Churg Strauss syndrome presenting as acute neuropathy resembling Guillain Barré syndrome

Riva, Nilo; Cerri, Federica; Butera, Calogera; Amadio, Stefano; Quattrini, Angelo; Fazio, Raffaella; Comola, M.; Comı, Gıancarlo

doi:10.1007/s00415-008-0035-3

Cited by 16 publications

(10 citation statements)

References 10 publications

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“…Although gastrointestinal symptoms are common, CSS is sometimes associated with ulcerative lesion in the gastrointestinal tract, however, perforated ulcers are relatively uncommon and only a limited number of cases have been documented in the literature [17]. Ulceration and perforation of the gastrointestinal tract are assumed to be the result of ischemia caused by vasculitis [18].…”

Section: Discussionmentioning

confidence: 98%

An aggressive and lethal course of Churg–Strauss syndrome with alveolar hemorrhage, intestinal perforation, cardiac failure and peripheral neuropathy

et al. 2009

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Churg-Strauss syndrome (CSS) is a rare type of necrotizing vasculitis affecting small to medium-sized vessels typically characterized by asthma, lung infiltrates, necrotizing granulomas and hypereosinophilia. Herein, we describe a case of CSS presenting severe and aggressive course. A 35-year-old male patient with weight loss, dyspepsia, dyspnea and hemoptysis was admitted. The laboratory analyses indicated a remarkable eosinophilia, elevated levels of serum total IgE and positive cANCA. Thorax CT findings were suggestive of alveolar hemorrhage. Bronchoalveolar lavage revealed alveolar hemorrhage with eosinophilia and transbronchial lung biopsy showed eosinophilic vasculitis. Cardiac enzymes were increased and murmurs were audible revealing cardiomyopathy proven by echocardiography. Pulse cyclophosphamide and methyl prednisolone was immediately started. On the 21st day, intestinal perforation developed and urgent surgery was performed. During a follow-up, although a radiological improvement was observed in the chest X-ray, cardiac failure, peripheral neuropathy and skin lesions developed and high-dose intravenous immunoglobulin and anti-TNF therapy (adalimumab) were applied. Despite the therapy, he died from heart failure and septicemia at 68th day of therapy.

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Section: Discussionmentioning

confidence: 98%

An aggressive and lethal course of Churg–Strauss syndrome with alveolar hemorrhage, intestinal perforation, cardiac failure and peripheral neuropathy

et al. 2009

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“…Polyneuropathy and Guillain-Barré-like syndromes have been described. 56 Central nervous system involvement is much less frequent 57 and no longer carries a poor prognosis ac-cording to the 2011 Five-Factor Score (FFS). 7 Clinical manifestations vary widely, ranging from hemiplegia to seizures, and rare subarachnoid hemorrhages.…”

Section: Pulmonary Manifestationsmentioning

confidence: 99%

Churg-Strauss Syndrome: Clinical Symptoms, Complementary Investigations, Prognosis and Outcome, and Treatment

Dunogué

Pagnoux

Guillevin

2011

Semin Respir Crit Care Med

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Churg-Strauss syndrome (CSS), first described in 1951, is a rare vasculitis of small- and medium-sized vessels. It is characterized by a constant association with asthma and eosinophilia, and by the presence of anti-myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibodies (ANCA) in ~40% of the patients. Vasculitis typically develops in a previously asthmatic and eosinophilic middle-aged patient and most frequently involves the peripheral nerves and skin. Other organs, however, may be affected and must be screened for vasculitis, especially those associated with a poorer prognosis, such as the heart, kidney, and gastrointestinal tract, as assessed by the recently revised Five-Factor Score (FFS). Overall survival of CSS patients is excellent, but relapses are not uncommon and require maintenance or steroid-sparing therapies, depending on the original FFS-based prognosis at diagnosis. All patients require corticosteroids, often for prolonged periods, combined with immunosuppressants [e.g., induction (cyclophosphamide) and maintenance therapy (azathioprine)], for those with poorer prognoses. Recent insights, especially concerning clinical differences associated with ANCA status, showed that CSS patients might constitute a heterogeneous group, both clinically and pathogenically. Future therapies might reflect these differences more strongly.

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“…In 1998, Keven et al reported the case of a patient diagnosed with GBS who later developed ANCA-positive nephrotic syndrome, suggesting that the polyneuropathy could be secondary to necrotizing vasculitis [ 8 ]. Since then, at least 3 other cases of EGPA presenting as GBS have been reported, all with acute ascending polyneuropathy and neurophysiologic studies suggestive of GBS [ 9 – 11 ]. Eosinophilia [ 10 ] and ANCA positivity [ 9 , 11 ] were the factors that led to the correct diagnosis, supported in some cases by treatment failure with IV immunoglobulin [ 10 , 11 ].…”

Section: Discussionmentioning

confidence: 99%

Eosinophilic Granulomatosis with Polyangiitis Presenting as Acute Polyneuropathy Mimicking Guillain-Barre Syndrome

Cámara-Lemarroy

Infante-Valenzuela

Villareal-Montemayor

et al. 2015

Case Reports in Neurological Medicine

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Eosinophilic granulomatosis with polyangiitis (EGPA) is a small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs) which commonly affects the peripheral nervous system. A 38-year-old female with a history of asthma presented with a 2-week history of bilateral lower extremity paresthesias that progressed to symmetric ascending paralysis. Nerve conduction studies could not rule out Guillain-Barre syndrome (GBS) and plasmapheresis was considered. Her blood work revealed marked eosinophilia (>50%), she had purpuric lesions in her legs, and a head magnetic resonance image showed evidence of pansinusitis. Coupled with a history of asthma we suspected EGPA-associated neuropathy and started steroid treatment. The patient showed rapid and significant improvement. ANCAs were later reported positive. ANCA-associated vasculitides present most often as mononeuritis multiplex, but they can mimic GBS and should always be considered in the differential diagnosis, since the treatment strategies for these conditions are radically different.

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Churg Strauss syndrome presenting as acute neuropathy resembling Guillain Barré syndrome

Cited by 16 publications

References 10 publications

An aggressive and lethal course of Churg–Strauss syndrome with alveolar hemorrhage, intestinal perforation, cardiac failure and peripheral neuropathy

An aggressive and lethal course of Churg–Strauss syndrome with alveolar hemorrhage, intestinal perforation, cardiac failure and peripheral neuropathy

Churg-Strauss Syndrome: Clinical Symptoms, Complementary Investigations, Prognosis and Outcome, and Treatment

Eosinophilic Granulomatosis with Polyangiitis Presenting as Acute Polyneuropathy Mimicking Guillain-Barre Syndrome

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