[On the clinical aspects of diffuse interstitial pulmonary fibrosis].
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Humans↗
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Clinical significance of surfactant protein D as a serum marker for evaluating pulmonary fibrosis in patients with systemic sclerosis.
OBJECTIVE: To determine the clinical significance of surfactant protein D (SP-D), a useful marker for evaluating various lung diseases, in patients with systemic sclerosis (SSc) and to clarify any clinical significance between SP-D and KL-6, which is known to be correlated with pulmonary fibrosis (PF) in SSc patients. METHODS: We used a specific enzyme-linked immunosorbent assay to measure serum SP-D levels in 83 patients with SSc and 31 healthy control subjects. RESULTS: The serum levels of SP-D were significantly higher in patients with SSc than in healthy controls (mean +/- SD 81.9+/-59.2 versus 34.8+/-13.7 ng/ml). Serum SP-D levels in patients with diffuse cutaneous SSc were significantly higher than those in patients with limited cutaneous SSc (98.8+/-72.1 versus 66.8+/-40.0 ng/ml). Serum SP-D levels in patients with PF were significantly elevated compared with those in patients without PF (99.7+/-64.1 versus 65.3+/-49.4 ng/ml). Moreover, the incidences of decreased percentage diffusing capacity for carbon monoxide and decreased percentage vital capacity were also significantly greater in patients with elevated SP-D levels than in those with normal levels (67% versus 43% and 36% versus 17%, respectively). There was a significant positive correlation between serum levels of SP-D and KL-6. Serum SP-D and KL-6 levels showed almost the same sensitivities and specificities in the diagnosis of PF (68% versus 73% and 70% versus 74%, respectively). These two markers also predicted PF to almost the same degree (31% versus 33%, respectively). CONCLUSION: These results suggest that SP-D, as well as KL-6, may be a useful serum marker for evaluating PF in patients with SSc.
Antibodies, Antinuclear↗
Anti-th/to-positivity in a cohort of patients with idiopathic pulmonary fibrosis.
OBJECTIVE: To evaluate the presence and clinical relevance of anti-Th/To-positivity in patients with idiopathic pulmonary fibrosis (IPF). METHODS: Antinuclear antibody (ANA) testing was performed in 285 patients with a clinical diagnosis of IPF and surgical lung biopsy-proven usual interstitial pneumonia. Twenty-five subjects (8.8%) were found to have a positive ANA with a nucleolar-staining pattern and were followed for 10 years. Immunoprecipitation analysis indicated that 13 of the 25 subjects had autoantibodies against Th/To antigen. RESULTS: All subjects presented with worsening dyspnea. Pulmonary physiology and gas exchange did not differ between those with and those without a positive ANA, those with and without a nucleolar-staining ANA, and those with and without anti-Th/To antibody positivity. Retrospective review of the clinical record revealed that none of the 25 subjects with a nucleolar-staining ANA had the characteristic cutaneous features of systemic sclerosis (SSc). Four of the 13 Th/To-positive subjects had 3 of 5 criteria of limited cutaneous SSc (CREST variant), and 9 met proposed criteria for SSc sine scleroderma. None of the 12 Th/To-negative subjects had 3 or more criteria of limited cutaneous SSc (CREST variant), and only one met proposed criteria for SSc sine scleroderma. Of the 25 subjects with nucleolar-staining ANA, cumulative survival was similar between those who were Th/To-positive and those who were Th/To-negative (log-rank test, p = 0.73). Cumulative survival was similar between the 13 Th/To-positive subjects and all other 272 IPF subjects (log-rank test, p = 0.34). CONCLUSION: Our findings indicate that a nucleolar-staining ANA is a common finding in patients with IPF, and that antibodies against Th/To are responsible for the majority of these. Given the high specificity of Th/To-positivity for SSc, our data suggest that these subjects may have SSc sine scleroderma, and that their prognosis is no different from those with IPF.
Adult↗
[Progressive pulmonary fibrosis during combination chemotherapy with BCNU (author's transl)].
In two patients with acute leukaemia, the development of progressive interstitial pulmonary fibrosis was observed following chemotherapy with BCNU, Cytoxan and Ara-C. The x-ray changes were accompanied by restrictive changes of pulmonary function and, later on, by severe hypoxia. Serologic tests did not reveal infection with cytomegaly virus or mycoplasma pneumoniae. These findings, together with reports in the literature, suggest a toxic effect of BCNU on the lung. The combination with Cyclophosphamide may contribute to this toxic reaction.
Acute Disease↗
Idiopathic pulmonary fibrosis: a historical review.
Hamman and Rich are generally considered to have been the first to describe idiopathic pulmonary fibrosis (IPF) as a new clinical and pathological entity. However, several earlier reports in the German-language literature described autopsy findings consistent with IPF from a contemporary point of view. The author discusses these and later reports in a review of the history, diagnosis and treatment of the disease.
History, 18th Century↗
Pulmonary fibrosis after bone marrow transplantation responsive to treatment with prednisone and cyclosporine.
Interstitial pneumonitis and biopsy-proven pulmonary fibrosis developed in a 35-year-old man with acute myeloblastic leukemia 4 months after conditioning with total body irradiation, etoposide and cyclophosphamide and allogeneic marrow transplantation from an HLA-identical sister. The patient had no evidence of graft-versus-host disease. Under treatment with cyclosporine and prednisone the patient became asymptomatic and radiographic changes of the chest normalized. Following discontinuation of immunosuppressive treatment the patient again became dyspneic, and chest radiographs showed bilateral diffuse interstitial infiltrates. Concurrently there was a rise in serum transaminases. Treatment with prednisone again resulted in resolution of all symptoms and normalization of radiographic and hepatic function abnormalities. This case indicates that late onset interstitial pneumonitis may respond to immunosuppressive therapy. Conceivably, such pulmonary disease may represent the first or only manifestation of chronic graft-versus-host disease.
Adult↗
Pulmonary fibrosis: a complication of 1,3-bis(2-chloroethyl)-1-nitrosourea (BCNU) therapy.
A pediatric patient is reported who experienced fatal progressive pulmonary fibrosis as a complication of 1,3-bis(2-chloroethyl)-1-nitrosourea (BCNU) therapy. The patient received a cumulative dosage of 1.29 g (1.72 g/m2) over a two-year period as adjuvant therapy for a medulloblastoma. Two and one-half years after cessation of therapy, cough, tachypnea and fatigue were noted. Progressive pulmonary insufficiency developed. Pulmonary pathologic findings included interstitial fibrosis and alveolar dysplasia. Other cases of BCNU pulmonary toxicity are cited from the medical literature.
Brain Neoplasms↗
Idiopathic pulmonary fibrosis: spectrum of high-resolution CT findings.
OBJECTIVE: Characteristic high-resolution CT (HRCT) findings of idiopathic pulmonary fibrosis (IPF) include reticulation, architectural distortion, and honeycombing involving mainly the lung periphery and the lower lobes. In 50% of IPF patients, HRCT is nonspecific. This article illustrates the HRCT findings of IPF correlating with the pathology. CONCLUSION: The spectrum of HRCT manifestations varies from typical findings that allow confident diagnosis to atypical patterns mimicking other diseases, including predominance of ground-glass opacity, consolidation, nodules, and atypical distribution of lesions.
Diagnosis, Differential↗
Hepatitis C virus infection, inflammatory myopathy, and pulmonary fibrosis: are they related?
We describe the clinical findings, laboratory and ancillary studies, and histopathologic findings of 2 patients with hepatitis C virus infection who developed inflammatory myopathy and interstitial lung disease. A review of pertinent literature revealed several other patients with a similar cluster of clinical manifestations. The inflammatory myopathy in the cases reported here was atypical and distinct from that found in the anti-synthetase syndrome because it was characterized by mild or intermittent elevation of creatine phosphokinase, absence of anti-JO1 antibodies, and excellent response to oral corticosteroids. Thus, this report supports the hypothesis that hepatitis C virus infection may cause both inflammatory myopathy and pulmonary fibrosis. We suggest that evaluation for hepatitis C virus infection should be pursued in all patients with inflammatory myopathy and pulmonary fibrosis irrespective of anti-JO1 status. We further suggest that hepatitis C should be considered as another cause of myositis and interstitial lung disease in addition to the anti-synthetase syndrome.
Journal Article↗
An autopsy case of sarcoidosis followed up for 27 years, with special reference to pulmonary fibrosis.
An autopsy case of sarcoidosis followed up for 27 years in a 48-year-old woman with pulmonary fibrosis is presented. Chest roentgenography demonstrated an interstitial pneumonia-like pattern with gradual contraction of the upper lobes. Focal and extending fibrosis and hyalinization were observed in various organs including the lung, lymph nodes, heart and liver. Most of the fibrosis was thought to be derived from solitary or confluent granuloma, showing hyalinized nodular, stellate or band-like fibrosis. There was also another type of fibrosis, not derived from granuloma, manifested as fibrosing alveolitis in the lung, and diffuse fibrosis extending throughout the other organs. In the lymph nodes, chromogenic bodies (Hamazaki-Wesenberg bodies) were seen. The process of fibrosis and the significance of chromogenic bodies in cases of chronic sarcoidosis are discussed.
Female↗
[Anesthetic management of a patient with Sjögren's syndrome and pulmonary fibrosis].
We described an anesthetic management of a 63 year old man complicated with Sjögren's syndrome and pulmonary fibrosis. Sjögren's syndrome is characterized by pathological dryness of the mouth, cornea and other exocrine glands. Anesthesia was induced with thiopental and the trachea was intubated smoothly following intravenous succinylcholine. Anesthesia was maintained with enflurane in nitrous oxide and oxygen. Intraoperative muscle relaxation was adequately obtained by pancuronium. Before and during anesthesia, atropine and other drying agents were avoided. Physiological saline solution was instilled on the eyes every ten minutes against dryness. A heat and moisture exchanger (Humi-Vent) was used to maintain high moisture of the respiratory tract. Anesthesia was carried out successfully.
Anesthesia, Inhalation↗
[Pulmonary fibrosis. Classification, diagnosis, therapy].
A new classification of the idiopathic interstitial pneumonia has recently been proposed, defining idiopathic pulmonary fibrosis (IPF) more rigorously and in contrast to further subentities. The new classification is of prognostic and clinical relevance. Diagnosis requires a combined clinical, radiological and pathological effort. In patients with characteristic clinical and HR-CT findings, the diagnosis of IPF can be made with sufficient confidence without surgical lung biopsy. Recent evidence suggests that the primary pathogenetic event in IPF involves epithelial injury and abnormal wound heeling. This explains the ineffectiveness of the usual anti-inflammatory therapy in the majority of patients. Since no antifibrotic drugs are available at present, the recommended standard therapy is a combination of prednisone with azathioprine or cyclophosphamide. New antifibrotic molecules are already tested in clinical trials.
Anti-Inflammatory Agents↗
Complement activation products in idiopathic pulmonary fibrosis: relevance of fragment Ba to disease severity.
Immune complexes have been implicated in the pathogenesis of idiopathic pulmonary fibrosis (IPF), a chronic inflammatory disease of unknown etiology. Using newly developed techniques to measure complement fragments, we assessed whether complement activation occurs in IPF and its relationship to circulating immune complexes (CIC), disease severity, and prognosis. We measured the fragments C3d, C4d, and Ba of the common, classical, and alternative pathway, respectively, along with the components C3, C4, and Factor B and CIC, in 18 patients and 19 healthy subjects. The fragment to parent molecule ratios C3d/C3, C4d/C4, and Ba/Factor B were derived. Complement determinations were repeated in 14 of the patients on a second occasion separated by 8-33 months (median 17 months) from the first. All parameters were higher in patients than controls, and the difference between the two groups was significant for C3d, C4d/C4, C3, CIC (P less than 0.05) and for C4d and Ba (P less than 0.01). Fragment Ba was found to correlate with disease duration and indices of disease severity, i.e., positively with the dyspnoea score (Rs = 0.44, P less than 0.04), the physiological score (Rs = 0.50, P less than 0.02), the fibrosis score (Rs = 0.45, P less than 0.04), and a composite clinical, radiological, and physiological score (Rs = 0.48, P less than 0.03) and negatively with the forced vital capacity (Rs = -0.47, P less than 0.03). Ba was higher in those patients who deteriorated on follow-up analysis than those who improved or remained unchanged (P less than 0.04). These results show that conversion products of all complement pathways are increased in IPF and demonstrate classical pathway activation. They also show that the measurement of Ba might prove a useful laboratory test of disease severity in this condition.
Adult↗
Systemic sclerosis sine scleroderma presenting with vitiligo-like depigmentation and interstitial pulmonary fibrosis.
We report a case of systemic sclerosis sine scleroderma in which vitiligo-like depigmentation and early interstitial pulmonary fibrosis predominated. The pigmentary changes occurring in systemic sclerosis are reviewed and the distinctive features of the case discussed.
Adult↗
Experience with single-lung transplantation for pulmonary fibrosis. The Toronto Lung Transplant Group.
Repeated success has now been obtained with single-lung transplantation for end-stage pulmonary fibrosis. Potential candidates for this procedure are individuals who are less than 60 years of age and who have progressive pulmonary deterioration with limited life expectancy. Since November 1983, eleven such transplantations have been performed; nine patients achieved discharge from the hospital and eight patients have remained alive up to 44 months after surgery. All of these recipients have good oxygenation on room air and good exercise tolerance and are capable of performing routine activities, including a return to previous employment. From our experience, it appears likely that single-lung transplantation can achieve the same success rates as other major organ transplantations.
Adolescent↗
[Effects of vitamin E deficiency on bleomycin-induced pulmonary fibrosis in hamsters].
We examined the effect of free radicals on lung defense mechanism in bleomycin (BLM)-induced pulmonary fibrosis in hamsters. The concentration of vitamin E (VE) in the lung tissue increased significantly after intratracheal BLM administration. VE deficient hamsters showed increased lipid peroxide values in the lung tissue at a very early stage after BLM treatment. This was followed by decreased superoxide dismutase activities in the lung tissue. The results indicate that; 1) VE appears to be necessary for the prevention of lipid peroxidation in BLM-induced oxidant lung injury, 2) VE deficiency produces a large number of free radicals at the early stage after BLM treatment, and might induce protease-antiprotease imbalance within the lung, which probably causes an emphysematous change in the BLM-treated lung at a late stage after BLM administration.
Animals↗
[Energy metabolism, respiratory heat loss, and lung functions in patients with chronic obstructive pulmonary disease and pulmonary fibrosis].
Resting energy expenditure (REE) and respiratory heat loss (RHL) were measured in 17 patients with chronic obstructive pulmonary disease (COPD), 7 patients with pulmonary fibrosis (PF), and 8 normal healthy subjects, three hours after a light breakfast. Correlations between these values, and also predicted basal metabolic rate (PreBMR) and normalized lung functions were assessed. REE, REE/Pre BMR, RHL and RHL/REE in all patients increased significantly compared with those of control subjects. When all the studied subjects were combined, significant negative relationships were found between REE, REE/PreBMR, RHL and RHL/REE and percent predicted values of forced expiratory volume in one second (FEV1.0), between RHL and RHL/REE and percent predicted values of vital capacity (VC), and between REE/PreBMR, RHL and RHL/REE and percent predicted values of carbon monoxide diffusing capacity (DLCO). These results indicate that resting energy expenditure and respiratory heat loss increase in patients with COPD and PF in association with even slight to moderate deterioration of lung function. Therefore, elevated resting energy expenditure and respiratory heat loss need to be taken into account in determining caloric and water requirements of these patients.
Aged↗
The number of interleukin 1 receptors on lung fibroblasts in patients with idiopathic pulmonary fibrosis.
BACKGROUND: We investigated the expression of the IL-1 receptor on lung fibroblasts in patients with idiopathic pulmonary fibrosis (IPF) the histology of which was diagnosed as usual interstitial pneumonia. METHODS: 125I-labeled IL-1alpha bound to lung fibroblasts in a specific and saturable manner in lung fibroblasts obtained from both IPF patients and control subjects. RESULTS: Scatchard plot analysis revealed a single type of binding site in both groups. In total, 8,600 +/- 1,450 (mean +/-SD) sites/cell with a dissociation constant (Kd) of 1.83 +/- 0.28 (mean +/-SD) nM in patients with IPF and 7,920 +/- 1,470 sites/cell with a Kd of 2.43 +/- 0.49 nM in control subjects were counted. Furthermore, affinity cross-linking experiments revealed that the IL-1 receptor on lung fibroblasts in patients with IPF and control subjects had a molecular size of 80 kD. CONCLUSION: In conclusion, type I receptor was present on human lung fibroblasts, but there were no differences in number or affinity between IPF patients and controls.
Aged↗