Case Studies
Massive pleural effusion in a patient with systemic lupus erythematosus
Durdu Mehmet Yavsan1, Ali Yavuz Karahan2 , Muhammed Sahin2, Tayfun Gungor2, Adem Kucuk3*, Emin Maden1, Recep Tunc3
Author Affiliations
1Departments of Chest Diseases, Meram School of Medicine, Necmettin Erbakan University, Meram, Konya, Turkey
2Department of Physical Medicine and Rehabilitation, Meram School of Medicine, Necmettin Erbakan University, Meram, Konya, Turkey
3Department of Internal Medicine, Division of Rheumatology, Meram School of Medicine, Necmettin Erbakan University, Meram, Konya, Turkey
Correspondence: Dr. Adem Kucuk
IJRCI. 2015;3(1):CS1
Received: 1 October 2014, Accepted: 31 December 2014, Published: 8 January 2015
© IJRCI
Abstract
Systemic lupus eryhtematosus (SLE) is a chronic systemic inflammatory disease of unknown etiology, which may cause life-threatening tissue and organ damage. Pleural effusion in SLE tends to be small and bilateral. The present case study focuses on the medical management of a 24-year-old SLE patient with massive pleural effusion who presented with cough and shortness of breath.
Introduction
Systemic lupus eryhtematosus is a chronic systemic inflammatory disease of unknown origin that causes an overactive immune system. It usually affects women of childbearing age.1-2 SLE may manifest itself with several symptoms including fever, fatigue, joint pain, mild rash, and arthritis. It can also cause life-threatening damage to tissues and organs (such as kidneys or heart).1 Pleural effusion affects 16-50% of SLE patients and tends to be small and bilateral.3 The aim of this case report is to draw attention to medical management of massive pleural effusion, which rarely occurs in SLE patients.
Case report
A 24-year-old female patient presented with shortness of breath and cough that became worse with exercise during the previous month. She reported a history of photosensitivity and episodes of intermittent arthritis, which affected her wrists and proximal interphalangeal joints. She did not have any family history of connective tissue disease. She rarely consumed alcohol and smoked 4 packs of cigarettes per year. Her heart sounds were distant upon physical examination. During ausculation, the respiratory sounds could not be obtained from the middle area of the left hemithorax and onwards, and the percussion sounds were dull. Lymphadenopathies were present in the bilateral submandibular area. Chest radiography revealed elevated cardiothoracic index as well as increased density in the middle and lower zones of the left hemithorax, which was consistent with pleural effusion (Fig. 1). A left hemithorax thoracentesis was performed for therapeutic and diagnostic purposes, which revealed that the thoracentesis fluid appeared serous, which met the Light criteria for exudative pleural effusion (Table 1). Cytological examination of the pleural fluid did not reveal signs of malignancy, nor were acid-resistant bacilli present in the pleural fluid. Direct Gram staining and culture were negative for bacteria and fungi.
Fig. 1: Increased density in the middle and lower zones of the left hemithorax, which was consistent with pleural effusion
Table 1: Serum and pleural fluid characteristics of the patient
Results of arterial blood gas analysis were as follows: pH - 7.44, PCO2 - 33.7, PO2 - 81.8, and HCO3 - 22.7. Therefore, the patient appeared to have metabolic acidosis. Whole blood analysis showed the following: hemoglobin level - 8.4 g/dl, platelet count - 420x103/u, MCV - 65.2 fL, red blood cell count - 4.38x106/uL, red cell distribution width - 13.2%, and ferritin - 132 ng/mL. Her partial thromboplastin time was 37.1 and the international normalized ratio was 1. Hemoglobin electrophoresis revealed hemoglobin A2 to be 5.3%, which was consistent with thalassemia minor. Complete urinalysis did not show any particular feature. Rheumatologic blood tests were highly positive. The anti-nuclear antibody was granular and homogenous (1:320 with indirect fluorescent antibody). Additionally, she was found to be positive for anti-dsDNA, anti-Smith (Sm) and anticardiolipin IgM antibodies. Complement levels were within normal range, and rheumatoid factor was negative. Protein electrophoresis revealed hypergammaglobulinemia, IgG of 33.5 g/L, IgM of 2.06 g/L, and IgA of 4.3 g/L. ECG revealed right bundle branch block, while echocardiography showed 60% ejection fraction and minimal pericardial effusion. Multiple lymph nodes with hilar structures were observed during the neck ultrasonography, with the largest nodes being 13x5 mm at the right side in the mandibular zone and 20x6 mm at the left side.
The patient was treated with hydroxychloroquine, acetylsalicylic acid, methylprednisolone, and azathioprine. High-dose, pulse intravenous methylprednisolone of 1 g was given daily for three days and the dose was decreased gradually. Her respiratory symptoms and pleural effusion were considerably regressed during the follow-up visits (Fig. 2). In addition, regression of bilateral lymphadenopathies was observed.
Fig. 2: Chest X- ray after treatment
Discussion
SLE, an autoimmune disorder affecting multiple organs, predominantly occurs in women. Isolated pulmonary involvement is infrequent in SLE. Although pleuritis is the most common respiratory symptom, pleural effusion occurs at a rate of 1-2% as the initial sign in SLE patients. Pleuritis is more commonly observed in patients with late-onset SLE.4-5 Typical signs of pleural involvement include acute chest pain accompanied by fever, cough, and dyspnea. Although the occurrence of serositis is frequent, the cause of pleural effusion in SLE is usually renal, cardiac or pulmonary embolism. Pleural effusion is typically small and bilateral, and is rarely massive. In SLE, pleuritis develops as a result of inflammation due to the production of immune complex and complement activation. Pleural fluid specimens often have protein levels above 3.5 g/dL, LDH levels below 500 U/L, and decreased glucose concentrations. In addition, low complement levels and high anti-nuclear antibody levels (>1/160) are suggestive of SLE. However, ANA titers may also increase in neoplastic effusions.
Lupus eryhtematosus (LE) cells are highly specific.5-10 There are different manifestations of respiratory system involvement in SLE, in addition to pleuritis (with or without effusion). One end of the wide spectrum comprises of interstitial lung diseases, acute lupus pneumonitis, diffuse alveolar hemorrhage, pulmonary arterial hypertension, and mostly pulmonary infections. Whereas, the other end includes minor conditions of lesser concern like acute reversible hypoxemia.6, 11 In the present case report, the patient had shortness of breath, cough, arthritis, lymphadenopathy, and photosensitivity. She also reported that she used to consume alcohol and smoke cigarettes. Smoking generally increases the risk of lupus flares. On the other hand, moderate/reasonable alcohol intake has been reported to confer a protective effect.12-14
Even though there are many potential causes of pleural effusion and congestive heart failure (CHF), the most common etiologic factors include malignancy, pneumonia and pulmonary embolism. Several disorders may cause effusion, despite the involvement of many different organs. History and a careful physical examination are very helpful for diagnosis. Weight loss and hemoptysis suggest malignancy, and fever may be indicative of empyema, pneumonia and tuberculosis. However, articular, ophthalmic and skin symptoms suggest connective tissue disorders.15-17
If pleural effusion observed in a SLE patient is small and asymptomatic, no treatment is required. For cases with mild effusion, non-steroidal anti-inflammatory drugs (NSAIDs) may be prescribed. Patients with more severe disease should receive steroid therapy. Hydroxychloroquine and azathioprine should also be given to selected cases. While SLE often responds to medical treatment, refractory cases have also been reported. There is no specific treatment regimen for resistant cases.3
In summary, connective tissue disorders and SLE are mainly responsible for the development of pleural effusion, particularly among younger women. Here, we described a case of SLE with a rare cause of massive pleural effusion. Marked bilateral effusion responded well to treatment with azathioprine 2.5 mg/kg, hydroxychloroquine and steroid, and did not require pleurodesis. A limitation of this case report was the failure to perform analyses for ANA and LE cell on the diagnostic thoracentesis fluid.
Conclusion
Early diagnosis of individual components of MAS and commencement of treatment is essential to curb morbidity and mortality associated with MAS. The case study also warrants the need for continued surveillance of predisposed patients for MAS. In any patient with vitiligo and anemia, pernicious anemia as an autoimmune cause for anemia should be considered.
Competing interests
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
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