Presentation and prognosis of shrinking lung syndrome in systemic lupus erythematosus: report of four cases

Systemic lupus erythematosus is an autoimmune systemic disease that commonly affects the respiratory system. Shrinking lung syndrome is a rare respiratory complication associated with systemic lupus erythematosus. Patients present with dyspnea alone or associated with chest pain and orthopnea, lung volume reduction with no parenchymal abnormalities and a restrictive ventilatory defect on pulmonary function tests. The pathogenesis, treatment, and prognosis of shrinking lung syndrome remain controversial. This study describes the clinical features, investigations, and outcome of a series of four patients with systemic lupus erythematosus and shrinking lung syndrome regularly followed on Rheumatology Service of the Clinics Hospital of the Federal University of Minas Gerais, Brazil, with a brief review of literature. It emphasizes that, despite prognosis of shrinking lung syndrome has been reported as good, it may cause severe functional pulmonary abnormalities and must be treated promptly and aggressively in order to, at least, stabilize pulmonary function tests.     

Estimating prognosis in systemic lupus erythematosus

Knowledge of prognosis is critical for rational choice of therapy. Systemic lupus erythematosus, with its broad clinical spectrum, presents an example of the problem of prognostication for the individual patient. The life table method can provide prognostic information. It need not be limited to estimation of survivorship, and its utility can be multiplied by a computer databank. Analysis of prognosis for different groups of patients, from different events in their course, over different time periods, and to different end points can then be performed. Using this technic, specific clinical variables in systemic lupus erythematosus were assessed for their effect upon particular outcomes. Clinical variables representing functional organ impairment were found to carry a worse prognosis than the popular serologic indicators. New abnormalities were found to occur less frequently in later years of the disease. Different subpopulations of patients have strikingly different expectations, and these differences carry important therapeutic implications.     

Outcome and prognosis in systemic lupus erythematosus

We have attempted to assess the factors associated with prognosis in SLE, and to document the temporal changes in outcome, related not only to improvements in survival but to the emergence of an increased prevalence or morbidity, related to disease manifestations, complications of treatment, and co-morbid conditions.     

Shrinking lung syndrome in systemic lupus erythematosus patients with dyspnea

Aim of the workTo identify the frequency of shrinking lung syndrome (SLS) in systemic lupus erythematosus (SLE) with dyspnea and study the clinical characteristics and differences in disease activity and damage.Patients and methodsThe study included 47 SLE patients complaining of dyspnea. SLS was considered in those with exertional dyspnea, restrictive pulmonary function tests (PFTs) and elevated copula of the diaphragm. Full history taking, thorough clinical examination, laboratory and relevant radiological investigations were performed for all the patients. Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Systemic Lupus International Collaborating Clinics (SLICC) indices were compared. High resolution CT chest was performed for patients with radiological findings consistent with SLS.ResultsThe mean age of the patients was 29.43 ± 7.45 years, mean disease duration 5.18 ± 3.62 years. The SLS was present in 8 patients (17.02%). There was bilateral elevation of the diaphragm copulae in 25% of SLS patients and two had associated basal atelectatic bands. The serum uric acid was significantly higher in those with SLS while the 24 h urine protein was significantly lower and C4 normalized. The levels of SLEDAI and SLICC tended to be lower in those with SLS, yet there was no significant difference from those without. The demographic features, clinical and laboratory manifestations, disease activity and damage scores, PFTs and radiological findings of the SLE patients are presented.ConclusionIn SLE patients with dyspnea, SLS should be looked for as it is present in a high proportion of cases.     

Systemic vascular lesions and prognosis in systemic lupus erythematosus

The purpose of this paper is to show the relationship of systemic vascular lesions to the clinical manifestations and prognosis in SLE. Thirty-four autopsied cases of SLE formed the subject material for this study. Histopathological tissue studies were made on vascular lesions from almost all organs. Vascular lesions were separated into three groups according to the size of the involved vessel: 1) medium-sized artery (9 cases), 2) small artery (12 cases) and 3) (13 cases) without systemic vascular lesions. Vascular lesions were also separated into five groups (a) fibrinoid degeneration (10 cases), b) intimal thickening (6 cases), c) thrombosis (6 cases), d) sclerosis (7 cases) and e) (13 cases) without systemic vascular lesions. Patients with involvement of medium-sized arteries had a low female incidence, photosensitivity, and positive LE cell incidence, and the cause of death in these patients was cerebral vascular involvement. The cause of death in patients with vascular fibrinoid degeneration and thrombosis was mainly uremia, whereas patients with sclerosis more often died from infection. Regarding systemic vascular lesions in SLE, the prognosis for the patients with thrombosis and vascular involvement of medium-sized arteries was the most grave.     

Interstitial lung disease in systemic lupus erythematosus

Systemic lupus erythematosis (SLE) is a heterogenous disease of unknown etiology. It is not uncommon to see pleuropulmonary involvement in isolation or along with the involvement of other organ systems in SLE. Pulmonary manifestations of SLE can involve the pleura, lung parenchyma, airways, pulmonary vasculature, and the respiratory muscles. In this review we discuss two important pulmonary manifestations of SLE: acute lupus pneumonitis and diffuse interstitial lung disease. These two conditions have a major impact on the mortality and morbidity of patients with SLE and it is essential to recognize and treat them appropriately. High-resolution computed tomographic scans of the chest and pulmonary function tests help to establish a diagnosis and aid long-term follow-up of these patients. High-dose corticosteroids are the mainstay of treatment for the two conditions, although other agents such as cyclophosphamide, azathioprine, intravenous gamma globulin, and plasmapheresis have been used with varying degrees of success.     

Shrinking lung syndrome in a 14-year-old boy with systemic lupus erythematosus

Pulmonary complications occur frequently in people with systemic lupus erythematosus. We report on an adolescent with an acute onset of dyspnea and pleuritic chest pain with severe restrictive lung physiology on pulmonary function testing (forced vital capacity, 20% of predicted) who had no evidence of parenchymal lung or pleural disease. He was found to have restricted diaphragmatic movement as assessed by fluoroscopy, without evidence of generalized respiratory muscle weakness. His clinical presentation and results of diagnostic tests were typical for shrinking lung syndrome. Given the rarity of shrinking lung syndrome in the pediatric age range, many clinicians are not aware of it as a clinical entity. Shrinking lung syndrome should be included in the differential diagnosis of dyspnea in both children and adults with systemic lupus erythematosus.     

Long-term prognosis and causes of death in systemic lupus erythematosus

Purpose

We studied survival rate, prognostic factors, and causes of death in patients with systemic lupus erythematosus (SLE), particularly focusing on the influence of disease severity.

Patients and Methods

A cohort of 207 consecutive Italian patients with SLE were prospectively studied. All prominent clinical and serologic parameters were evaluated and considered as prognostic risk factors. Causes of death were defined on the basis of clinical data and, when available, postmortem examination. Survival was calculated from the time of diagnosis by Kaplan-Meier method.

Results

A total of 17 of 207 patients died; causes of death were active disease manifestations in 35.3% of cases and complication of the disease or its treatment in 64.7% of cases. The survival rates at 5, 10, and 15 years after the diagnosis were 96%, 93% and 76%, respectively. By multivariate analysis of the risk factors, a predictive model consisting of male gender, positive lupus anticoagulant, and “severe” SLE was identified. The survival curve of the patients with severe disease was similar to that of patients with mild disease until 10 to 15 years from the diagnosis. Thereafter the two curves tended to diverge, showing a clear survival decline in patients with severe disease.

Conclusions

Our study confirms the increase of short- and medium-term survival in patients with SLE, but long-term prognosis remains poor in patients with severe SLE manifestations.     

Miller Fisher syndrome in systemic lupus erythematosus

We describe the first case of Miller Fisher syndrome, a Guillain Barré variant, complicating systemic lupus erythematosus. The symptoms and signs mimicked a brainstem syndrome. Despite treatment with high dose gamma globulin, our patient worsened and required mechanical ventilation. After plasma exchange, the patient improved.     

Atenolol induced systemic lupus erythematosus syndrome

We report the first case of an illness resembling idiopathic lupus erythematosus, with fever, pleuropericarditis, antinuclear antibodies and antidenaturated DNA antibodies after 18 months of treatment with atenolol for hypertension. After withdrawal of atenolol our patient's clinical symptoms disappeared and laboratory test results returned to normal, which strongly suggests the role of atenolol in inducing the syndrome, therefore atenolol should be added to the list of beta blocking agents capable of inducing a lupus-like syndrome.     

POEMS syndrome in systemic lupus erythematosus.

POEMS syndrome [plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, monoclonal (M) protein, skin changes] although mimicking the connective tissue diseases in several ways has not been reported in a patient who also meets criteria for the diagnosis of systemic lupus erythematosus (SLE). We describe a patient with POEMS syndrome and SLE and review the similarities and differences between POEMS syndrome and other connective tissue disorders.     

Intestinal pseudo-obstruction syndrome in systemic lupus erythematosus

Intestinal pseudo-obstruction (IpsO) occurs in a small subgroup of patients with systemic lupus erythematosus (SLE), and has been under-recognized. The etiology and mechanisms are unclear. Herein, we report two cases. In case 1, IpsO occurred as the complication in a previously diagnosed SLE patient. In case 2, IpsO with an image-evidenced intussusception and a peripheral eosinophilia, was the initial presentation, leading to the diagnosis of SLE. In both cases, IpsO existed concomitantly with ureterohydronephrosis, and evolved into peritonitis, which we called together IpsO syndrome. However, this surgical abdomen-like syndrome had a satisfactory response to glucocorticosteroids instead of antibiotics and surgery. Physician awareness and early diagnosis could positively affect outcomes of this syndrome, avoiding unnecessary surgical intervention.