Churg-Strauss syndrome that presented with mediastinal lymphadenopathy and calculous cholecystitis

No abstract available.

An update of neurological manifestations of vasculitides and connective tissue diseases: a literature review / Atualização das manifestações neurológicas das vasculitides e das doenças do tecido conjuntivo: revisão de literatura

Vasculitides comprise a heterogeneous group of autoimmune disorders, occurring as primary or secondary to a broad variety of systemic infectious, malignant or connective tissue diseases. The latter occur more often but their pathogenic mechanisms have not been fully established. Frequent and varied central and peripheral nervous system complications occur in vasculitides and connective tissue diseases. In many cases, the neurological disorders have an atypical clinical course or even an early onset, and the healthcare professionals should be aware of them. The purpose of this brief review was to give an update of the main neurological disorders of common vasculitis and connective tissue diseases, aiming at accurate diagnosis and management, with an emphasis on pathophysiologic mechanisms.

As vasculites são um grupo heterogêneo de doenças autoimunes primárias ou secundárias a uma grande variedade de doenças infecciosas sistêmicas, malignas ou do tecido conjuntivo. Estas últimas são as que ocorrem com mais frequência, porém os mecanismos patogênicos ainda não foram plenamente determinados. Diversas e frequentes complicações do sistema nervoso central e periférico ocorrem nas vasculites e doenças do tecido conjuntivo. Em muitos casos, os distúrbios neurológicos têm evolução clínica atípica ou mesmo início precoce, ao que todos os profissionais de saúde devem estar cientes. O objetivo desta breve revisão foi atualizar os principais distúrbios neurológicos da vasculite comum e das doenças do tecido conjuntivo, visando ao diagnóstico e ao tratamento corretos, com ênfase nos mecanismos fisiopatológicos.

Infarto agudo do miocárdio como apresentação inicial de granulomatose eosinofílica com poliangiite (anteriormente, síndrome de Churg Strauss) / Acute myocardial infarction as Eosinophilic granulomatosis with polyangiitis (formerly Churg Strauss syndrome) initial presentation

A granulomatose eosinofílica com poliangiite é uma vasculite primária rara, caracterizada por hipereosinofilia, asma de surgimento tardio e granulomas eosinofílicos extravasculares. Relatamos um caso apresentado inicialmente com infarto do miocárdio e que, ulteriormente, teve prosseguimento apenas com asma, manifestações cutâneas e neuropatia periférica. Os parâmetros laboratoriais revelaram hipereosinofilia com um padrão perinuclear negativo de autoanticorpos citoplásmicos antineutrófilos (p-ANCA). A biópsia de pele demonstrou vasculite leucocitoclástica com infiltração eosinofílica, diante de uma angiografia coronária normal. Os sintomas do paciente melhoraram com metilprednisolona IV, pulsoterapia com ciclofosfamida e azatioprina.

Eosinophilic granulomatosis with polyangiitis is a rare primary vasculitic disease characterized by hypereosinophilia, late onset asthma and extravascular eosinophil granulomas. We report a case presented initially with acute myocardial infarction which later only proceed with asthma, skin manifestations and peripheral neuropathy. Laboratory parameters showed hypereosinohpilia with negative perinuclear pattern of antineutrophil cytoplasmic autoantibodies (p-ANCA). Skin biopsy showed leucocytoclastic vasculitis with eosinophilic infiltration while coronary angiography was normal. The patient's symptoms improved with IV methylprednisolone, pulse cyclophosphamide and azathioprine.

Parálisis diafragmática y de cuerda vocal en un paciente cón Síndrome de Churg-Strauss: reporte de un caso / Diaphragmatic and vocal cord paralysis in Churg-Strauss syndrome: a case report

El Síndrome de Churg-Strauss es una vasculitis sistémica que afecta vasos de pequeño y mediano calibre y que suele presentarse con asma, fiebre, hipereosinofilia, insuficiencia cardiaca, daño renal y neuropatía periférica. esta última se observa en el 65 por ciento al 80 por ciento de los casos, siendo el compromiso de nervios craneales en una minoría, y aún más excepcional la parálisis de cuerdas vocales y el diafragma. Las neuropatías por vasculitis sistémicas pueden resultar en morbilidad grave e incluso la muerte, por esto la necesidad de instaurar un tratamiento temprano. Reportamos el caso de un paciente que padeció parálisis diafragmática y de cuerda vocal por síndrome de Churg-Strauss.

Churg-Strauss syndrome is a systemic vasculitis of the small and medium sized vessels that usually occurs with asthma, fever, hypereosinophilia, cardiac failure, renal damage and peripheral neuropathy. The latter affects 65 percent to 80 percent of patients, cranial nerves involvement is rare while vocal cord and diaphragmatic paralysis are exceptional. Neuropathies due to systemic vasculitis may result in significant disability and death, therefore the importance to institute an early treatment. We report here a patient who suffered diaphragmatic and vocal cord paralysis due to Churg-Strauss syndrome.

Anterior Ischemic Optic Neuropathy in a Patient with Churg-Strauss Syndrome

We describe a patient with Churg-Strauss syndrome who developed unilateral anterior ischemic optic neuropathy. A 54-year-old man with a history of bronchial asthma, allergic rhinitis, and sinusitis presented with sudden decreased visual acuity in his right eye that had begun 2 weeks previously. The visual acuity of his right eye was 20 / 50. Ophthalmoscopic examination revealed a diffusely swollen right optic disc and splinter hemorrhages at its margin. Goldmann perimetry showed central scotomas in the right eye and fluorescein angiography showed remarkable hyperfluorescence of the right optic nerve head. Marked peripheral eosinphilia, extravascular eosinophils in a bronchial biopsy specimen, and an increased sedimentation rate supported the diagnosis of Churg-Strauss syndrome. Therapy with methylprednisolone corrected the laboratory abnormalities, improved clinical features, and preserved vision, except for the right central visual field defect. Early recognition of this systemic disease by ophthalmologists may help in preventing severe ocular complications.

Churg-strauss syndrome: a rare systemic vasculitis

Churg-Strauss syndrome is a rare diffuse small/medium sized vessel vasculitis that is almost invariably accompanied by severe asthma. The cause is unknown, but its characteristic histological findings and association with asthma distinguish it from other vasculitides. We report here a middle aged lady who presented with sudden onset of vasculitic skin lesions in association with long standing asthma. Skin biopsy and serology revealed diagnosis of Churg-Strauss syndrome. She was successfully managed with oral prednisolone

Hemorragia pulmonar secundaria a una reactivación tardía de un síndrome de Churg-Strauss en un paciente en hemodiálisis crónica: Report of one case / Late reactivation of Churg-Strauss syndrome

We report a 22 years old male with chronic allergic rhinitis, who presented with asthma, prolonged fever, eosinophilia, cutaneous vasculitis, subcutaneous nodules, polyarthritis, ulcers in the nasal mucosa and external auditory canal, hematuria, proteinuria, renal failure, severe hypertension, pulmonary infiltrates and mesenteric ischemia with a perforation of the sigmoid colon. Arteriography showed multiple aneurysmae of intrarenal arteries and a skin biopsy showed a leukocytoclastic vasculitis. A diagnosis of Churg-Strauss syndrome was made. He was initially treated with steroids and cyclophosphamide but abandoned therapy. Eighteen years after the onset of the disease, he required hemodialysis. Eight months after being on dialysis, he suffered a reactivation of the disease with lung hemorrhage and finally died, due to an upper gastrointestinal bleeding caused by a duodenal ulcer.

Multiple pulmonary nodules.

A 48-year-old post-menopausal female was referred to us in view of her chest radiograph showing multiple pulmonary nodules (Figure 1). She complained of fever and arthralgia since one month. She had been diagnosed as allergic rhinitis with mild episodic asthma since childhood on the basis of symptomatology and was on inhaled steroids and bronchodilators for the same. There was no history of receiving oral corticosteroids or leukotreine antagonists for asthma. Clinical examination did not reveal any abnormality.

Paciente con polineuropatía, eosinofilia y asma / Patient with polyneuropathy, eosinophilia and asthma

RESUMEN. La vasculitis denomina un grupo de enfermedades clínicamente heterogeneas pero con un sustrato histológico común: la presencia de un infiltrado inflamatorio en la pared de los vasos sangíneos. Se dice que las vasculitis constituyen uno de los más complejos capítulos de la patología médica y que su complejidad deriva entre muchas otras cosas de la rareza de los casos, nomenclatura, etc. Aquí describimos un caso de un paciente masculino que se presentó con los hayazgos clásicos del Sindrome de Churg-Strauss. este es el primer caso registrado en la literatura médica hondureña.

Asymmetrical polyneuropathy associated with Churg-Strauss syndrome.

A 26-year-old man who presented with fever, leg edema, weakness and numbness of both hands and feet and painless palpable purpura was reported. He had had a history of asthma and arthalgia before this admission. Physical examination revealed distal asymmetrical polyneuropathy. Electrodiagnostic study was consistent with polyneuropathy. Skin biopsy revealed vasculitis with prominent eosinophilic infiltration. His motor power was markedly improved by prednisolone treatment.

Churg-Strauss syndrome with perforating ulcers of the colon

We report a case of a 72-year-old woman with Churg-Strauss syndrome, who presented with intestinal perforation. She has had bronchial asthma with peripheral blood eosinophilia for 30 years. Gross findings of a resected colon showed multiple ulcers with perforation. Histologic findings demonstrated transmural inflammation infiltrated with large numbers of eosionophils, neutrophils and lymphoplasma cells, and characteristic extravascular granuloma in the subserosa. There were multifocally-distributed transmural vasculitis showing all stages of activity in medium and small-sized arteries and veins located in the submucosa, and proper muscle and subserosal layers of the colon, some of which revealed granulomatous inflammation. Histologic finding of liver showed chronic viral hepatitis B with mild inflammatory activity and macronodular cirrhosis. Immunohistochemical findings, acid fuschin orange G staining and electromicroscope found no evidence of hepatitis B virus infection contributing to the pathogenesis of this lesion.

Vasculite de Churg-Strauss / Churg-Strauss`s vasculitis

Tema livre apresentado no Simpósio Multidisciplinar sobre vasculites, na II Jornada Anual da Sociedade de Reumatologia do Rio de Janeiro, em 22-23 de agosto de 1997, no Colégio Brasileiro de Cirurgiöes, sobre o tema. Universidade do Estado do Rio de Janeiro - Hospital Universitário Pedro Ernesto - Serviço de Dermatologia e Sifilografia

Angeitis alérgica granulomatosa

Presentamos el caso de una mujer de 38 años con asma, eosinofilia periférica, sinusitis y opacidades interticiales difusas, bilaterales y fugaces en la radiografía de tórax. El procedimiento diagnóstico fue biopsia pulmonar a cielo abierto donde se encontró una vasculitis eosinofilica características del Síndrome de Churg-Strauss. Comentamos los principales hallazgos clínicos, radiológicos e histopatológicos.

Angeítis y granulomatosis alérgica (Síndrome de Churg-strauss). Presentación de un caso sin asma

Se presenta un caso comprobado de síndrome de Churg-Strauss, el tercero que se aplica en el país, con dos características clínicas interesantes: ausencia de asma bronquial y atopia y compromiso predominate a nivel del aparato gastrintestinal. Se hace notar que la ausencia de asma no excluye el diagnóstico.