A case of allergic granulomatosis and angiitis (Churg-Strauss syndrome) in a 15-year-old girl]

A 15-year-old girl with a 6-month history of bronchial asthma was admitted to our hospital because of fever, dyspnea, weight loss, dysesthesia, muscle weakness, gait disturbance and purpuric rash. In addition, leukocytosis, hypereosinophilia and elevation of CRP were observed. Chest radiograph and computed tomography on admission showed non-segmental patchy air-space consolidation in both lung fields. Skin biopsy was performed and the pathologic diagnosis was necrotizing arteritis with eosinophilic infiltration. Transbronchial lung biopsy revealed eosinophilic pneumonia. From the clinical course, laboratory data and pathologic findings, the diagnosis of allergic granulomatosis and angiitis (Churg-Strauss syndrome) was made. Following the skin and lung biopsies, intravenous pulse corticosteroid and oral prednisolone treatment was started and her clinical findings improved. Angiitis with allergic granulomatosis is a vasculitis that is found in adults. This is an extremely rare and interesting case of angiitis with allergic granulomatosis in childhood.     

Lymphocyte subpopulations in allergic granulomatosis and angiitis (Churg-Strauss syndrome)

We report a case of allergic granulomatosis and angiitis (Churg-Strauss syndrome) in which immunologic parameters, including lymphocyte subpopulations, were determined in the acute phase of the disease and during remission. Hyperimmunoglobulinemia E and immune complexes were present. A low proportion of suppressor/cytotoxic (T8+) lymphocytes and a high helper/suppressor ratio were seen throughout the course of the disease, although immunoglobulin levels and circulating immune complex levels decreased with therapy. We hypothesize that the deficiency of suppressor cells may play a role in the pathogenesis of this syndrome.     

Two cases of allergic granulomatosis and angiitis (AGA); Churg-Strauss syndrome

AGA is an angiitis syndrome that has some characteristic features, for example preceding asthma and polyneuritis. And histological findings are granulomatous angiitis or extravascular granuloma. We report two typical cases of AGA. Case 1; 51-year-old woman had been suffering from asthmatic dyspnea for one year and developed in 1987 multiple neuritis in her extremities. Eosinophilia and high level of IgE were noted. Pathologic diagnosis of the biopsied right calf muscle specimen was granulomatous angiitis. Case 2; 40-year-old woman had been suffering from asthmatic dyspnea for two years. She complained of severe cough and myalgia in 1986 and her chest X-ray showed homogeneous shadows in right upper and left lower fields. And her blood showed eosinophilia and high level of IgE. The histology of the biopsied subcutaneous nodules of hands showed extravascular granuloma. These two cases had specific features of AGA. About symptoms of angiitis, case 1 showed multiple neuritis and case 2 had subcutaneous nodules of hands. About laboratory data, case 1 showed WBC count of 9400/mm3 with 85% eosinophils and high level of IgE at 1400 IU/ml, case 2 had WBC count of 13200/mm3 with 22% eosinophils and IgE at 846 IU/ml. The vary of eosinophil count and IgE level were related to the degree and course of illness. These symptoms and laboratory data, except neuritis, improved by an administration of prednisolone. In early stage of AGA, prednisolone is effective, so the criteria of AGA and usage of corticosteroids must be considered.     

A case of the limited from of Wegener's granulomatosis without c-ANCA]

A 46-year-old woman was admitted to our hospital because of fever, cough and headache in December 2001. Although she had been treated for nasal obstruction and epistaxis by an otorhinolaryngologist in our hospital since 1996, no accurate diagnosis had been made despite repeated biopsies of the nasal mucosa. A chest CT taken in 1999 showed ground-glass opacities in both upper lobes. On admission, chest radiography and CT showed mass shadows without cavitation, corresponding to the lesions causing the ground-glass opacities. In addition, paranasal sinus MRI showed a deformity of the nasal septum accompanied by a space-occupying lesion, suggesting Wegener's granulomatosis. However, the cytoplasmic-antineutrophil cytoplasmic antibody (c-ANCA) test was negative. To achieve a definitive diagnosis, we performed an open lung biopsy. The specimen, obtained from the right upper lobe, showed the typical findings of a Wegener's granulomatosis including necrotizing vasculitis. Oral prednisolone treatment initiated at 20 mg daily, combined with oral cyclophosphamide at 50 mg daily markedly improved not only the clinical symptoms, but also the mass shadows in the left upper lobe. Patients with the limited form of Wegener's granulomatosis are occasionally seronegative and respond well to therapy. However, the natural course and the changes in chest radiographs are not understood well in such cases. In this paper, we report a case of the limited form of Wegener's granulomatosis that progressed slowly over a period of 6 years.     

A case of Wegener's granulomatosis without PR3-ANCA at relapse]

A 49-year-old man was admitted to our hospital with fever. His chest radiograph showed some nodules in the right upper and lower lung fields. The cytoplasmic-antineutrophil cytoplasmic antibody test was positive, and histopathologic biopsy of a small nasal polyps yielded a diagnosis of Wegener's granulomatosis. He was started on prednisolone and cyclophosphamide. The findings on his chest radiograph and his symptoms improved rapidly, and we stopped these drugs after one year. Two years after cessation of treatment, his chest radiograph showed two nodules with cavities. Relapse of Wegener's granulomatosis was diagnosed. The proteinase 3-antineutrophil cytoplasmic antibody test was negative. He was started on prednisolone and cyclophosphamide, and the findings on his chest radiograph improved rapidly. Chest radiographs are useful for follow-up observation of patients with Wegener's granulomatosis after treatment.     

Allergic bronchopulmonary aspergillosis progressing to allergic granulomatosis and angiitis (Churg-Strauss syndrome)

A 50-yr-old woman had a right upper lobectomy that revealed the pathologic changes of eosinophilic pneumonia and mucoid impaction. In the mucus plug within the segmental bronchus, aspergillus was demonstrated by direct staining. A diagnosis of allergic bronchopulmonary aspergillosis was made. After a latent period of 17 yr, she presented with the clinicopathologic features of the Churg-Strauss Syndrome. The putative role of aspergillus in the etiology of this syndrome is discussed.     

Bronchial biopsy in allergic bronchopulmonary aspergillosis without clinical asthma]

A 24-year-old man who had had bronchial asthma between the ages of 10 and 12 years was admitted to our hospital on October 10, 2000. In May 1999, he had received antituberculosis therapy for left upper lobe infiltrate, which resolved two months later. Chest radiography on admission showed recurrence of the left upper lobe infiltrate. He complained of cough and low grade fever. Thoracic CT demonstrated gloved-finger shadows in the left upper lung field, as well as central bronchiectasis. Wheeze was not ausculated, and flow volume curve revealed no obstructive changes. Total IgE was markedly increased (6,084 IU/ml), and IgE RAST was positive for multiple allergens including Aspergillus species and precipitating antibody test against Aspergillus fumigatus was also positive. Bronchofiberscopy revealed mucoid impaction at the left B1 + 2, and culture of lavage fluid demonstrated Aspergillus fumigatus. A bronchial biopsy at the orifice of the left upper lobe bronchus revealed thickening of the basement membrane, eosinophil infiltration, and marked hypertrophy of the mucus glands. The diagnosis was allergic bronchopulmonary aspergillosis (ABPA), and 30 mg prednisolone was initiated and tapered. The infiltrate detected on chest radiography was resolved. Eight months later, asthmatic symptoms were observed, and Fluticasone dipropionate administration was started. However, the infiltration seen in the chest radiographs have not recurred until now. Asthmatic inflammation of the bronchial mucosa was demonstrated in a case of ABPA without clinical asthma.     

A case of allergic bronchopulmonary aspergillosis with no history of bronchial asthma]

A 53-year-old male was admitted to our hospital because of an abnormal shadow in the left upper lung. Bronchofiberscopy revealed edematous mucosa and pus at the orifice of the left upper lobe bronchus. Pathological examination revealed bronchial inflammatory change with infiltration of eosinophils and also the existence of aspergillus in the pus. Mild eosinophilia and elevation of serum IgE level were observed in the peripheral blood, and serum precipitin against Aspergillus fumigatus was positive. Bronchogram showed central bronchiectasis, and the diagnosis of allergic bronchopulmonary aspergillosis (ABPA) was made. In cases of ABPA, bronchial asthma is usually present prior to presentation, but this patient had no history of asthma even though airway hyperresponsiveness to methacholine was confirmed.     

A case of Wegener's granulomatosis with pachymeningitis]

A 62-year-old woman who had been receiving corticosteroid therapy for pachymeningitis since 1997 was admitted to our hospital when an abnormal shadow was noticed in her chest radiograph. In bronchial and nasal mucosal biopsies, the findings of a necrotic granulomatous lesion and vasculitis were compatible with Wegener's granulomatosis, although this is rarely seen with pachymeningitis. After further corticosteroid therapy together with cyclophosphamide treatment, the size of the thoracic X-ray shadow decreased. Methicillin-resistant Staphylococcus aureus (MRSA) was cultured from the sputum and the nasal fluid, and may have contributed to the advance of the disease in the airway. This case will require continuing careful observation.     

A case of limited Wegener granulomatosis with hypereosinophilia]

A 51-year-old female was admitted to Nagano Matsushiro General Hospital because of fever, cough and dyspnea on exertion. Her laboratory data revealed leukocytosis with hypereosinophilia, a high erythrocyte sedimentation rate and c-reactive protein. Chest radiography revealed an infiltration shadow with a cavity in the right upper lobe. A lung abscess was diagnosed and antibiotics were administered. Laboratory results showed improvement, but chest radiography continued to show cavities. She was admitted to our hospital because of fever, left pleural effusion and progression of cavities on chest radiographs. She showed no abnormalities of the upper airway or kidney, and was negative for c-antineutrophil cytoplasmic antibody (c-ANCA). Because a positive c-ANCA was seen on day 8 of hospitalization, L-type limited Wegener granulomatosis (WG) was diagnosed according to Gross et al. Prednisolone (PSL) was administered, which improved the anemia, eosinophilia and the cavities. On day 7 of PSL administration, of the left pneumothorax occurred as a complication caused by perforation of the left chest cavity, but her clinical course was good after a cavernectomy was performed. Some studies have reported that limited WG shows a negative c-ANCA, and that antibiotic therapy improves inflammation. The L-type of limited WG revealed a low-grade positive ratio and titer of c-ANCA. Moreover, L-type limited WG responds well to therapy. We therefore selected PSL administration only against L-type limited WG. We have reported L-type limited WG with eosinophilia and the negative effects of c-ANCA at an early clinical stage.     

A case of Wegener's granulomatosis complicated by hypopituitarism]

A 71-year-old male complaining of chest pain was admitted to our hospital. A single cavitary mass shadow was observed on chest X-ray films. Urinalysis revealed microscopic hematuria. CT examination demonstrated a tumorous shadow in the maxillary sinus. The diagnosis of Wegener's granulomatosis was histologically established by biopsy specimens from the nasal mucosa which showed necrotizing vasculitis and granuloma with fibrinoid degeneration. He was treated with combination therapy of prednisolone and cyclophosphamide. The abnormal shadows on chest X-ray and in the maxillary sinus on CT improved rapidly, but the patient developed progressive weight loss and complained of cold intolerance, weakness and dysphagia. Serum T3, T4 and TSH were found to be reduced. Anterior pituitary function tests showed reduction of TSH, GH and ACTH responses, which was probably due to irreversible vasculitis.     

A case of bronchocentric granulomatosis associated with uveitis]

A 32-year-old female was admitted to our hospital because of abnormal pulmonary shadows and a decrease in visual acuity. Analysis of peripheral blood revealed eosinophilia, and chest roentgenogram demonstrated multiple infiltrates in the right upper lung field. Pathological examination of transbronchial lung biopsy specimens revealed necrotizing granulomatous lesions in the walls of bronchioles, and a definitive diagnosis of bronchocentric granulomatosis was made. The cause of bronchocentric granulomatosis in this patient was suggested to be an allergic reaction to Aspergillus because of positive response to skin test for Aspergillus. Although it is reported that extrapulmonary involvement is rare in bronchocentric granulomatosis, the present case was associated with uveitis, and to our knowledge is the first reported case.