have extra-cutaneous findings, such as fever, malaise, arthralgia, and neuropathy; however, unlike patients with polyarteritis nodosa (PAN), they do not develop internal organ involvement, i.e. kidney, heart, and liver [1–3]. Clinical manifestations of CPAN include tender subcutaneous nodules, livedo reticularis, cutaneous ulcers, and necrosis [1,2]. As for the cutaneous lesions, CPAN and PAN share the same histopathological features of necrotizing arteritis of small-to-medium-sized arteries, and four stages have been described, i.e. degenerative, acute inflammatory, granulation tissue, and healed end stage [2].