A retrospective study of prognostic factors in patients with interstitial pneumonia receiving long-term oxygen therapy.

PURPOSE: We retrospectively analyzed patients with clinically diagnosed interstitial pneumonia to investigate the factors which contribute to the difference in prognosis from the initiation of long-term oxygen therapy (LTOT) among subtypes. METHODS: Seventy-six patients with clinically diagnosed idiopathic interstitial pneumonia (IIP; n = 49) or interstitial pneumonia associated with collagen vascular disease (CVD-IP; n = 27) in whom LTOT was initiated in our facility from January 1999 to December 2012 were analyzed. RESULTS: Patients with CVD-IP had significantly longer survival time from the initiation of LTOT than those with IIP with the median survival of 51.7 months versus 18.8 months, respectively. The 1-year survival rate was 92.4% for patients with CVD-IP versus 76.5% for those with IIP, and 2-year survival was 88.6 versus 36.0%, respectively. The patterns classified with high-resolution computed tomography (HRCT) were not associated with prognosis. The association between pulmonary hypertension and prognosis was unclear. In results of the multivariate Cox analysis which included factors demonstrating p < 0.1 in the univariate Cox analysis, male gender, low body mass index, and the absence of collagen vascular disease (CVD) were significantly associated with poor prognosis. CONCLUSIONS: After the initiation of LTOT, patients with IIP had poor prognosis regardless of the patterns classified with HRCT, while those with CVD-IP survived longer. Male gender, low body mass index, and the absence of CVD were the independent negative prognostic factors in patients with interstitial pneumonia receiving LTOT.

Clinical features and outcome of acute exacerbation of interstitial pneumonia: collagen vascular diseases-related versus idiopathic.

BACKGROUND: Relatively little is known about acute exacerbation (AE) of interstitial pneumonia associated with collagen vascular diseases (CVD-IPs). OBJECTIVES: This study was aimed at clarifying clinical characteristics and outcome in AE of CVD-IPs, compared with those of idiopathic interstitial pneumonias (IIPs). METHODS: We retrospectively reviewed 112 admission cases with suspected AE of CVD-IPs or IIPs during 2003-2009. IIPs were diagnosed with idiopathic pulmonary fibrosis (IPF) or non-IPF, mostly based on radiologic findings. Of these, 15 AEs of CVD-IPs (6 rheumatoid arthritis, 6 dermatomyositis and 3 systemic sclerosis) and 47 AEs of IIPs (13 IPF and 34 non-IPF) were included. RESULTS: The clinical characteristics in AE of CVD-IPs were similar to those of IIPs, except for younger age (63.3 ± 6.8 vs. 73.8 ± 9.1 years; p = 0.0001) and higher PaO(2)/FiO(2) at the onset of AE (205 ± 81.2 vs. 145 ± 53.8 mm Hg; p = 0.002) in the former. Dermatomyositis-related interstitial pneumonia (IP) showed a relatively indolent onset and was often associated with worsening control of the underlying disease, whereas AE of other CVD-IPs resembled that of IIPs. 90-day mortality of 33% in AE of CVD-IPs was similar to that of IIPs (44%; p = 0.44) or non-IPF (34%; p = 0.94), but was significantly better than that of IPF (69%; p = 0.04). CONCLUSION: Clinical features and outcome in AE of CVD-IPs were similar, if not identical, to those of IIPs, having a significant impact on the clinical course. AE of advanced IPF with typical radiologic features seems to have higher mortality compared with other forms of IP.

[Epidemiology of collagen diseases].

Collagen diseases have been generally thought to be rare, but the number of patients with rheumatoid arthritis(RA) becomes over 700,000 in recent days. In addition, the survival rate has markedly increased, but many lupus patients are still suffering from so called intractable organ involvements such as severe lupus nephritis and CNS lupus, etc. The main purposes of the treatment for RA are to prevent bone/cartilage destruction, to improve the quality of life(QOL) of patients, and to improve prognosis. For these purposes, new type of drugs such as biologic agents are introduced, and showing remarkable effects comparing with conventional DMARDs. But the side effects and cost of these agents are becoming problems. To overcome these problems, the epidemiology that can clearly show the benefits of these agents on daily life of patients is becoming much more important issue.

[Diagnosis, therapy and prognosis in systemic autoimmune disorders like lupus erythematosus]. / Diagnose, Therapie und Prognose der Kollagenosen am Beispiel des Lupus erythematodes.

Systemic lupus erythematosus (SLE) is the prototype of a systemic autoimmune disorder, in which immune complexes or cytotoxic antibodies give rise to tissue damage and organ failure, which often results in death. Due to more sensitive diagnostic tools and more sufficient therapeutic methods, the five-year survival rate in patients with systemic lupus erythematosus has improved dramatically during the past decades from less than 50% to 95%. Mortality is still 4 to 5 times higher and is mostly caused by uncontrolled disease flares, infections, and thromboses. Risk factors influencing the course of SLE and favouring the higher mortality are serum antibodies (anti-dsDNA, anticardiolipin, lupus anticoagulant), infections, hypertension, osteoporosis with fractures, cytopenia, renal involvement, higher age at onset, and genetic factors. Morbidity and social consequences are conducted by individual multisystemic disease manifestations.

Pathologic and radiologic differences between idiopathic and collagen vascular disease-related usual interstitial pneumonia.

BACKGROUND: Patients with usual interstitial pneumonia (UIP) associated with collagen vascular disease (CVD) have been reported to have a better prognosis than those with idiopathic pulmonary fibrosis with a UIP pattern (IPF/UIP) seen on histology. The aim of this study was to evaluate the pathologic and radiologic differences between the two conditions and their relationship with clinical outcome. METHODS: A retrospective review of 100 patients (CVD-UIP, 39 patients; IPF/UIP, 61 patients) with UIP pattern diagnosed by surgical lung biopsy at one tertiary referral center. RESULTS: The median follow-up period was 34.4 months. The CVD-UIP group was younger, included more women and nonsmokers, and showed better survival than the IPF/UIP group. Pathologically, CVD-UIP patients had fewer fibroblastic foci and smaller honeycombing (HC) spaces with higher germinal centers and total inflammation scores than IPF/UIP patients. Radiologically, CVD-UIP patients had a lower emphysema score and more likely a nontypical UIP pattern without HC. The germinal centers score was the best distinguishing feature between CVD-UIP and IPF/UIP patients (odds ratio, 2.948; p = 0.001) and was marginally related to survival (p = 0.076). The HC score (hazard ratio [HR], 1.134; p < 0.001), total lung capacity (TLC) [HR, 0.932; p = 0.004], and age (HR, 1.052; p = 0.017) were significant predictors of survival in all patients with UIP histology, regardless of the presence of CVD. Among IPF/UIP patients, those with positive autoantibodies were pathologically more similar to CVD-UIP than to IPF/UIP without autoantibodies, despite no difference in survival between them. CONCLUSIONS: The germinal centers score was the best discriminative between CVD-UIP and IPF/UIP patients; it was of marginal prognostic significance. Age, TLC, and HC score were independent prognostic factors in all patients with UIP histology.

[Importance of interstitial lung disease in collagen vascular disease: analysis of outcome].

We studied length of survival and related clinical findings in 715 inpatients with collagen-vascular diseases (1984 through 1994), the diagnostic Kaplan-Meier analysis showed that patients with polymyositis/dermatomyositis and those with systemic sclerosis did not survive as long as those with other types of collagen-vascular disease. Of the patients who died 37% died of respiratory failure due to interstitial lung disease. Patients with interstitial lung disease had better outcomes than did those with idiopathic interstitial pneumonia: they were younger, had higher initial vital capacities, and fewer episodes of acute exacerbation of lung disease than did those with idiopathic interstitial pneumonia. Among patients with interstitial lung disease, those who died of polymyositis/dermatomyositis did so within 1 year, but those who died of systemic sclerosis lived longer. Interstitial lung disease is an important prognostic factor in collagen-vascular disease, and needs further evaluation.

Fibroblastic foci in usual interstitial pneumonia: idiopathic versus collagen vascular disease.

A histologic feature of usual interstitial pneumonia is the presence of fibroblastic foci. As some patients with usual interstitial pneumonia and an underlying collagen vascular disease have a better prognosis, we hypothesized that they would have fewer fibroblastic foci. Pathologists reviewed surgical lung biopsies from 108 patients with usual interstitial pneumonia (nine with collagen vascular disease) and assigned a score (absent 0, mild 1, moderate 2, and marked 3) for fibroblastic foci. Patients with idiopathic usual interstitial pneumonia had a higher median profusion of fibroblastic foci (1.75 vs. 1.0, p = 0.003). Baseline characteristics were similar, although patients with a collagen vascular disease were younger, had a shorter duration of symptoms, and had a higher percentage of predicted total lung capacity. Profusion of fibroblastic foci was the most discriminative feature for separating idiopathic from collagen vascular disease-associated usual interstitial pneumonia (odds ratio 8.31; 95% confidence interval, 1.98, 59.42; p = 0.002 for a one-unit increase in fibroblastic foci score). No deaths were noted in the collagen vascular disease-associated usual interstitial pneumonia group; 52 deaths occurred in the idiopathic usual interstitial pneumonia group (log rank; p = 0.005). We conclude that patients with collagen vascular disease-associated usual interstitial pneumonia have fewer fibroblastic foci and improved survival.