Pulmonary involvement in early systemic lupus erythematosus

Aim of the workSLE is an autoimmune disease characterized by a variety of clinical and laboratory abnormalities. It may affect many organs and pulmonary involvement is a common finding in SLE. The purpose of this study was to disclose the pulmonary involvement in early SLE patients not more than 2 years of disease duration using the computed tomography (CT) as well as the pulmonary function tests as ways of pulmonary involvement assessment.Patients and methodsForty-two patients aged 29 ± 12.5 with early SLE not more than 2 years of disease duration were recruited for the study. All patients were assessed clinically for their SLE with BILAG which was utilized for disease activity determination.ResultsNine and half percent of our patients were found to be clinically involved by ILD, where 28.6% have abnormal HRCT finding, 26.2% with abnormal PFT. Variants that were associated with an abnormal forced vital capacity FVC < 80% in a significant manner were: smoking, long disease duration, self-reported pulmonary symptoms (p 0.001), BILAG global score (p 0.006), Anti dsDNA (p 0.001), Antiphospholipid (IgM or IgG) (p 0.01), anti Sm (p 0.002), anti-RNP (p 0.005), HRCT abnormalities (p 0.001), current medication of steroid (any dose) (p 0.005), immunomodulator therapy (p 0.002), and Rituximab therapy (p 0.001).ConclusionsILD occurs as early as in the first 2 years in the course of SLE patients. There was a clear predilection of ILD with certain variables in our cohort of patients.     

Pulmonary involvement in patients with childhood-onset systemic lupus erythematosus

OBJECTIVE: Pulmonary involvement is a common finding in adults with systemic lupus erythematosus (SLE). The aim of this study was to investigate the frequency of pulmonary abnormalities in patients with childhood-onset SLE, with particular reference to interstitial lung disease (ILD), and to examine any association between pulmonary abnormalities and other disease-related variables. METHODS: A cohort of 60 Norwegian patients with childhood-onset SLE was examined in a cross-sectional study by high-resolution computed chest tomography (HRCT) and pulmonary function tests (PFT). Median disease duration was 11.2 years. Disease activity, cumulative organ damage and immunological markers were also assessed. RESULTS: Five patients (8%) had abnormal HRCT findings, including micronodules in four patients and bronchiectasis in one. None of the patients had radiographic evidence of ILD. PFT results were impaired in 37% of the patients, the most frequent pulmonary dysfunction was reduced carbon monoxide diffusing capacity (26%). HRCT findings, disease activity or serology did not correlate with PFTs. Reduced diffusion capacity was associated with smoking (p-value < 0.05). CONCLUSION: Lung function was moderately impaired, while the frequency of pulmonary parenchymal involvement was low. There was no radiographic evidence of ILD, which is an unexpected finding given the high frequencies reported in adult SLE patients assessed with HRCT. The results suggests that PFT values are often abnormal, but these are infrequently associated with development of ILD or other substantial parenchymal alterations in childhood-onset SLE, and do not require further HRCT investigation in asymptomatic patients.     

Laryngeal involvement in systemic lupus erythematosus.

Laryngeal involvement in systemic lupus erythematosus (SLE) can range from mild ulcerations, vocal cord paralysis, and edema to necrotizing vasculitis with airway obstruction. In this report, four cases showing the range of severity of this disease manifestation are presented, accompanied by a comprehensive review of the literature. The clinical course of 97 patients with laryngeal involvement with SLE are reviewed, of whom 28% had laryngeal edema and 11% had vocal cord paralysis. In the majority of cases, symptoms such as hoarseness, dyspnea, and vocal cord paralysis resolved with corticosteroid therapy. Other, less common causes of this entity included subglottic stenosis, rheumatoid nodules, inflammatory mass lesions, necrotizing vasculitis, and epiglottitis. The clinical presentation of laryngeal involvement in patients with SLE follows a highly variable course, ranging from an asymptomatic state to severe, life-threatening upper airway compromise. With its unpredictable course and multiple causations, this complication remains a diagnostic and therapeutic challenge to physicians involved in the care of patients with SLE.     

Pulmonary hemorrhage in systemic lupus erythematosus.

The clinicopathological features of four patients with systemic lupus erythematosus and pulmonary hemorrhage are described. Our study confirms that pulmonary hemorrhage may be a dominant clinical expression of lung involvement in this disease. Its clinical manifestations are usually quite characteristic. However, hemoptysis may be absent. Radiographically, bilateral alveolar infiltrates resembling pulmonary edema or infection may be seen. Pulmonary hemorrhage was a major contributing factor to the death of three of our patients. The possible pathogenetic mechanisms responsible for pulmonary hemorrhage in our patients and other patients previously recorded in the literature are reviewed. Evidence supporting an immune complex pathogenesis is presented. Our immunopathological and ultrastructural studies demonstrate deposition of immune aggregates in the lungs in the alveolar septa, large blood vessels, and bronchioles in a manner similar to that which has been observed in the experimental serum sickness model of immune complex mediated pulmonary injury. The histological abnormalities, although nonspecific, are consistent with this interpretation, and collectively show diffuse alveolar lining cell and endothelial cell injury. However, an immune complex pathogenesis may not completely explain the occurrence of pulmonary hemorrhage in SLE. Other factors, including bleeding disorders, pulmonary infection, oxygen toxicity, and the "shock lung" syndrome, may also have contributed to lung hemorrhage in some of these patients.     

Nervous system involvement in systemic lupus erythematosus.

In a retrospective analysis of 80 patients with systemic lupus erythematosus (SLE) seen over a 10-year period, 41 (51%) exhibited neurological manifestations. Nervous system involvement was characterized by a significantly greater involvement of black patients (P less than 0-02), a higher incidence of renal failure after the first appearance of neurological features (P less than 0-05), and a significantly worse 10-year survival rate (P less than 0-05). Specific neurological investigations were generally unhelpful in diagnosis. The finding of hypoglycorrhachia in 4 patients was striking and may have pathogenetic significance. The lack of a specific diagnostic test for central nervous system involvement may have hindered recognition of a cerebral abscess in one patient. Treatment with massive doses of corticosteroids was not obviously more effective than treatment with smaller doses. Autopsy findings showed scattered small cerebral infarcts but vasculitis was apparent in only one case.     

Musculoskeletal involvement in systemic lupus erythematosus

Involvement of the musculoskeletal system is common if not universal in the clinical course of systemic lupus erythematosus (SLE). Joint involvement on the whole does not cause major erosive disease, however, recent developments in musculoskeletal imaging show clearly the presence of significant bony and soft tissue involvement. It might well explain the frequently observed discordance between the clinical signs and the articular symptoms assuming that fibromyalgia has been excluded. The clear demonstration of tendon involvement in SLE by MRI would merit considering tendonitis and tenosynovitis as candidates for inclusion in the diagnostic criteria.     

Cardiac involvement in systemic lupus erythematosus

Pericarditis is the most common cardiac abnormality in systemic lupus erythematosus (SLE) patients, but lesions of the valves, myocardium and coronary vessels may all occur. In the past, cardiac manifestations were severe and life threatening, often leading to death. Therefore, they were frequently found in post-mortem examinations. Nowadays cardiac manifestations are often mild and asymptomatic. However, they can be frequently recognized by echocardiography and other noninvasive tests. Echocardiography is a sensitive and specific technique in detecting cardiac abnormalities, particularly mild pericarditis, valvular lesions and myocardial dysfunction. Therefore, echocardiography should be performed periodically in SLE patients. Vascular occlusion, including coronary arteries, may develop due to vasculitis, premature atherosclerosis or antiphospholipid antibodies associated with SLE. Premature atherosclerosis is the most frequent cause of coronary artery disease (CAD) in SLE patients. Efforts should be made to control traditional risk factors as well as all other factors which could contribute to atherosclerotic plaque development.     

Cardiovascular involvement in systemic lupus erythematosus

SLE is an inflammatory disease of unknown etiology with the potential of affecting virtually all organ systems. Cardiovascular involvement occurs frequently, although it is often mild enough not to cause clinical concern. Pericarditis is most commonly subclinical, noted only on echocardiogram. Pericardial fluid, which can accumulate rapidly enough to cause tamponade, is inflammatory in nature and can totally mimic infection. The occurrence of Libman-Sacks endocarditis, usually a pathological diagnosis of little clinical significance, has little if any correlation with the presence of audible murmurs. However, valve replacement is occasionally necessary secondary to sterile destruction. These valvular lesions can also embolize or become infected. The incidence of ischemic coronary disease is increased, both secondary to premature atherosclerosis and, rarely, coronary arteritis. Conduction disease and arrhythmias are infrequently reported in adult patients, but congenital CHB has been noted in children born to mothers who have circulating anti-Ro antibody. Evidence is accumulating that suggests there is a mild cardiomyopathy associated with SLE that may be due to thrombotic or inflammatory microvascular coronary disease. Acute clinical myocarditis also rarely occurs. Therapeutically, at present, a reasonable course would seem to be to limit all known possible contributing factors to premature coronary artery and myocardial disease (hypertension, hypercholesterolemia, smoking, steroid therapy, etc), to be vigilant about recognizing the rarer complications associated with SLE (infectious pericarditis and endocarditis, coronary arteritis, pericardial tamponade, clinical myocarditis), and to remember that these uncommon complications are indeed uncommon. The importance of vigorously treating systemic hypertension cannot be overstressed.     

Pulmonary manifestations of systemic lupus erythematosus.

The respiratory system is affected more commonly in systemic lupus erythematosus (SLE) than in any other collagen vascular disease. In this article the many different manifestations of SLE in the pleura, lung parenchyma, lung vasculature, upper and lower airways, and respiratory muscles are reviewed. Moreover, indirect involvement of the respiratory system with SLE is discussed with special reference to pulmonary infection and drug-induced SLE pulmonary disease.     

Neuropsychiatric involvement in systemic lupus erythematosus

The correct diagnosis of central and peripheral nervous system manifestations in patients with systemic lupus erythematosus (SLE) can be challenging because of many SLE-related and non-SLE-related processes that can be present in a patient. The American College of Rheumatology (ACR) has published case definitions for 19 neuropsychiatric SLE (NPSLE) syndromes, with careful attention to important exclusion criteria. These criteria were developed for research purposes but can be helpful to clinicians with a patient who has nervous system dysfunction. This report reviews the data regarding the application of the ACR NPSLE criteria and the influence of ethnicity and disease duration on the development of NPSLE syndromes. Cognitive dysfunction and psychiatric disorders are the two most common manifestations. The work-up and treatment of nervous system syndromes are also discussed.     

Respiratory involvement in systemic lupus erythematosus

Respiratory involvement in systemic lupus erythematosus (SLE) is not as well-known as the cutaneous, rheumatological and renal manifestations. It occurs frequently but the diagnosis may be difficult because of the heterogeneity of the anatomical and clinical presentations. A precise diagnosis is crucial as new immunosuppressive drugs have considerably improved the prognosis. The pathology involves genetic, endocrine, environmental, pharmacological and immunological factors with a cytotoxic reaction of auto-antibodies against complement, a circulating immune complex reaction and a hyperactivity of B lymphocytes. Respiratory involvement in SLE can be classified in five groups based on the anatomy: pleural involvement, infiltrating pneumonia (lymphoid interstitial pneumonia, bronchiolitis obliterans with organizing pneumonia and acute lupus pneumonitis), airways involvement (upper airways, bronchi), vascular involvement (pulmonary hypertension, acute reversible hypoxaemia, alveolar haemorrhage, and antiphospholipid syndrome), muscular and diaphragmatic involvement (shrinking lung syndrome).Treatment is based, depending upon the type of involvement and its severity, on steroids which may be combined with immunosuppressants and plasmapherisis.     

Pulmonary disease in systemic lupus erythematosus

Pulmonary problems are common in systemic lupus erythematosus, and may be the presenting feature of this multi-system disease. The clinical spectrum ranges from mild, self-limited, pleuritic chest pain to fulminant and rapidly fatal, diffuse, pulmonary hemorrhage. Accordingly, treatment must be individually tailored to the clinical features of each patient. Non-steroidal-anti-inflammatory drugs may be adequate therapy for pleuritic pain. High dose corticosteroids may be indicated in more severe cases of pleurisy with effusion, lupus pneumonitis, and diffuse interstitial lung disease. Immunosuppressive drugs such as azathioprine and cyclophosphamide should be considered in cases of lupus pneumonitis or interstitial lung disease unresponsive to steroids. Combined therapy with corticosteroids, immunosuppressives and plasmapheresis should be considered for fulminant cases of diffuse pulmonary hemorrhage attributed to lupus. There is no definitive therapy for pulmonary hypertension at this time. Decisions regarding treatment in each instance must be made with the recognition that there is little strong clinical evidence to support the use of any of these therapies. Finally, no pulmonary process should be attributed to lupus until infection has been rigorously excluded in these patients.     

Pulmonary hypertension in systemic lupus erythematosus

The occurrence of pulmonary hypertension in systemic lupus erythematosus (SLE) has been rarely reported. To date only 18 well documented cases of SLE with pulmonary hypertension have been published. We review the literature on pulmonary hypertension in SLE, and add the case of a young woman with a recent onset of both SLE and pulmonary hypertension. Hemodynamic studies were carried out before and during treatment with nifedipine and hydralazine alone and in combination. Despite improvement in pulmonary vascular resistance, pulmonary pressures were unaffected by the drugs, although our patient had symptomatic relief.     

Pulmonary hypertension in systemic lupus erythematosus

A prospective study was performed in our center on 60% (n = 36) of patients with systemic lupus erythematosus (SLE) to determine the prevalence and severity of pulmonary hypertension. Twenty-six healthy subjects of similar age and sex served as controls. Pulmonary artery systolic pressure was calculated from the sum of the peak tricuspid insufficiency Doppler pressure gradient and an estimate of right atrial pressure based on inferior vena cava size and its degree of inspiratory collapse. Five patients with SLE (14%) had pulmonary hypertension, defined as pulmonary artery systolic pressure greater than 30 mm Hg. Cardiac indices determined by planimetry of biplane apical 2-dimensional echocardiographic images were low or normal in the patients with pulmonary hypertension implying increased pulmonary vascular resistance as the etiology for elevated pulmonary artery pressure. The mean pulmonary artery systolic pressure in patients with SLE was 25 +/- 10 mm Hg vs 20 +/- 2 in controls (p = 0.002). No control had a pulmonary artery systolic pressure greater than 23 mm Hg. Patients with pulmonary hypertension had a shorter duration of SLE and steroid therapy and a higher prevalence of cytotoxic treatment and Raynaud's phenomenon in comparison to those with normal pulmonary artery pressures. The prevalence of systemic hypertension, interstitial lung disease, pleurisy, pericarditis, cutaneous manifestations, arthritis, renal disease, central nervous system involvement, and hematologic abnormalities was similar in patients with SLE with normal and elevated pulmonary artery pressure. Our study suggests that pulmonary hypertension in SLE is common but usually mild.     

Pulmonary hemorrhage in systemic lupus erythematosus

OBJECTIVE: To determine the clinical features of our systemic lupus erythematosus (SLE) patients presenting with pulmonary hemorrhage (PH). METHODS: We reviewed the records of all SLE patients who had PH between 1994 and 2001, a total of 22. RESULTS: All patients had radiographic infiltrates. The mean drop in hemoglobin was 3.2 +/-1.1 g/dL, hemoptysis occurred in 50%, the Dlco was increased in 10 of 11 patients (91%), and 11 of 14 patients who underwent bronchoscopy had positive findings. All received high-dose prednisolone and most also were given pulse methylprednisolone (MEP) and cyclophosphamide. All patients required care in the intensive/monitored care unit, 14 were intubated, 11 had plasmapheresis, and 8 died as a result of PH (mortality rate, 36%). SLEDAI and SLAM were able to indicate active disease in SLE patients with PH (median SLEDAI, 19 [interquartile range, 10 to 24] and mean SLAM, 16.1 +/- 5.8). There was a statistically significant increase in the SLEDAI from 1 month prior to PH to the time of PH (P =.014), indicating that the patients were having a significant disease flare. The median SLEDAI and SLAM scores of patients who died were slightly higher than that of survivors. CONCLUSION: PH in SLE patients occurred in those with severe, multiorgan involvement, with high SLEDAI scores. A high degree of suspicion should be maintained in lupus patients with active disease and unexplained infiltrates on chest radiographs and dropping red cell indices, even in the absence of hemoptysis. Early aggressive management with high-dose steroids and intravenous pulses of cyclophosphamide is advocated and may explain recent trends of improved survival. Plasmapheresis may be useful for the acutely ill patient who does not respond to the above measures but does not clearly lead to improved survival.     

Pulmonary hypertension in systemic lupus erythematosus

A prospective echocardiographic and clinical study was performed on 84 Chinese patients with systemic lupus erythematosus (SLE) and 99 controls to investigate the prevalence and the mechanism of pulmonary hypertension (PH) in SLE. Comparison between Doppler estimation and catheterization measurement was made in 12 cases to validate the predictive method. Compared to normal subjects, lupus patients had significantly increased sys-tolic pulmonary artery pressure (SPAP) (29.59±12.52 vs 19.64±5.82, P<0.001), mean pulmonary artery pressure (MPAP) (15.11±7.36 vs 10.21±4.72, P<0.001) and total pulmonary resistance (TPR) (315.85±190.65 vs 220.37± 55.92, P<0.001). Nine of the 84 patients presented PH, defined as SPAP >30 mmHg and MPAP >20 mmHg. Pulmonary hypertensive patients had higher serum endothelin (ET) than non-pulmonary hypertensive patients, were more commonly in active stages, and presented Raynaud's phenomenon and rheumatoid factors. ET level was correlated with echocardiographic pulmonary pressure. Pulmonary hypertension commonly occurs in Chinese patients with SLE (11%), and it correlates with the lupus activity and the elevation of serum endothelin. Received: 29 April 1998 / Accepted: 30 September 1998