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[Bronchoalveolar lavage in fibrosing alveolitis--a parameter for evaluating activity and prognosis?].

Bronchoalveolar lavages (BAL) were performed in 19 patients in whom fibrotising alveolitis had been detected and confirmed. The patients were then followed up and controlled clinically for more than 12 months. The courses of the diseases did not reveal any group-specific behaviour (taking lymphocytic and granulocytic lavages into consideration) and were not predictable on the basis of the lavage patterns. With a BAL lymphocytosis, 40% of the patients showed clinical improvement and in 30% the disease became stable whereas in another 30% there was a deterioration. If more than 10% BAL granulocytes were present, 50% of the patients showed clinical deterioration, whereas the course became stable in 37.5% and clinical improvements were recorded in 12.5% of the patients.

Adult↗

[Fibrosing alveolitis in a 7-year-old girl with autoimmune hemolytic anemia and autoimmune hepatitis].

A Coombs-positive haemolytic anaemia with proof of heat autoagglutinins and autoantibodies against cell nuclei was diagnosed in a girl, now 7 1/2 years of age, in the sixth month of her life as a sequel to jaundice. Shortly afterwards liver biopsy yielded a confirmed diagnosis of giant cell hepatitis. The signs of the disease receded under treatment with decortin and imurek. An attempt to discontinue the medication at 5 1/2 years of age resulted in a histologically confirmed recurrence of autoimmune hepatitis. When the patient was 5 years old, the x-ray revealed a mainly interstitial pneumonia; at 6 years of age, bronchiectases in the region of the left lower lobe were confirmed by bronchography. Resection of the lower lobe was performed after occurrence of atelectasis. Histopathological findings confirmed fibrotic alveolitis associated with giant cells. Based on the overall clinical course and the histological and immunological findings, autoimmune etiology of fibrotic alveolitis must be discussed. Progression of the disease could be avoided during the past 6 months by intensifying the immunosuppressive prednisolone therapy.

Anemia, Hemolytic, Autoimmune↗

[The effect of oral N-acetylcysteine on glutathione concentration in bronchoalveolar lavage of patients with fibrosing lung diseases].

Glutathione (GSH) is an important constituent in protecting the cellular elements within the lower respiratory tract against oxydants. We measured GSH in bronchoalveolar lavage fluid (BAL) in twelve patients with lung fibrosis and compared the data to eight healthy controls. GSH in BAL was 0.71 +/- 0.34 mumol/l in patients with lung fibrosis and 0.88 +/- 0.35 mumol/l in the controls (p greater than 0.05). After relating GSH on the volume of the epithelial lining fluid (ELF), determined by the urea method, GSH/ELF was 93 +/- 71 mumol/l in lung fibrosis and significantly different (p less than 0.005) from 387 +/- 240 mumol/l in the controls. In seven patients (four lung fibrosis, one desquamative pneumonitis, two asbestosis) GSH in BAL was determined before and after seven days of medication with 1800 mg N-acetylcysteine/day. We observed a significant increase of GSH in BAL from 0.93 +/- 0.46 to 1.56 +/- 0.92 mumol/l. Our observations confirm that in patients with lung fibrosis the protective ability against oxydants is diminished and that one parameter of the antioxydative capacity (GSH) can be increased in BAL by oral administration of N-acetylcysteine. Further studies are necessary to investigate the clinical and therapeutic implications of our findings.

Acetylcysteine↗

[An unusual case of chronic fibrosing endometritis and mucometra in a Watusi cow (Bos primigenius f. taurus) with uterine foreign bodies].

Genital organs, ureter, urinary bladder, and blood were collected from a 12 year old watusi cow which never exhibited estrous behaviour. Post-mortem findings and hormone assays, however, indicated the incidence of recent estrous cycles. The animal showed a chronic panmetritis, retained fetal membranes or fetal residues, an infected mucometra with cystic glandular hyperplasia, and a leiomyoma. Tubular genitalia, ureter, and urinary bladder exhibited subacute inflammation.

Animals↗

[Fibromyalgia (fibrositis) syndrome--a case report].

A 25-year-old woman was admitted in our hospital with back pain and both hip joint pain. Pain was abruptly occurred from the beginning of March 1990. Physical examination revealed wide spread pain (occipital area, both shoulder, lumber area, bilateral gluteal area, inguinal area, both Achilles-plantar area) and more than 12 tender points (occiput, trapezius, second rib, supraspinatus, gluteal, greater trochanter, hip joints, pubic bone). Laboratory examination showed no abnormal findings except ANF (1:160). Any examination including X-ray, bone scintigraphy, CT and MRI did not disclose spondylitis, sacroiliitis and enthesopathy. She was diagnosed as primary fibromyalgia/fibrositis syndrome. Treatment with maprotine hydrochloride (30 mg/day) and phenobarbital (120 mg/day) brought approximately 1/3 reduction of pain and tenderness. Psychoanalysis revealed that she had psychological conflicts against her parents and her colleagues at the work. EEG showed a borderline record with irregular basic pattern and 14 & 6 Hz positive burst at the sleep stage. Although the newly proposed criteria for the classification of fibromyalgia was proposed by ACR, fibromyalgia/fibrositis syndrome has been seldom discussed in the Japanese literature. As this syndrome is frequently associated with various rheumatic diseases, hypothyroidism and malignant diseases, we should pay much more attention to understand this syndrome.

Adult↗

[Biopathology of fibrosing alveolitis].

Fibrosis is a condition where the functional tissue of an organ has been replaced by mesenchymal cells and their extracellular matrix. The process is frequent and may be regarded as univocal and independent of aetiology. An initial tissue damage is responsible for an inflammatory reaction which modulates and controls tissue repair with accumulation of mesenchymal cells, an accumulation which results from their migration and replication under the influence of chemotactic and/or mitogenic mediators (cytokines). Fibrosis occurs as an accentuation of this physiological process and may be understood as a healing process that has succeeded beyond normal expectation. Studies of idiopathic pulmonary fibrosis have improved our understanding of the physiopathology of pulmonary fibrosis. Alveolitis sets in response to an initial injury of undetermined nature, and from then on lymphocytes, neutrophils and macrophages intervene in the pathological process: (1) lymphocytes contribute to the perpetuation of the process by local production of immune complexes and coactivation of other inflammatory cells; (2) neutrophils and, to a lesser extent, eosinophils worsen the initial tissue injury by producing proteases and oxydants; (3) finally, macrophages control the local accumulation of mesenchymal cells by producing chemotactic and growth factors and by modulating the secretion by these cells of extracellular matrix.

Cytokines↗

[Diagnosis of diffused interstitial fibrosing lung diseases].

Diagnosing chronic diffuse infiltrative lung diseases is often difficult and requires a strict diagnostic approach. The patient's history and in some cases the presence of extrathoracic signs make it possible to distinguish between primary and secondary lung disease (notably pneumopathy). Radiological imaging, functional exploration, bronchoalveolar lavage and histopathology must be performed in a rational order, taking into account their degree of invasiveness and the diagnostic criteria they may provide. When as a result of these investigations the disease has been classified in its aetiological category, its anatomical repercussions must evaluated by non-invasive explorations yielding a true "lesional diagnosis".

Humans↗

[Contribution of imaging in the diagnosis of interstitial fibrosing lung diseases].

Standard radiography of the chest shows pulmonary infiltration and is determinant for the diagnosis of chronic diffuse infiltrative lung diseases. Since lung biopsy is an invasive exploratory technique, using computed tomography (CT) in the diagnostic approach is perfectly justified as a direct prolongation of plain radiography. CT identifies patients who, in spite of normal chest X-ray films, do have pulmonary infiltration. It is superior to radiography in suggesting the correct aetiological diagnosis, as it provides signs that are more discriminant than the radiographic signs. It also gives a better approach to the localization of lesions, thereby helping in guiding lung biopsies. Finally, once the diagnosis has been made CT remains useful in evaluating the effects of treatment of pulmonary lesions and in detecting possible complications.

Humans↗

[Value of bronchoalveolar lavage in the diagnosis and management of diffuse interstitial fibrosing lung diseases].

Bronchoalveolar lavage (BAL) is an exploratory technique which is easy to perform and collect cellular constituents, notably inflammatory and immunocompetent cells, as well as biological elements that are present in the distal airways. In patient with chronic diffuse infiltrative lung disease, analysis of these parameters reveals various disturbances such as neutrophilia frequently associated with eosinophilia and, in some cases, lymphocytosis. These features are not specific, but they differentiate chronic diffuse infiltrative lung diseases from sarcoidosis and hypersensitivity lung diseases. Alveolar inflammation (alveolitis) correlates with the evolutive potential of the lesions and their ability to respond to treatment. The presence of lymphocytosis is usually associated with response to corticosteroid therapy, whereas the presence or persistence of neutrophilia and, even more, eosinophilia is characteristic of patients who do not respond to corticosteroids but are amenable to the cyclophosphamide-corticosteroid combination. BAL therefore increasingly appears as an irreplaceable tool to understand the physiopathology of chronic diffuse infiltrative lung diseases.

Bronchoalveolar Lavage Fluid↗