Manejo anestésico de un paciente con síndrome de Churg-Strauss en el contexto ambulatorio: informe de un caso / Anesthesia management in a patient with Churg-Strauss syndrome in the ambulatory setting: A case report

La granulomatosis eosinofílica con poliangitis, también denominada síndrome de Churg-Strauss, es una vasculitis rara con compromiso multisistémico. Existe poca literatura en cuanto al enfoque anestésico de los pacientes con esta enfermedad. A nuestro saber, presentamos aquí el primer informe sobre cirugía ambulatoria en un paciente con granulomatosis eosinofílica con poliangitis. Este caso subraya la preocupación por la seguridad de la cirugía ambulatoria y el manejo anestésico exitoso de un paciente con granulomatosis eosinofílica con poliangitis con anestesia regional.(AU)

Eosinophilic granulomatosis with polyangiitis, also known as Churg-Strauss syndrome, is a rare type of vasculitis with multisystemic involvement. Very few authors have described the anaesthesia technique in these patients. We present the first report on ambulatory surgery in a patient with eosinophilic granulomatosis with polyangiitis. This case dispels concerns about the safety of day surgery and reports successful regional anaesthesia management in a patient with eosinophilic granulomatosis with polyangiitis.(AU)

Gynecological involvement in eosinophilic granulomatosis with polyangiitis.

Gynceological involvement in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides is rare. We describe a case of ANCA-associated vasculitis in an elderly woman presenting with fevers, cough and a rash. Further investigations revealed vasculitic involvement of the ovaries, fallopian tubes, uterus and omentum with an eosinophil-rich infiltrate.

[Anesthetic management of laparoscopic cholecystectomy for a patient with Churg-Strauss syndrome: a case report].

Churg-Strauss syndrome (CSS) is an uncommon disease characterized by bronchial asthma, eosinophilia and systemic vasculitis. Many patients with CSS are suffering from cardiovascular disorders, neurological disorders and/or renal disorders which are associated with systemic vasculitis. Cardiac diseases are considered as the main cause of the death in patients with CSS. Steroid administration is the standard pharmacological therapy for CSS. There are very few clinical reports concerning anesthetic management for the patients with CSS. We suppose that precise perioperative managements are required for the patients with CSS, including the appropriate control of bronchial asthma and the careful treatments of disorders in cardiovascular system, neurological system and/or kidney. In addition, we believe that the steroid cover should be considered during the perioperative period of the patients with CSS. Here, we describe an anesthetic management of a 28-year-old man with CSS undergoing laparoscopic cholecystectomy. General anesthesia was induced with midazolam and fentanyl. Rocuronium was administered to facilitate tracheal intubation. After tracheal intubation, anesthesia was maintained with sevoflurane and remifentanil. Prior to the surgery, 100 mg of hydrocortisone was administered for the steroid cover. The surgery was uneventful. The patient emerged from general anesthesia smoothly, and was extubated safely.

Heart transplantation in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).

BACKGROUND: Heart involvement is the leading cause of death of patients with eosinophilic granulomatosis with polyangiitis (EGPA; formerly Churg-Strauss syndrome) and is more frequent in anti-neutrophil cytoplasm antibody (ANCA)-negative patients. Post-transplant outcome has only been reported once. METHODS: We conducted a retrospective international multicenter study. Patients satisfying the criteria of the American College of Rheumatology and/or revised Chapel Hill Consensus Conference Nomenclature were identified by collaborating vasculitis and transplant specialists, and the help of the Churg-Strauss Syndrome Association. RESULTS: Nine ANCA(-) patients who received transplants between October 1987 and December 2009 were identified. The vasculitis and cardiomyopathy diagnoses were concomitant for 5 patients and separated by 12 to 288 months for the remaining 4 patients. Despite ongoing immunosuppression, histologic examination of 7 (78%) patients' explanted hearts showed histologic patterns suggestive of active vasculitis. The overall 5-year survival rate was low (57%), but rose to 80% when considering only the 6 patients transplanted during the last decade. After survival lasting 3 to 60 months, 4 (44%) patients died sudden deaths. CONCLUSIONS: The search for EGPA-related cardiomyopathy is mandatory early in the course of this type of vasculitis. Indeed, prompt treatment with corticosteroids and cyclophosphamide may achieve restore cardiac function. Most patients in this series were undertreated. For patients with refractory EGPA, heart transplantation should be performed, which carries a fair prognosis. No optimal immunosuppressive strategy has yet been identified.

The Churg-Strauss syndrome: diagnosed for the first time in a gastrectomy specimen.

The Churg-Strauss syndrome (CSS), first described in 1951 and characterised by eosinophilic inflammation and necrotising vasculitis in patients with asthma can in principle affect any organ. We report on a 47-year-old male patient, in whom the diagnosis of CSS was finally established for the first time on the basis of the histological work-up of a gastrectomy specimen. Endoscopic inspection had revealed a rigid gastric wall and a large irregularly shaped ulcer in the prepyloric antrum. Despite the fact that no carcinoma was demonstrable in the biopsy material, a gastrectomy was nevertheless performed since the endoscopic appearance was strongly suspicious for a carcinoma. The gastrectomy specimen revealed massive eosinophilic gastritis in combination with granulomas and necrotising vasculitis--localised mainly in the muscularis propria. In view of the subsequent information that the patient also suffered from asthma, the diagnosis of CSS (previously unrecognised) was established--for the first time--in the gastrectomy specimen.

Bowel perforations in a patient affected by Churg-Strauss syndrome under high-dose steroid treatment: will alternative drugs reduce risk of surgery?

Churg-Strauss syndrome is a relapsing-remitting vasculitis that frequently involves digestive system. Ischemic perforation of the large bowel is relatively rare and potentially life threatening. We report a case treated with high dose of steroids for a relapsing of Churg-Strauss vasculopathy that underwent emergency surgery for multiple large-bowel perforations. Massive use of steroids is common for controlling relapse of this disease, but this increases the risk of intestinal perforation. A prompt switching to alternative drugs when intestinal tract is involved should be considered in order to prevent surgery.

Living related intestinal transplantation for Churg-Strauss syndrome: a case report.

Exceptionally, gastrointestinal involvement of Churg-Strauss syndrome (CSS) may require extensive bowel resection resulting in a short bowel syndrome. Living related intestinal transplantation (IT) has emerged as an alternative to deceased-donor IT in the management of patients with irreversible short bowel syndrome. Herein, we have presented a 35-year-old patient with isolated intestinal involvement of CSS lesions refractory to steroids and azathioprine requiring multiple abdominal resections resulting in an ultrashort bowel syndrome. A living related IT (from the mother) was performed. She underwent several acute rejection episodes treated with additional immunosuppressive therapy. Despite higher doses of immunosuppression, these repeated acute rejection episodes eventually evolved into a syndrome of chronic allograft rejection. Eventually, owing to her poor general condition and to avoid life-threatening infections, transplantectomy was inevitable. Recent immunologic studies indicate that peripheral mononuclear cells from patients with CSS secrete large amounts of T-helper type 1 and 2 cytokines. It is likely that patients with CSS are at higher risk for acute and chronic rejection after transplantation.

Is routine histopathological examination of FESS material useful?

Analysis of the clinical value of histopathological examination of material collected during functional endoscopic sinus surgery. Retrospective analysis of collected data of patients undergoing functional endoscopic sinus surgery over a 7-year period. Data were collected from a pathology database of the Sint Lucas Andreas Hospital, Amsterdam, The Netherlands. All material collected from 1,944 functional endoscopic sinus surgery cases in 1,695 patients (some patients underwent revision surgery) operated between 2000 and 2007 was examined histologically. Patients with a histological outcome different from chronic inflammation or polyposis nasi, were checked on indication of surgery, type of surgery and preoperative clinical suspicion for (pre)malignancy. The impact of the histological diagnosis on the clinical course was evaluated. Thirty-seven unusual diagnoses were reported: 18 cases of inverted papilloma, one squamous cell carcinoma, two malignant lymphomas, one leiomyosarcoma, eight squamous cell papillomas, one Churg Strauss syndrome, one Schneiderian papilloma, two cases of Wegener's granulomatosis and three cases of sarcoidosis. All other specimens were identified as chronic inflammation and/or nasal polyposis. Only two clinically significant, unexpected diagnoses, both inverted papilloma, have resulted from the histological investigation. We conclude that the close correlation between histopathology outcome and examination on clinical grounds or gross inspection indicates that routine histopathological examination of functional endoscopic sinus surgery material has little clinical value and it is questionable if this should be performed. In patients in whom there is a high degree of suspicion for (pre)malignancy, histopathological examination of functional endoscopic sinsus surgery material remains indicated.

Primary vasculitis at high altitude.

We describe a 34-year-old mountaineer who presented with gut infarction from necrotizing vasculitis, probably due to Churg-Strauss syndrome. Subsequently, relapses occurred whenever he climbed to 4000 meters. We hypothesize that the effects of vasculitis were compounded by the physiological changes at high altitude. We suggest that patients with systemic vasculitis should be cautious about climbing and trekking at high altitude.

[Churg-Strauss syndrome--case report]. / Zespól Churga i Strauss--opis przypadku.

A case of Churg-Strauss syndrome in patient admitted to the Department of Rheumatology with suspected obliterative atherosclerosis is described. Churg-Strauss syndrome is an uncommon disease and may therefore has not been immediately recognized. The diagnosis was based on course of the disease, clinical manifestations, laboratory and serological tests. Treatment with methylprednisolone and cyclophosphamide resulted in dissolutions of many disease symptoms.

Multiple perforated ulcers of the small intestine associated with allergic granulomatous angiitis: report of a case.

Although allergic granulomatous angiitis (AGA) is occasionally associated with gastrointestinal lesions, multiple perforated ulcers of the gastrointestinal tract are uncommon. We report herein a case of AGA associated with multiple perforated ulcers that erupted in the small intestine during corticosteroid therapy. A 31-year-old Japanese man was admitted to our hospital with epigastralgia, edema of the bilateral lower extremities, and general malaise. He had a persistent high fever, abdominal pain, and watery diarrhea. Laboratory data showed remarkable eosinophilia. The abdominal pain became exacerbated after the administration of oral prednisolone. Physical examination indicated positive signs of peritoneal irritation in the entire abdomen, and abdominal computed tomography scanning showed intra-abdominal free air, suggesting generalized peritonitis due to intestinal perforation. Laparotomy revealed multiple perforated ulcers in the jejunum and ileum. Histological examination indicated remarkable eosinophilic infiltration in the surrounding area of the small arteries and arterioles located in the submucosal layer, which was compatible with AGA. Although the association of intestinal perforation with AGA is relatively infrequent, intensive perioperative management is essential to ensure a favorable clinical outcome, particularly during the period on corticosteroid therapy.

Churg-Strauss syndrome with critical endomyocardial fibrosis: 10 year survival after combined surgical and medical management.

A case is presented of the Churg-Strauss syndrome with hypereosinophilia and severe cardiac involvement, namely biventricular endomyocardial fibrosis and gross encroachment of the right ventricular cavity. The clinical picture was similar to Loeffler's syndrome and the idiopathic hypereosinophilic syndrome. Combined aggressive surgical and medical management led to full recovery and survival at 10 years. The good long term outcome is attributed to strict control of peripheral eosinophil count by oral corticosteroids. This case illustrates the damaging effects of hypereosinophilia on the heart.

Churg-Strauss syndrome complicated by eosinophilic endomyocarditis.

A 34-year-old woman with asthma had increasing dyspnea on exertion for 9 months and new-onset mononeuritis multiplex. An examination demonstrated sinus tachycardia, elevated jugular venous pressure, and a tender nonpulsatile liver. The leukocyte count was 15.8 x 10(9)/L, with 23% eosinophils. Echocardiography revealed a laminated thrombus obliterating much of the right ventricular cavity, with encasement of the tricuspid valve. Ultrafast computed tomography showed no evidence of pulmonary emboli. Biopsy specimens of skin nodules revealed extravascular palisading granulomas. The thrombus was refractory to corticosteroids, and right ventricular thrombectomy was performed. To our knowledge, this is the third reported case of Churg-Strauss syndrome with thrombotic complications from coexistent eosinophilic endomyocarditis. In an asthmatic patient with chronic dyspnea, eosinophilic tissue infiltration, and neuropathy, Churg-Strauss syndrome should be considered; evaluation for cardiac involvement may be warranted.