Pulmonary diffusion capacity in patients with systemic lupus erythematosus

OBJECTIVE: The aim of this study was to clarify the characteristics of pulmonary function tests (PFT), especially carbon monoxide diffusion capacity (DLCO), and their correlation with clinical features and immunological findings in patients with systemic lupus erythematosus (SLE). METHODOLOGY: Vital capacity (VC) and DLCO were analysed retrospectively in 110 sequential Japanese SLE patients with active disease between 1985 and 1999. In 38 patients, serial measurements of PFT were also assessed during high-dose corticosteroid therapy. RESULTS: DLCO was reduced in 52 patients (47%) and a restrictive impairment of PFT was observed in nine patients (8%). The prevalence of pulmonary fibrosis was 13%. Reduced DLCO was frequently observed, even in patients with neither pulmonary fibrosis nor a restrictive pattern. No correlation between immunological data and reduced DLCO was found, except for the presence of anti-RNP. Patients with Raynaud's phenomenon showed a higher prevalence of DLCO impairment than those without this phenomenon. Although immunological parameters improved significantly after the corticosteroid therapy, no significant change in the level of DLCO was observed. CONCLUSIONS: Impairment of DLCO was frequently observed in patients with SLE who had no clinical respiratory abnormalities. DLCO impairments were correlated with Raynaud's phenomenon clinically, and the presence of anti-RNP immunologically. No significant correlation was found between impairment of DLCO and disease activity of SLE.     

Effect of corticosteroid treatment on the recovery of pulmonary function in farmer's lung.

The effect of corticosteroid treatment on the course of farmer's lung (FL) was studied in 36 patients randomly allocated in a double-blind placebo-controlled study. All patients were in the acute stage of the disease and had had the first diagnosed attack of FL. Twenty patients were given prednisolone treatment for 8 wk. Sixteen patients received an 8-wk placebo treatment. One patient was withdrawn from the analysis when she terminated corticosteroid treatment because of side effects. After 1 month of treatment there was a significant difference (p = 0.03) in DLCO between the treatment groups. After a follow-up of 5 yr no statistically significant differences were found between the treatment groups in FVC, FEV1, or DLCO. FL recurred in six patients during the follow-up in the corticosteroid group and in one patient in the placebo group, but the difference was not statistically significant. In conclusion, in the corticosteroid group the improvement of pulmonary function was more rapid than in the placebo group, but no influence on the long-term result was found. The possibility that corticosteroid treatment may favor the occurrence of recurrent attacks of FL needs attention.     

Beitrag zur Diagnose,Prophylaxe und Therapie der Taubenzüchterlunge

Pigeon breeders have been informed about pigeon breeder's disease in meetings and by communications in Germany. As a result 42 breeders presented the typical symptoms of the disease. In 24 patients diagnosis could be evaluated by clinical examination, laboratory tests, pulmonary function tests, x-ray examination, and by inhalation of antigens. As there are more than 100,000 pigeon breeders in West Germany and a large number of people are exposed to the corresponding allergens, pigeon breeder's disease has probably a higher incidence of occurrence than has yet been established. Cases of advanced lung fibrosis with cor pulmonale had been detected, mediated by allergic alveolitis versus pigeon antigens. But in more patients, who consented to avoid further exposure, fibrosis of the lung could be prevented or progress of the disease could be stopped.     

Pulmonary and hematological alterations in idiopathic ulcerative colitis.

BACKGROUND: Patients with idiopathic ulcerative colitis (IUC) may suffer from one or more extraintestinal manifestations. We decided to prospectively study the prevalence of extraintestinal manifestations among patients with IUC, with special reference to pulmonary and hematological alterations. METHODS: Fifty-one consecutive patients with IUC attending the gastroenterology services of our tertiary-care referral center were evaluated prospectively. A detailed clinical evaluation of the musculoskeletal system, eye and skin, X-ray examination of the sacroiliac joints and chest, liver function tests, coagulation profile, hemogram, pulmonary function tests (PFT) and diffusion capacity for carbon monoxide (DLCO) were done in all patients. ERCP, liver biopsy and high-resolution computerized tomography (HRCT) of the chest were performed whenever indicated. RESULTS: Nearly half (24/51; 47%) the patients had one or more extraintestinal manifestations; these included pulmonary function abnormalities (14 patients; 27%), sacroiliitis (8; 16%), arthritis (5; 10%), ocular complications (4; 8%), and pyoderma gangrenosum and Budd-Chiari syndrome (one patient each). Of the 14 (27%) patients who had abnormal pulmonary function, isolated PFT abnormalities were seen in 8 (restrictive pattern in 7, obstructive pattern in 1), decreased DLCO in four, and both of the above in two. All patients with decreased DLCO (n=6) were asymptomatic and had normal chest X-ray and HRCT chest. Thirteen (25%) patients had thrombocytosis, 19 (37%) had increased fibrinogen level, and one patient had decreased antithrombin level. Disease activity had significant association with decreased DLCO (p=0.008), increased platelet count (p<0.0001), increased fibrinogen level (p=0.016), low antithrombin levels (p=0.046) and arthritis (p=0.002). CONCLUSION: Extraintestinal manifestations of IUC were seen in 47% of patients. Asymptomatic pulmonary interstitial involvement was seen in 12%, more often among patients with active disease.     

Variable response to long-term corticosteroid therapy in chronic beryllium disease

OBJECTIVES: Chronic beryllium disease (CBD) shares many of its characteristics with sarcoidosis and is often treated with corticosteroids. There is limited available literature regarding the effect of long-term corticosteroid therapy on the natural history of CBD. METHODS AND MATERIALS: We conducted an observational retrospective study of six patients with CBD who received prolonged corticosteroid treatment with a mean pulmonary function test follow-up period of 10.1 years. Five of the six patients were exposed to beryllium at the same workplace. The diagnosis in four of the six cases was confirmed by a positive beryllium lymphocyte proliferation test result on blood or BAL fluid. Periodic pulmonary function tests were analyzed in relation to removal from beryllium exposure and treatment with corticosteroids. MEASUREMENTS AND RESULTS: Two broad patterns of response were noted in these patients. The first pattern seen in two patients showed no improvement in FVC or diffusion capacity of the lung for carbon monoxide (Dlco) with corticosteroids. However, a significant improvement in these parameters was noted on cessation of beryllium exposure in one of the two patients. The second pattern showed an initial improvement in FVC and Dlco with corticosteroids, which was not sustained. An improvement was noted on stopping beryllium exposure. CONCLUSIONS: The response to long-term corticosteroids in CBD, quite like that in sarcoidosis, is variable. Significant lung function improvement may be seen following cessation of beryllium exposure.     

硬金属致巨细胞间质性肺炎一例并文献复习

目的 提高对硬金属粉尘致巨细胞间质性肺炎的临床、胸部影像学和病理表现的认识.方法 对1例经胸腔镜肺活检证实的巨细胞间质性肺炎患者的硬金属粉尘职业史、临床、胸部x线、CT、病理资料并结合有关文献进行回顾性分析.结果 患者女,30岁,钨棒磨削工,接触含钴和钨等硬金属粉尘3年,咳嗽和活动后喘息40 d.肺功能检查示混合性通气功能障碍,弥散功能明显下降(D<,L>CO占预计值%为39%),X线胸片和高分辨率CT示两肺弥漫性分布的磨玻璃影和边界不清的小结节影.肺活检病理表现为巨细胞间质性肺炎,肺泡腔内可见巨噬细胞和大量的多核巨细胞聚集,位于肺泡腔内的多核巨细胞内可见被吞噬的炎症细胞;细支气管及周围肺泡腔可见慢性炎症细胞浸润和肺间质纤维化.结合患者的职业史及特征性病理改变,诊断为硬金属致巨细胞间质性肺炎和硬金属肺疾病.患者脱离接触金属粉尘工作后,经糖皮质激素治疗临床症状缓解,胸部异常阴影明显吸收.结论 巨细胞间质性肺炎是硬金属肺疾病的特征性病理改变.在间质性肺疾病的诊断和鉴别诊断中需重视患者的职业暴露史.     

Isolated diffusing capacity reduction in systemic sclerosis.

OBJECTIVE. To determine the long-term outcome of patients with systemic sclerosis (SSc) and an isolated reduction in the diffusing capacity for carbon monoxide (DLCO) at the time of initial evaluation. METHODS. Patients with an isolated reduction in DLCO (i.e., normal forced vital capacity [FVC] and normal ratio of the forced expiratory volume in one second [FEV1] to the FVC) on initial evaluation were identified from among 815 patients with SSc who were carefully followed up throughout their illness. We requested that patients have repeat pulmonary function testing (PFT), and the outcomes of these tests, as well as cardiopulmonary and survival outcomes, were determined. RESULTS. An isolated reduction in DLCO, with a normal FVC was detected in 152 (19%) of the 815 patients. A subset of those with an isolated reduction in DLCO (11%) developed isolated pulmonary hypertension and had severely reduced survival rates. Pulmonary hypertension was strongly associated with an initial DLCO of less than 55% of predicted normal and a FVC (% predicted)/DLCO (% predicted) ratio of greater than 1.4. Among all patients in whom this ratio was greater than 1.4, 22% developed isolated pulmonary hypertension, compared with only 2% of those whose ratio was less than 1.4 (P less than 0.01). Of the 152 patients with isolated DLCO reduction, 73 (48%) underwent PFTs a mean of 5.4 years (range 2.0-13.2) after the initial PFT. Only 6 (8%) of these 73 patients ever had serious pulmonary disease: 5 had isolated pulmonary hypertension, and 1 had severe pulmonary fibrosis. Half of the patients with a low initial DLCO demonstrated a significant improvement (greater than 20%) at followup testing that could not be explained by the demographic, clinical, or laboratory findings at the first visit. CONCLUSION. Isolated reduction in DLCO is a frequent abnormality in SSc. Overall, it is associated with a good prognosis for survival and for pulmonary morbidity. A small subset of patients (11%) who have a very low DLCO (less than 55% of predicted) have developed isolated pulmonary hypertension, all of whom had limited scleroderma.     

INCREASE OF PULMONARY DIFFUSING CAPACITY IN SYSTEMIC SCLEROSIS

Pulmonary function tests and chest radiographs of 29 non-smokingsystemic sclerosis (SSc) patients were analysed, featuring anapparently paradoxic finding of an increased diffusing lungcapacity for carbon monoxide (DLCO). Twenty-one patients (72%)had abnormal pulmonary function, 11 of them had restrictivedisease (38%), six (21%) had isolated DLCO increase, four(14%)had isolated DLCO reduction, while two patients had obstructivedisease (7%). Chest X-ray revealed interstitial abnormalitiesconsistent with pulmonary fibrosis in all four patients withisolated DLCO reduction, in one obstructive patient and in sixrestrictive patients. Inpatients with DLCO increased steroidtreatment significantly reduced DLCO (P<0.05) and membraneDLCO component (Dm) (P<0.05). Hitherto unobserved findingof DLCO increase in SSc patients was associated with shorterduration of SSc (P<0.05), normal lungmechanics and roentgenogram(P<0.05) and absence of pulmonary symptoms (P<0.05). Thefindings that in some SSc patients DLCO increases suggest thatDLCO might prove to be an early and sensitive indicator of acutepulmonary involvement. KEY WORDS: Scleroderma, Pulmonary function tests, Pulmonary diffusing capacity, Memebrane diffusing capacity, Pulmonary capillary blood volume, Corticosteroids     

Pulmonary dysfunction due to combination of extra-pulmonary causes and alveolar damage is present from first the day of hospital admission in the early phase of acute pancreatitis

BackgroundOnly few studies have attempted to evaluate the pulmonary function in the early phase of acute pancreatitis (AP), although pulmonary dysfunction is the most frequent complication in the early phase of AP. We aimed to evaluate the changes in pulmonary function tests during the early phase of AP.MethodsProspective cohort study including 44 patients (52% men; median age 54 years) admitted with first attack of AP and 22 healthy controls. Patients underwent assessments on day 1, 2, 3, 6, and 10 as well as one month after discharge. Pulmonary function tests included the % predicted: forced expiratory volume during the first second (FEV1), forced vital capacity (FVC), total lung capacity (TLC), diffusion lung capacity (DLCO) and the ratio between DLCO and alveolar volume (DLCO/VA).ResultsIn total, 9% developed severe acute pancreatitis, 7% died, and 14% required treatment at the intensive or semi-intensive care unit. From admission, patients had impaired FEV1, FVC, DLCO, and TLC compared with controls (p < 0.0001 in all analyses). Patients with CRP >150 mg/L had significantly lower lung function tests. One month after discharge, lung function tests improved but patients had lower FEV1 (p = 0.014), FVC (p = 0.022), TLC (p = 0.020), and DLCO (p < 0.001) compared with controls.ConclusionThis study found that patients with AP had evidence of pulmonary impairment from the first day after hospital admission. The impairment lasted several weeks after hospital discharge.     

Complement consumption in the diagnosis of pigeon breeders' disease

Two laboratory techniques have been applied for the diagnosis and follow-up of pigeon breeders' disease, viz. detection of precipitins against specific antigens from pigeon droppings and assays of human haemolytic complement (huC) consumption by pigeon dropping antigens. A simple laboratory test of huC consumption by pigeon antigens is described, revealing high huC sensitivity in almost all sera of pigeon breeders with manifest or former disease. False-positive results were seldom observed. Discontinuation of antigen exposure and/or corticosteroid therapy had no effect on the huC consumption level in the test system, despite precipitin titres falling below the level of detection. Titration studies revealed an initial decrease of the huC-consuming serum factors, but titres settled at a definite constant level, even after antigen avoidance for many years. Combined application of both the precipitin test and the huC consumption test appears to be a good procedure for the laboratory diagnosis of pigeon breeders' disease.     

Pulmonary function of marrow transplant patients. I. Effects of marrow infusion, acute graft-versus-host disease, and interstitial pneumonitis

The pulmonary function of patients receiving marrow transplants was studied during a two-year period. The 81 patients studied before transplantation showed a slight reduction in average lung volumes and diffusing capacity (DLCO). Studies were repeated within 48 h after marrow infusion to look for evidence of fat embolism syndrome. There was no change in the DLCO, but there was a 4% decrease in the lung volumes. Sixty-three patients (20 with aplastic anemia, 43 with hematologic malignancies) completed studies on admission and every other week during hospitalization (mean of six studies per patient). When categorized by diagnosis or conditioning regimen (including with and without total body irradiation), no differences were seen. The patients developing interstitial pneumonitis (IP) had restrictive ventilatory changes and decreases in the DLCO. The patients not developing IP remained unchanged. The patients developing IP averaged a 20% decrease in the DLCO before the clinical diagnosis of pneumonia, but a decrease in the DLCO lacked specificity for predicting occurrence of IP. Among 18 patients developing graft-versus-host disease, there was no evidence of air-flow obstruction. We conclude that patients developing IP have restrictive ventilatory changes, but in the absence of complicating IP, the marrow transplant regimen (including marrow infusion and total body irradiation) leaves pulmonary function largely unchanged.     

Echocardiography and pulmonary function as screening tests for pulmonary arterial hypertension in systemic sclerosis

OBJECTIVE: A prospective study to evaluate echocardiography and gas transfer (DLCO) by comparison with cardiac catheterization in discriminating between patients with and without systemic sclerosis-associated pulmonary arterial hypertension (SScPAH). METHOD: A total of 137 (52 with and 85 without pulmonary fibrosis) had echocardiography and lung function tests within 3 months of their definitive invasive study. RESULTS: At cardiac catheterization 99 of these patients were found to have PAH, while PAH was excluded in 38. Echocardiographically estimated tricuspid gradient (TG) showed a moderate positive correlation (r(2) = 0.44, P<0.005) with both mean pulmonary pressure and invasively determined tricuspid gradient. DLCO showed a weak correlation (r(2 )= 0.09, P = 0.006), when compared with mean pulmonary arterial pressure. In total, 97% of patients with an echocardiographically determined TG of > 45 mmHg were found to have pulmonary hypertension at catheterization. However, no threshold could be defined with either screening test that safely excluded PAH. CONCLUSIONS: The positive predictive accuracy of currently used non-invasive tests are adequate for the diagnosis of advanced PAH provided sufficiently high thresholds (TG > 45 mmHg or DLCO < 55% predicted) are used. These tests cannot be relied upon to exclude pulmonary hypertension where pre-test probability is high.     

Alterations in pulmonary function in inflammatory bowel disease are frequent and persist during remission

OBJECTIVES: Information on the occurrence and frequency of pulmonary involvement in patients with inflammatory bowel disease (IBD) is inconsistent. The aim of this prospective study was to determine the frequency and type of pulmonary dysfunction in patients with IBD. METHODS: Sixty-six patients with IBD (35 with Crohn's disease [CD] and 31 with ulcerative colitis [UC]) and 30 control patients were investigated with respect to the following pulmonary function tests: forced expiratory volume in 1 s (FEV1), inspiratory vital capacity (IVC), Tiffeneau value (FEV1/IVC), and lung CO transfer capacity (D(LCO)). Disease activity in IBD patients was assessed by the CD activity index for CD and the Truelove index for UC, respectively. Smoking habits and medication were documented in every patient. RESULTS: Fourteen of 36 CD patients (39%) and 14 of 31 UC patients (45%) but only one of the controls exhibited at least one pathological (<80% of predicted value) pulmonary function test. In both CD and UC lung function tests were significantly decreased in comparison to the control group. This could be shown for FEV1 (-14% of predicted value in CD and -17% in UC, p < 0.01), IVC (-10% in CD and -12% in UC, p < 0.05), and DLCO (-20% in CD and -31% in UC, p < 0.01) without significant differences between both disease entities. The impairment of pulmonary function tests was more pronounced in patients with active disease than in those with inactive disease (FEV1, 81.4% vs 93.4% predicted, p < 0.02; IVC, 84.4% vs 93.7%, p < 0.05; DLCO, 80.4% vs 95.8%, ns). CONCLUSIONS: IBD patients show significantly decreased lung function tests in comparison to healthy controls. The impairment in active disease exceeded that during remission.     

Lung involvement in “stable” undifferentiated connective tissue diseases: a rheumatology perspective

Previous studies of the occurrence of interstitial lung disease (ILD) in undifferentiated connective tissue diseases (UCTD) were conducted in patients admitted to Respiratory Medicine Units. The aim of the present prospective study was to investigate lung involvement in UCTD patients admitted to a Rheumatology Unit. Eighty-one consecutive UCTD patients were enrolled in the study. Each patient underwent history and physical examination, routine laboratory investigations, antinuclear antibody (ANA) profiling, B-mode echocardiography, and lung function study according to previously reported methods. Lung high resolution computed tomography (HRCT) was performed in patients who provided informed consent. Six patients (7.4%) had a history of grade II dyspnea. Three of them had a DLCO ranging from 42 to 55% of the predicted value; and a HRCT-documented ILD with a non-specific interstitial pneumonia (NSIP) pattern. Symptoms in the other three patients were due to cardiac disease. None of the 75 asymptomatic patients, had relevant findings at physical examination, 26/75 had a DLCO <80% (<70% in 10 cases). Of these, 3 of the 30 patients who underwent lung HRCT were affected by NSIP-ILD. Six of the 81 enrolled were affected by ILD, which was symptomatic in three patients. A higher percentage of patients had a reduced DLCO. The latter finding may reflect a preradiographic ILD or a preechocardiographic pulmonary vascular disease.     

Pulmonary function tests

The clinical significance of pulmonary function tests (including blood gas analysis) lies in their sensitivity for detecting PCP. PCP has most consistently been found to cause abnormalities in the DLCO and the exercise arterial blood gas; both are highly sensitive for the presence of Pneumocystis infection. These tests are more sensitive for the detection of PCP than are the resting arterial blood gas and chest x-ray. Therefore, measuring these values can be especially helpful in evaluating HIV-infected individuals who have pulmonary symptoms but whose resting arterial blood gas and/or chest radiograph are normal. The advantage of performing the exercise test over measuring the DLCO is that the exercise test is simple. It can be done without pulmonary function equipment and without a technologist. Furthermore, since many AIDS patients with non-PCP pulmonary disorders maintain "normal" exercise tests despite abnormal DLCO, it can be useful in evaluating patients for PCP who have known underlying lung disease with progressive symptoms. Measurement of lung volumes and spirometry lacks both sensitivity and specificity for detecting pulmonary disease in general and PCP in particular. Spirometry is helpful in detecting airways obstruction, which is not an uncommon finding in AIDS patients. The etiology, clinical significance, and treatment of obstructive ventilatory defects in the AIDS population remains unclear.     

Lung function in allogeneic bone marrow transplantation recipients

In order to investigate the incidence of pulmonary function complications following bone marrow transplantation (BMT), 17 patients with leukaemia and 8 with aplastic anaemia were sequentially assessed over a one year period. Before BMT, all the patients were free of respiratory symptoms and had both normal chest X-ray and routine lung function tests. However, 5 patients disclosed airway hyperreactivity. Aplastic anaemia patients had significantly lower haemoglobin-adjusted diffusing capacity for carbon monoxide (DLCO) than those with leukaemia, a finding significantly related to the lower haemoglobin values shown in the former individuals. Following BMT there were transient mild to moderate reductions in DLCO and static lung volumes; moreover, patients with leukaemia had lower DLCO than those with aplastic anaemia. Fourteen of the 25 patients had ventilatory defects, including 10 individuals with bronchial hyperresponsiveness. Post-BMT lung function changes were transiently accompanied by mild to moderate symptoms of respiratory disease in most of the patients.     

Early treatment of stage II sarcoidosis improves 5-year pulmonary function.

STUDY OBJECTIVE: To evaluate the 5-year prognosis of patients with stage I and stage II newly detected (< 3 months) pulmonary sarcoidosis treated immediately after diagnosis with prednisolone for 3 months followed by inhaled budesonide for 15 months. DESIGN: Randomized, double-blind, placebo-controlled, parallel-group study for 18 months. Thereafter, open follow-up without treatment. SETTING: Twenty pulmonary medicine departments in Finland. PATIENTS: One hundred eighty-nine adult patients, most of them with normal lung function, were randomized to treatment. One hundred forty-nine patients were followed up for 5 years: 79 patients with initial stage I disease and 70 patients with stage II disease. TREATMENT: Oral prednisolone for 3 months followed by inhaled budesonide for 15 months (800 microg bid), or placebo tablets followed by placebo inhaler therapy. Thereafter, treatment only on an individual basis in the case of clinical deterioration. MEASUREMENTS: Yearly follow-up visits with chest radiographs, lung function tests (FEV(1), FVC), diffusion capacity of the lung for carbon monoxide (DLCO), serum angiotensin-converting enzyme (SACE), and serum and urinary calcium measurements. RESULTS: No initial differences were observed in chest radiographic findings between the active-treatment and placebo-treatment groups, either in patients with initial stage I or stage II(-III) disease. However, after the 5-year follow-up, 18 steroid-treated patients (26%) and 30 placebo-treated patients (38%) still had remaining chest radiographic changes. Placebo-treated patients more frequently required treatment with corticosteroids during the 5-year follow-up (p < 0.05). Steroid-treated patients with initial stage II(-III) disease improved more in FVC and DLCO (p < 0.05). No differences in reported adverse events or in SACE, serum calcium, or urinary calcium values were seen. CONCLUSION: Immediate treatment of pulmonary stage II(-III) sarcoidosis-but not stage I disease-improved the 5-year prognosis with regard to lung function variables.     

Pulmonary function of nonsmoking patients with rheumatoid arthritis in the presence and absence of secondary Sj?gren's syndrome, a controlled study

One hundred nonsmoking patients with rheumatoid arthritis, including 63 with rheumatoid arthritis alone (RA group) and 37 who also had secondary Sj?gren's syndrome (sSS group), underwent a detailed evaluation of their pulmonary function. The results were compared with those of 110 age-matched nonsmoking controls. Normal function was significantly less common in both patient groups than in the controls. A significant percentage of patients had small airways disease (SAD) which was observed with similar frequency in the control group. If this were excluded, then isolated impairment of carbon monoxide diffusing lung capacity (DLCO) was the most commonly detected significant abnormality in both patient groups. Restrictive disease was following in frequency in the RA group, but was absent in the sSS group. Obstructive disease was very uncommon in all of the patients. In general, there were no significant differences in the frequency of the various respiratory function abnormalities between patients with RA only and those with concomitant sSS, whereas between patients and controls, the only statistically significant differences were the higher frequencies of isolated DLCO impairment and restrictive disease in the RA group.     

Pulmonary involvement in juvenile dermatomyositis: a two-year longitudinal study

OBJECTIVE: To investigate the prevalence and features of asymptomatic pulmonary involvement in juvenile dermatomyositis (JDM). METHODS: Twelve JDM patients underwent pulmonary function tests at baseline, 12 and 24 months. Disease activity, duration, serum lactate dehydrogenase (LDH) values and antinuclear antibody (ANA) titres were also evaluated. RESULTS: Five patients showed lung impairment at baseline and four at 12 and 24 months. Forced expiratory volume in 1s, forced vital capacity (FVC), carbon monoxide diffusing capacity (DLCO) and alveolar volume were the most frequently altered variables, indicating a restrictive pattern and impairment of diffusion. The prevalence and features of pulmonary alterations did not change during follow-up. FVC values were significantly lower in active JDM patients and were inversely related to LDH. DLCO values were significantly lower in ANA-positive patients. About half of the patients of this small case series of JDM had asymptomatic lung disease. CONCLUSIONS: We suggest that lung function should be evaluated at disease onset and regularly during follow-up, as pulmonary function tests can detect otherwise unpredictable pulmonary involvement.     

Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement

OBJECTIVE: To determine whether there are factors, such as the diffusing capacity for carbon monoxide (DLCO) or pulmonary artery pressure (PAP) on echocardiogram, that can predict the development of pulmonary hypertension (PHT) in patients with limited scleroderma. METHODS: Using the large Pittsburgh Scleroderma Databank, 106 patients who had the diagnosis of PHT after January 1, 1982, were matched with 106 controls by scleroderma subtype, age, sex, race, disease duration, and the mean time to the diagnosis of PHT after the initial Pittsburgh visit. Autoantibodies, vascular features, use of calcium channel blockers, extent of pulmonary function, and echocardiogram findings were determined at any time prior to the diagnosis of PHT (or prior to the matched time in controls). RESULTS: Patients with PHT had a mean DLCO of 52% of predicted at an average of 4.5 years prior to the diagnosis of PHT. This was markedly decreased compared with the values in controls, whose mean DLCO was 81% of predicted (P < 0.0001). The estimated mean PAP on echocardiogram was only slightly higher in the PHT patients compared with controls (34 mm Hg versus 29 mm Hg; P not significant). Nineteen PHT patients had 4 serial measurements of the DLCO during the 15 years prior to the diagnosis of PHT. The initial mean DLCO was 80% of predicted, which decreased in a linear manner to a mean of 35% of predicted at the time of diagnosis of PHT, whereas the value in controls remained at approximately 80% of predicted (P < 0.0001). PHT patients had more severe Raynaud's phenomenon and more severe digital tip ulcers, but they used calcium channel blockers significantly less frequently (37% versus 61% of controls; P < 0.01). The predominance of nucleolar autoantibodies and the absence of anti-Scl 70 antibody were associated with PHT. CONCLUSION: A decreasing DLCO is an excellent predictor of the subsequent development of isolated PHT in limited scleroderma. The DLCO may be significantly decreased for many years prior to the diagnosis of PHT. The presence of autoantibodies and the PAP may also be helpful predictors. The long-term use of calcium channel blockers may be protective, but newer agents that are more effective in treating PHT may also be helpful in altering the natural history of this serious complication in limited scleroderma.