Eosinophilic granulomatosis with polyangiitis in childhood: retrospective experience from a tertiary referral centre in the UK.

OBJECTIVE: To describe the presenting clinical features, treatment and outcome in children with eosinophilic granulomatosis with polyangiitis (EGPA) and to define factors that predicted mortality. METHODS: A retrospective case notes review of patients fulfilling the Chapel Hill Consensus Conference definition and/or ACR criteria for EGPA seen at Great Ormond Street Hospital, London. Demographics, clinical features, histopathology, treatment and outcomes were recorded. Descriptive statistics were expressed as median and range. Fisher's exact test was used for group comparisons. The Paediatric Vasculitis Activity Score and Paediatric Vasculitis Damage Index (PVDI) were calculated. RESULTS: Thirteen children (38% female) aged at diagnosis 14.1 (4-15.6) years were identified. The median time to diagnosis was 2 (0-7.3) years. History of asthma was documented in 76%. The most common presenting features were pulmonary (69%), skin (61%), gastrointestinal (46%), cardiac involvement (46%), paranasal sinus abnormality (38%), arthritis/arthralgia (38%) and neurological involvement (15%). Paediatric Vasculitis Activity Score at presentation was 8/63 (2-25/63); ANCA was negative in all 10/13 patients tested. Treatment included corticosteroids in all, combined with CYC in 38% or AZA in 23%. PVDI at 12 (3-48) months follow-up was 3/72 (0-13/72). Relapses were recorded in 46%. Mortality was 15%; cardiomyopathy and PVDI scores ⩾5 significantly associated with mortality risk (P = 0.012). CONCLUSION: EGPA in the paediatric population is a rare and potentially life-threatening vasculitis. Increased awareness is essential to secure a timely diagnosis and to promptly initiate treatment since our data emphasize a high mortality, particularly in those with cardiac involvement and significant accrued damage.

Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists.

PURPOSE: To determine the association of antineutrophil cytoplasmic antibodies (ANCA) and leukotriene receptor antagonists with disease activity in a large series of patients with Churg-Strauss syndrome. METHODS: Potential subjects were identified by a computerized search of the Mayo Clinic Rochester database for the years 1990 to 2000. Patients meeting one of three classification schemes for Churg-Strauss syndrome were included. RESULTS: Ninety-one patients met the inclusion criteria. Clinical manifestations were similar to those in previous reports. Mortality was similar to that in the general population. ANCA testing was performed in 74 patients. Seventy-three percent (n = 22) of the 30 patients tested before therapy were ANCA positive, as were 75% (n = 12) of the 16 patients tested during a disease flare. In comparison, 16% (n = 8) of the 49 tested during remission were ANCA positive. Serial measurements indicated a correlation of ANCA levels with disease activity. Central nervous system involvement was the only clinical manifestation that correlated with ANCA status (P = 0.05). Twenty-three patients received leukotriene receptor antagonists, of whom 16 (70%) began treatment before diagnosis and 6 (27%) began during remission. Two of those treated after diagnosis relapsed. In 1 patient the relation between disease and leukotriene receptor antagonist use could not be determined. Use of leukotriene receptor antagonists did not affect the time between onset of asthma and manifestations of vasculitis, and was not correlated with organ manifestations, except sinus disease. CONCLUSION: No one classification scheme identified all patients. Churg-Strauss syndrome has a better prognosis than other ANCA-associated vasculitides. ANCA status correlates with disease activity, whereas a pathogenic role for leukotriene receptor antagonists in the development of Churg-Strauss syndrome was not noted.

[Pulmonary eosinophilic granuloma--clinical analysis of 17 patients].

We studied the clinical features of 17 Japanese patients with pulmonary eosinophilic granuloma. Fourteen of the patients were men and three were women; they ranged in age from 19 to 64 years, with a mean of 34 years at the time of the first examination. Pathologic diagnosis in all patients was based on histologic findings of specimens obtained by open lung biopsy. Major symptoms were dry or productive cough, chest pain, dyspnea, and fever; 23.3% of the patients were asymptomatic. Five patients had pneumothorax. Most patients did not have abnormal physical signs. All 17 patients had histories of smoking, and 14 had started to smoke cigaretts before the age of 20 years. Ten patients (58.8%) first presented with cough or dyspnea, and in the other patients (41.2%) the first abnormalities detected were pulmonary infiltrates on chest radiographs during health examinations. Chest roentgenograms usually showed bilateral abnormalities. These abnormalities were distributed over all lung fields in 9 cases (52.9%), in the upper and middle lung fields in 4 cases (23.5%) in the upper lung fields in 3 cases (17.7%), and in the middle lung fields in 1 case (5.9%). Micronodular, reticular, cystic or linear shadows were evident in most cases, and were mixed in various proportions. Eleven patients (65%) had abnormalities of pulmonary function. Low %VC and %FEV1 and high RV/TLC ratios were observed in 20-40% of the patients. Low DLCOs (%DLCO < 70%) were observed in 53% of the patients. Arterial blood gases were normal in 11 of 15 patients. The extent of shadows in the chest roentgenogram was related to the frequency of dyspnea, to the total number of cells in bronchoalveolar lavage fluid, and to the abnormally low %FVC and %FEV1, but not to the %DLCO. Data from bronchoalveolor lavage fluid were non-specific in this disease, but further studies will be needed. Follow-up data were collected on 16 patients. The mean time from the histologic diagnosis after open lung biopsy to the last observation was 81.8 +/- 45.1 months (range, 2 months to 15 years). One patient died of pulmonary eosinophilic granuloma. The usefulness of steroid therapy remains uncertain.

Histiocitosis X pulmonar primaria (granuloma eosinofílico pulmonar)

La histiocitosis X pulmonar primaria es una patología infrecuente que afecta predominantemente a personas entre los 20 y los 40 años, usualmente con historia de tabaquismo. Presentamos un grupo de tres pacientes con esta entidad que se estudiaron en el Hospital Santa Clara en los últimos 20 años. Los síntomas más frecuentes fueron tos con expectoración mucosa y disnea. En todos se documentó historia de tabaquismo. La radiografía de tórax mostró infiltrado intersticial reticulonodular difuso, con volumen pulmonar normal y signos de hipertensión pulmonar precapilar. Todos se presentaron con hipoxemia severa y cor pulmonale. La histopatología confirmó el diagnóstico de histiocitosis X pulmonar primaria.

[Radiotherapy of solitary and multiple eosinophilic granulomas of bone (stage I)]. / Strahlentherapie des solitären und multiplen eosinophilen Granuloms des Knochens (Stadium I).

Between 1957 and 1990 15 patients with solitary (stage Ia by Greenberger) and 2 patients with multiple (stage Ib) eosinophilic granuloma of the bone were treated by radiotherapy in 18 locations after previous surgery. The doses applied varied from 6 Gy to 42 Gy (median for patients under 18 years: 8 Gy, for patients over 18 years: 30 Gy). Median follow-up was 9 years (range, 0.3-33 years). Local control rate and survival were 100%. Three patients with granuloma developed a second granuloma within 13 years after primary diagnosis (two bone manifestations, one cutaneous manifestation). Long-term follow-up of patients with solitary eosinophilic granuloma is necessary.