Bronchocentric granulomatosis and pulmonary hydatidosis

Bronchocentric granulomatosis is a non-specific necrotizing granulomatous reaction in the bronchi and bronchioles. In asthmatic patients, bronchocentric granulomatosis is considered a hypersensitivity reaction to intrabronchial fungi. However, in non-asthmatic patients the possible etiology has not been identified. We report the case of a 46-year-old woman with bronchocentric granulomatosis associated with pulmonary hydatidosis. The likely immuno-pathogenic mechanisms leading to bronchial lesions are briefly discussed.     

Bronchocentric granulomatosis with glomerulonephritis

Bronchocentric granulomatosis is a chronic pulmonary disease treated with short-term therapy with corticosteroids, and the disease has an excellent prognosis. We describe a patient, and review an additional case from the literature, in whom bronchocentric granulomatosis was accompanied by glomerulonephritis. A misdiagnosis of Wegener's granulomatosis was made, and therapy with cyclophosphamide was either considered or given in each of these cases. We emphasize the need for careful histopathologic evaluation of open lung biopsies in patients suspected of having Wegener's granulomatosis in order to rule out the possibility of bronchocentric granulomatosis with concurrent renal disease, and thus avoid the unnecessary use of cytotoxic agents.     

Bronchocentric granulomatosis associated with influenza-A virus infection

Bronchocentric granulomatosis is an unusual pathologic entity that is characterized by a necrotizing granulomatous inflammation surrounding the airways. The diagnosis is usually made retrospectively, after histopathologic examination of an open-lung biopsy or resection of a pulmonary lesion. Although the aetiology has not been fully elucidated, the current pathogenetic mechanism is considered to be an immunologic reaction against endobronchial antigens, since most patients exhibit signs of bronchial asthma, eosinophilia and allergic bronchopulmonary aspergillosis. However, non-asthmatic patients may develop bronchocentric granulomatosis without signs for endobronchial fungal infections, but probably as a consequence of other pulmonary infections. An 80-year-old female patient presented with high fever and bilateral pulmonary infiltrates and nodules. After extensive investigations and open-lung biopsy, the diagnosis bronchocentric granulomatosis was established that was possibly associated with an influenza-A virus infection. Treatment consisted of immunosuppressive drugs (prednisone and cyclophosphamide), which led to complete clinical and radiological recovery.     

Bronchocentric granulomatosis and rheumatoid arthritis

We report a 42-year-old patient with an 11-year history of rheumatoid arthritis (RA) who presented 8 years later with haemoptysis and was found to have multiple pulmonary opacities with histological features of bronchocentric granulomatosis (BCG). The association between BCG and RA is discussed and the literature is reviewed.     

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.     

Bronchocentric granulomatosis due to Aspergillus terreus in an immunocompetent and non-asthmatic woman.

This is the first report of bronchocentric granulomatosis due to Aspergillus terreus in a healthy and non-asthmatic 74-year-old Japanese woman. Following identification of the fungus, oral itraconazole therapy was begun after intrabronchial infusion of amphotericin B. No recurrence has occurred after treatment for 24 months. We should consider the possibility of bronchocentric granulomatosis including Aspergillus terreus, when an intrabronchial lesion is found even in a healthy and non-asthmatic person. Oral itraconazole after intrabronchial infusion of amphotericin B seems to be effective in such cases.     

Erosive polyarthritis as presenting manifestation of Wegener's granulomatosis

Summary We describe a 55-year old female with an erosive symmetric polyarthritis and negative rheumatoid factor tests. A preliminary diagnosis was made of sero-negative rheumatoid arthritis. About two years after the first signs of arthritis, a diagnosis was made of Wegener's granulomatosis, based on bloody nasal discharge with active inflammation of the nasal mucosa with giant cells, the development of a saddle nose deformity, micro-haematuria and a strongly positive test for c-ANCA. After treatment with prednisone and cyclophosphamide the patient markedly improved. Wegener's granulomatosis has to be considered in a patient presenting with (erosive) (poly) arthritis.     

Asbestosis,endobronchialAspergillus infection,and bronchocentric granulomatosis presenting with hemoptysis

A patient with pulmonary asbestosis developed hemoptysis and a lesion on chest radiograph secondary to bronchocentric granulomatosis (BG) associated withAspergillus fumigatus. Although pulmonaryAspergillus infection is a known complication of asbestos exposure, BG has not been previously reported in this disorder. This patient’s case further documents thatAspergillus hyphae may be present in BG unassociated with allergic bronchopulmonary aspergillosis. A wide range of processes affecting airways, including asbestos exposure, may be associated with bronchocentric granulomatosis.     

Corticosteroid monotherapy in a case of bronchocentric granulomatosis with a two-year follow-up

Bronchocentric granulomatosis (BCG) is a rare disease. Because of the possibility of fungal infection, BCG has usually been treated with corticosteroids and antifungal agent. However, fungi are not detected in all BCG tissues. We report a case of proven BCG by open lung biopsy without fungi in a woman with corticosteroids monotherapy and two-year follow-up.KEYWORDS : Bronchocentric granulomatosis (BCG), fungal infection, open lung biopsy, corticosteroids, allergic diseaseBronchocentric granulomatosis (BCG), which was first described by Liebow (1) in 1973, is a rare disease characterized by inflammation that begins within and is centered around the walls of bronchioles and manifests as fever, cough, wheezing, dyspnea, and blood eosinophilia. It is considered to occur primarily in asthmatic patients and to be closely related to fumigatus infection (2-4).Because of the possibility of fungal infection, BCG is usually treated with corticosteroids and an antifungal agent (5). However, fungi are not detected in all BCG tissues. The question thus arises of whether antifungal agents should be used in addition to corticosteroids in these patients. We here report a case of biopsy-proven BCG without accompanying fungal infection in a woman who was treated with corticosteroids alone and followed-up for two years.     

Leflunomide for the treatment of rheumatoid arthritis and autoimmunity

Leflunomide, a new oral immunomodulatory agent, is effective for the treatment of rheumatoid arthritis. Its mechanism of action in suppressing inflammation is based in its inhibition of dihydroorotate dehydrogenase, an enzyme responsible for de novo synthesis of pyrimidine containing ribonucleotides. It is the first disease-modifying antirheumatic drug approved for treatment of rheumatoid arthritis with an indication for retardation of joint damage by radiography. Side effects are generally mild and include diarrhea, rashes, reversible alopecia, and elevation of hepatic transaminases. Despite the concern about hepatotoxicity, combination use with methotrexate in treating patients with rheumatoid arthritis has been shown to be safe. Other autoimmune diseases in which leflunomide has been used successfully include Felty syndrome, vasculitis, Sjogren syndrome, Wegener granulomatosis, and bullous pemphigoid.     

Pulmonary tuberculosis and bronchocentric granulomatosis

Bronchocentric granulomatosis (BG) is an uncommon inflammatory lesion of unknown etiology defined on morphologic grounds by the presence of necrotizing granulomata centered on bronchi and bronchioles. We report the typical pathologic features of BG in a patient with tuberculosis. Mycobacterial and other types of infection should be excluded by appropriate stains and cultures in all patients with BG on lung biopsy, especially those who are nonasthmatic.     

Wegener's granulomatosis in a patient with a rheumatoid arthritis.

A 38-year-old woman with rheumatoid arthritis who developed Wegener's granulomatosis is described. Wegener's granulomatosis appeared with saddle nose, perforation in her nasal septum, and granuloma in the nasal cavity. Laboratory evaluation showed a positive rheumatoid factor and circulating immune complex. Radiographic examination revealed ankylotic changes in both wrist and elbow joints. Bilateral anosmia and other disease manifestations completely responded to treatment with oral cyclophosphamide and prednisolone.     

Case of bronchocentric granulomatosis that became recognized in the course of allergic bronchopulmonary aspergillosis]

A 56-year-old man with allergic bronchopulmonary aspergillosis (ABPA) was admitted due to the appearance of nodular opacities in the right upper lung field on chest radiography, after discontinuing itraconazole and clarithromycin on the suspicion of possible hepatic adverse effects. Chest CT scans on admission revealed nodular opacities in the right S3 and lingula bronchus, and bilateral bronchiectasis with mucoid impactions. A specimen obtained by transbronchial lung biopsy showed complete replacement of bronchioles by necrotizing granulomatous inflammation, containing the diagnosis of bronchocentric granulomatosis. Treatment with corticosteroids and micafungin sodium resulted in marked resolution of nodular opacities and mucoid impacts. This case suggests that abrupt cessation of antifungal agents and macrolides may provoke acute exacerbation of ABPA and development of bronchocentric granulomatosis.     

Rheumatische Erkrankungen mit Erstmanifestation im mittleren Lebensalter

Rheumatic diseases are manifested in all ages. First manifestations of gout are frequent between the ages of 40-60 years. Furthermore, the incidence of rheumatoid arthritis increases as well as anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides. Recently new treatment options for gout were established but colchicine, which has been used to treat gout for a long time was also further optimized in its use. Remarkable advantages in the treatment of rheumatoid arthritis towards remission have been made by the introduction of biological agents 10 years ago. In granulomatosis with polyangiitis (formerly Wegener's disease) therapeutic optimization has led to a reduction in toxicity and rituximab has proven to be an effective treatment option.     

Pseudallescheria boydii or Aspergillus fumigatus in a lady with an unresolving lung infiltrate, and a literature review

Abstract:   A 53-year-old lady with blood-stained sputum and pleuritic pain had a lingular opacity on CXR which failed to resolve. A bronchial aspirate and transbronchial biopsy revealed features of bronchocentric granulomatosis with dichotomous branching hyphae suggestive of Aspergillus infection. However, subsequent fungal culture grew Pseudallescheria boydii . This case demonstrates the similarity of clinical and histological features caused by these two fungi. This appears to be the first reported case of pulmonary pseudallescheria with a bronchocentric granulomatous response.     

ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES IN INFLAMMATORY ARTHRITIS--POTENTIAL FOR MISDIAGNOSIS?

Antineutrophil cytoplasmic antibodies (ANCA) are well describedin Wegener's granulomatosis and some forms of vasculitis. Theyhave also been described in patients with arthritis, but thespecificity of these ANCA and their relationship to the presenceof vasculitis, antinuclear antibodies (ANA) and granulocyte-specificANA (GS-ANA), and to disease activity are uncertain. We studied101 patients with forms of inflammatory arthritis and detectedfour cytoplasmic ANCA, eight perinuclear ANCA and 16 atypicalANCA. There was no association between the presence of ANCAand ANA or rheumatoid factor. No anti-PR3 antibodies were foundand no strong anti-myeloperoxidase antibodies were detected.Four GS-ANA were detected and were distinct from ANCA. Therewas no association between rheumatoid arthritis disease activityor disability and ANCA status. ANCA did not predict vasculitisover a 3 yr follow-up. These ANCA appear to be epiphcnomcna.Their importance lies in their potential to mislead physicianstowards a misdiagnosis of vasculitis. KEY WORDS: ANCA, Wegener's granulomatosis, Vasculitis, RA, Myeloperoxidase, Proteinase 3     

Leflunomide in clinical practice

Leflunomide (LEF) is a prodrug that is rapidly converted to its active metabolite A77 1726, that inhibits the novo pyrimidine nucleotide biosynthesis, mediated especially by the dihydroorotate dehidrogenase (DHODH). DMARD properties were documented in rheumatoid arthritis with efficacy, safety and limiting of radiological progression demonstrated in multiple studies. LEF has been also used in other autoimmune diseases, like Psoriatic Arthritis, Wegener granulomatosis, Systemic Lupus Erythematosus, Sarcoidosis and others. This article reviews the place of LEF in clinical practice and outlines its potential applications beyond the officially recognized indication: rheumatoid arthritis (RA).     

Inflammatory conditions of the eye associated with rheumatic diseases

Ocular inflammation occurs in many patients with systemic rheumatic disease. The best examples are rheumatoid arthritis, juvenile rheumatoid arthritis, temporal arteritis, systemic lupus erythematosus, Wegener’s granulomatosis, polyarteritis nodosa, relapsing polychondritis, and Adamantiades-BehÇet’s disease. Ocular inflammation may precede the symptoms of the systemic disease and can be helpful in systemic diagnosis. After diagnosis, ocular inflammation can mark the severity of the systemic condition. Thus, prompt diagnosis and treatment of inflammatory conditions of the eye are warranted and may be sight- and life-saving.     

Bronchocentric mycosis occurring in transplant recipients

Although a variety of long-term, probably immunologically induced pulmonary changes have been described in recipients of both combined heart-lung and bone marrow transplantation, pulmonary infections continue to remain causes of significant morbidity and mortality as well. Herein we describe three patients (two heart-lung and one bone marrow transplant recipient) who had bronchocentric granulomatous mycosis, a tissue manifestation of fungal infection not previously described in the setting of a transplant host. Although one patient was being treated successfully with antifungal agents for his mucormycosis, two other patients eventually died of invasive aspergillosis. This emphasizes that although this process is histologically somewhat similar to bronchocentric granulomatosis, a high index of suspicion for infection needs to be maintained when this pathologic process is identified in a transplant host.     

Bronchocentric granulomatosis as a first clinical manifestation in an adult patient with p67phox deficiency

We report on a case of adult chronic granulomatous disease which first manifested as a pulmonary mass, and was histologically diagnosed as bronchocentric granulomatosis associated with aspergillosis in a patient with a deficiency of p67phox and a low oxidative response. Antifungal treatment was required for clinical resolution. Copyright Copyright 1999 S. Karger AG, Basel