[Multiple mononeuropathy in the course of the Churg-Strauss syndrome--a case study].
0
Citation
0
Reference
10
Related Paper
Abstract:
The Churg-Strauss syndrome (CSS) is a systemic vasculitis. The symptoms of CSS normally occur between the ages of 20 and 40. We present a case of a 60-year-old man with the CSS evolving in three phases. The initial symptoms included bronchial asthma and inflammation of the ethmoid sinuses. Later, the patient was diagnosed with peripheral blood eosinophilia, pulmonary changes, skin changes and neurological symptoms that progressed to multiple mononeuropathy. Electrophysiological tests confirmed progressive damage of the peripheral nervous system. An improvement of the patient's neurological state was observed after application of corticosteroids and rehabilitation. CSS is one of the causes of multiple mononeuropathy and should be taken into account in differential diagnosis. In patients with bronchial asthma, hypereosinophilia and progressive damage of many nerves, the syndrome is diagnosed in accordance with the criteria defined by the American College of Rheumatology.Keywords:
Mononeuropathy
Hypereosinophilia
Cite
The Churg-Strauss syndrome (CSS) is a systemic vasculitis. The symptoms of CSS normally occur between the ages of 20 and 40. We present a case of a 60-year-old man with the CSS evolving in three phases. The initial symptoms included bronchial asthma and inflammation of the ethmoid sinuses. Later, the patient was diagnosed with peripheral blood eosinophilia, pulmonary changes, skin changes and neurological symptoms that progressed to multiple mononeuropathy. Electrophysiological tests confirmed progressive damage of the peripheral nervous system. An improvement of the patient's neurological state was observed after application of corticosteroids and rehabilitation. CSS is one of the causes of multiple mononeuropathy and should be taken into account in differential diagnosis. In patients with bronchial asthma, hypereosinophilia and progressive damage of many nerves, the syndrome is diagnosed in accordance with the criteria defined by the American College of Rheumatology.
Mononeuropathy
Hypereosinophilia
Cite
Citations (0)
The Churg-Strauss Syndrome (CSS) or allergic granulomatosis and angiitis is a relatively unusual disease. It is a subset of the group of systemic necrotizing vasculitis and the clinical manifestations involve multiple organ system. The CSS is characterized typically by three phases, asthma or atopic disease, peripheral eosinophilia and ultimately, vasculitis. The authors report two cases of CSS. The first patient presented allergic rhinitis and asthma for 19 years when she developed acute abdominal pain, mononeuritis of her right leg, pulmonary infiltrates and hypereosinophilia. The lung biopsy showed extravascular granulomas and eosinophilic infiltration. The second patient presented asthma for two years when she developed a mononeuritis of the left leg and then a hypereosinophilia with cutaneous eruption of hands and feet, and a myo-pericarditis. In both cases, treatment with prednisolone produced a dramatic improvement in symptoms and eosinophilia.
Hypereosinophilia
Mononeuritis Multiplex
Necrotizing Vasculitis
Churg-Strauss Syndrome
Cite
Citations (0)
Churg-Strauss Sindrome or allergic granulomatosis is a small vessel systemic vasculitis characterized by asthma, hypereosinophilia and necrotizing vasculitis with extravascular eosinophil granulomas. We describe a case assisted in our hospital who presented pulmonary infiltrates, fever, peripherical neuropathy, weight loss, myalgia, rhinosinusitis, with antecedents of nasal polyposis, and facial edema. Our aim is to review the main otolaryngologic manifestations of this rare illness that is treated with oral corticosteroids, immunosupresor drugs like ciclofosfamide and plasmapheresis.
myalgia
Hypereosinophilia
Churg-Strauss Syndrome
Necrotizing Vasculitis
Plasmapheresis
Cite
Citations (1)
Objective To further understand and improve the diagnostic accuracy of Churg-Strauss Syndrome.Methods We analyzed the etiology,clinical manifestations,athological characteristic,imaging manifestations,diagnosis,Differential diagnosis and the therapy of Churg-Strauss Syndrome after diagnosing one case and reviewing some literatures.Results The etiology remained unknown,and the clinical manifestations were nonspecific.The major clinical symptoms was asthma,and extrapulmonary manifestations of systemic vasculitis.The pathological manifestation was characterized by hypereosinophilia.For treatment,systemic corticosteroid was helpful.The prognosis was good for most patients.Conclusion Churg-Strauss Syndrome is a rare system vasculitis disease,and easily misdiagnosed.We should highly suspected Churg-Strauss Syndrome,when the patients present asthma,eosinophilia,and vasculitis with granuloma.
Hypereosinophilia
Etiology
Churg-Strauss Syndrome
Systemic vasculitis
Cite
Citations (0)
Churg-Strauss syndrome is a rare disorder characterized by hypereosinophilia and systemic vasculitis which usually occurs in patients with asthma and allergic rhinitis. We, here, presented a case with Churg-Strauss syndrome with no extra-pulmonary presentation despite involvement in peripheral nervous system as in the form of peripheral mononeuropathy in femoral nerve.
Hypereosinophilia
Churg-Strauss Syndrome
Mononeuropathy
Eosinophilic pneumonia
Systemic vasculitis
Peripheral Nervous System
Necrotizing Vasculitis
Cite
Citations (1)
Churg-Strauss syndrome is a rare systemic vasculitis occurring in patients with asthma and blood eosinophilia. Lungs, skin, and nervous system are the most common sites of involvement, although many other organs are affected frequently. The diagnosis often is established from clinical findings or biopsy of extrapulmonary sites, and lung biopsy is performed infrequently. The classic pathologic findings in the lung include a combination of eosinophilic pneumonia, granulomatous inflammation, and vasculitis. All 3 features may not be present in every case, however, and diagnosis often requires careful correlation of the clinical and pathologic findings. The differential diagnosis in the lung includes diseases that are associated with eosinophil infiltrates or a combination of eosinophil infiltrates and granulomatous inflammation. Distinguishing these various diseases from Churg-Strauss syndrome is especially important, since many are more common than Churg-Strauss syndrome, and treatment is usually different.
Eosinophilic pneumonia
Churg-Strauss Syndrome
Systemic vasculitis
Pulmonary Eosinophilia
Lung biopsy
Cite
Citations (137)
In 1951 the pathologists Churg and Strauss identified 13 patients who presented with a clinical syndrome characterised by asthma, hypereosinophilia, and evidence of vasculitis affecting a number of organs.1 The three main histological features found on pathological examination of these cases were extravascular granulomas, tissue eosinophilia, and necrotising vasculitis (fig 1). Most of these patients had been previously diagnosed with “periarteritis nodosa”. After reviewing a number of cases of periarteritis nodosa without asthma and finding no evidence of an eosinophilic granulomatous process, Churg and Strauss suggested that their 13 cases represented a separate disease process and coined the term “allergic granulomatosis and angiitis”. Later “Churg-Strauss syndrome” (CSS) became the accepted title of this distinctive form of systemic vasculitis. More recent pathological case series involving patients with CSS have highlighted the fact that not all patients have the three main histological features originally described by Churg and Strauss.2 3 Given the absence of a histological definition, it was proposed by Lanham et al that the following clinical criteria were required for the diagnosis of CSS: asthma, peripheral blood eosinophilia >1.5 × 109/l (or >10% of total white cell count), and evidence of a systemic vasculitis involving two or more extrapulmonary organs.2 More recently the American College of Rheumatology has developed diagnostic criteria for CSS when there is biopsy proven vasculitis.4 In this review we provide a clinical overview of CSS, review the evidence on which treatment protocols are based, and discuss the current understanding of the pathogenesis of the disease. In addition, we highlight recent controversies in the classification of systemic vasculitis and possible alternative management strategies.
Figure 1
Open lung biopsy specimen from a patient with Churg-Strauss syndrome demonstrating the three main histological criteria originally described by Churg and Strauss. (A) Extravascular granulomas (arrowheads) are seen …
Hypereosinophilia
Systemic vasculitis
Churg-Strauss Syndrome
Polyarteritis Nodosa
Eosinophilic gastroenteritis
Cite
Citations (110)
Churg-Strauss syndrome (CSS) is a rare systemic vasculitis of the small- and medium-size vessels. It is mostly seen in elderly patients presenting as de novo asthma, eosinophilia, and vasculitic organ involvement. In childhood, CSS is extremely rare. The course of pediatric CSS is usually severe and often lethal. We present a case of a 13-year-old girl with a short history of asthma, marked eosinophilia, and multiorgan involvement. The extremely high level of blood eosinophilic granulocytes (51.6 × 109/L) prompted a workup for eosinophilic leukemia before the diagnosis CSS could be made. Subsequently, the disease was successfully treated. This case report shows a classical case of childhood CSS, remarkable because of the presence of extreme hypereosinophilia. It underlines the importance of CSS as a life-threatening cause of hypereosinophilia in children.
Hypereosinophilia
Presentation (obstetrics)
Hypereosinophilic Syndrome
Rare disease
Girl
Cite
Citations (12)
Churg-Strauss syndrome (CSS) or allergic granulomatous angiitis is a rare syndrome that is characterized by hypereosinophilic systemic necrotizing vasculitis affecting small-to medium-sized arteries and veins.In general, it occurs in individuals with pre-existing allergic asthma.When CSS appears in patients, it has the following characteristics: eosinophilia of more than 10% in peripheral blood, paranasal sinusitis, pulmonary infiltrates, histological proof of vasculitis with extravascular eosinophils, and mononeuritis multiplex or polyneuropathy.Therapeutic trials dedicated to Churg-Strauss syndrome have been limited due to the rarity of this disorder and the difficulty in making a histological diagnosis.Proper treatment of patients with CSS is not widely known.In this case study, we report on our experience with an unusual patient case, characterized by purpura and a perforation of the small intestine after inadequate steroid therapy.
Mononeuritis Multiplex
Churg-Strauss Syndrome
Hypereosinophilia
Necrotizing Vasculitis
Hypereosinophilic Syndrome
Perforation
Cite
Citations (4)
Prednisone therapy induced prompt clinical remission in a 38-year-old woman with hypereosinophilic vasculitis. Smoldering vasculitis accounts for the 38% fatality rate in treated patients with the Churg-Strauss syndrome. Complete suppression of the peripheral hypereosinophilia will reflect the efficacy of therapy more accurately than clinical response.
Hypereosinophilia
Churg-Strauss Syndrome
Eosinopenia
Hypereosinophilic Syndrome
Cite
Citations (5)