|Year : 2016 | Volume
| Issue : 1 | Page : 38-41
Lupus erythematosus cells in vivo (pleural fluid): An unusual presentation of systemic lupus erythematosus
Majed Momin1, G Amitha1, Abhijeet Ingle1, C Sandhya Rani1, Karishma Rosann Pereira2
1 Department of Pathology, Microbiology and Medical Publications, Yashoda Hospital, Hyderabad, Telangana, India
2 Department of Medical Publications, Yashoda Hospital, Hyderabad, Telangana, India
|Date of Web Publication||2-Aug-2016|
Karishma Rosann Pereira
Department of Medical Publications, Yashoda Hospital, Nalgonda X-Roads, Malakpet, Hyderabad - 500 036, Telangana
Source of Support: None, Conflict of Interest: None
Systemic lupus erythematosus (SLE) is an autoimmune disease in which organs and cells undergo damage mediated by tissue-binding autoantibodies and immune complexes. In earlier times, the diagnosis of SLE included the lupus erythematosus (LE) cell assay. Though the LE cell test has now been declared obsolete, its presence carries importance especially when one finds LE cells in vivo. We report an unusual case of SLE in a 27-year-old female who presented with acute shortness of breath, fever, and cough. On initial outpatient basis investigations, the patient was detected with anemia, bilateral pleural effusion, synovitis, and juxta-articular swelling of the soft tissues. Her chest radiograph effusion was tapped and sent to the cytopathology laboratory. The cytological examination of the pleural fluid revealed numerous LE cells that prompted the diagnosis of SLE. Autoimmune serology techniques such as antinuclear antibody staining have replaced the LE cell assay. However, as presented in this report and found in a review of the literature, the in vivo finding of LE cells by cytopathology can provide an important clue to the diagnosis of SLE. This case is interesting because although pleural effusion is common, very rarely LE Cells are encountered in vivo. There are very few case reports of SLE diagnosed in a cytopathology laboratory. Moreover, our finding of LE cells in the pleural fluid of the patient led to swift diagnosis, helped rule out the differential diagnosis of rheumatoid arthritis and prompted the immediate initiation of treatment. Furthermore, our case highlights the fact that no matter the advances in diagnostic testing methods, practitioners must always keep an eye open for the basic pathognomonic findings of diseases.
Keywords: Lupus erythematosus cells, pleural effusion, rheumatoid arthritis, systemic lupus erythematosus
|How to cite this article:|
Momin M, Amitha G, Ingle A, Rani C S, Pereira KR. Lupus erythematosus cells in vivo (pleural fluid): An unusual presentation of systemic lupus erythematosus. Indian J Allergy Asthma Immunol 2016;30:38-41
|How to cite this URL:|
Momin M, Amitha G, Ingle A, Rani C S, Pereira KR. Lupus erythematosus cells in vivo (pleural fluid): An unusual presentation of systemic lupus erythematosus. Indian J Allergy Asthma Immunol [serial online] 2016 [cited 2019 Sep 22];30:38-41. Available from: http://www.ijaai.in/text.asp?2016/30/1/38/187568
| Introduction|| |
Systemic lupus erythematosus (SLE) is a common autoimmune disorder that primarily affects women and can involve virtually any organ in the body.  SLE is typically diagnosed by a combination of physical findings and clinical laboratory testing.
In olden days, lupus erythematosus (LE) cell test was one of the earliest tests to exclude connective tissue disorders. With the advent of new analyzers such as the indirect immunofluorescence and cheminoluminoscence for SLE, the diagnostic sensitivity of antinuclear antibodies (ANA) test has increased so markedly that LE cell test has almost become obsolete.
Lupus pleuritis is the most common manifestation of SLE in the chest, but it occurs as an initial presentation in only 2.5-3% of patients. Pleural effusion itself can be very rarely the first manifestation of SLE, seen in 1-2% of patients. The prognosis of lupus pleuritis/effusion is generally good. 
The LE preparation examines the conversion of neutrophils to typical LE cells when normal neutrophils are incubated in vitro in the presence of serum factor obtained from individuals with SLE. However, this in vitro phenomenon may not represent the in vivo phenomenon occurring in pleural fluid. As presented in this report and review of literature, the in vivo finding of LE cells by cytopathology remains an important finding consistent with the diagnosis of SLE.
| Case report|| |
A 27-year-old female was referred to our hospital (Yashoda Hospital, Malakpet Branch, Hyderabad), with a history of low-grade continuous fever not associated with chills and rigors. She had a cough (nonproductive), joint pains, and accompanying swelling over joints. The symptoms mimicked rheumatoid arthritis (RA) and so the patient was treated with cox-2 inhibitors and antibiotics. However, there was no significant improvement in the symptoms and the patient developed difficulty in breathing and epigastric discomfort. At the time of presentation in the emergency room, the patient had shortness of breath, low-grade fever, and swelling of proximal interphalangeal (PIP) joints. On general examination, the patient exhibited pallor; all the PIP joints of both the upper limbs were swollen andnon-tender [Figure 1]. Auscultation revealed decreased breath sounds in both lower lung fields. Routine investigations and chest X-ray including joints were advised and conducted.
A chest radiograph demonstrated blunting of costophrenic angles and bilateral pleural effusion [Figure 2]. In the meanwhile, worsening of dyspnea necessitated surgical drainage of the pleural effusion. Approximately, 500 mL of serosanguinous fluid was tapped and sent to the cytopathology laboratory for evaluation.
X-ray hand revealed symmetrical soft tissue swelling at the PIP joint-synovitis, juxta-articular osteopenia, and no erosion [Figure 3].
|Figure 3: Symmetrical soft tissue swelling at proximal interphalangeal joint-synovitis|
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On cytopathology examination of the May-Grünwald-Giemsa-stained cytospin preparation of the pleural fluid, many LE cells were seen, characterized by pink homogenous nuclear material engulfed by neutrophils. These cells were present in the background of numerous segmented neutrophils and few macrophages [Figure 4].
|Figure 4: (a-c) Cytospin preparation of the pleural fluid showing plenty of lupus erythematosus cells|
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Other laboratory investigations included the white blood cell count (11 × 10 3 /μL with a normal differential count), hemoglobin 9.7 g% hematocrit (29%), platelet count (180 × 10 3 /μL), and erythrocyte sedimentation rate (70 mm/h). RA factor and anti-cyclic citrullinated peptide (CCP) antibody came negative. A battery of tests was conducted including ANA, antibody profile, and ANA immunofluorescence (IF) study, which came positive for ANA, ds DNA and nucleosomes. ANA IF was positive with homogenous nuclear pattern with 3+ intensity. Finally, this was treated as a case of SLE, started on oral corticosteroids and hydroxychloroquin. There was rapid clinical response including resolution of the fever and pleural effusion [Figure 5].
| Discussion|| |
SLE is a chronic inflammatory autoimmune disorder that commonly presents with arthralgias, arthritis, a rash (which may be photosensitive), and renal involvement. Pulmonary involvement in SLE is common, pleuritis being the most frequent manifestation. Pleural inflammation is a common feature of SLE; however, as an initial presentation in SLE, it is very rare, reported only in 1-2% of cases.  Other pulmonary manifestations of SLE include pneumonitis, alveolar hemorrhage, bronchiolitis obliterans with organizing pneumonia, lymphocytic interstitial pneumonia, pulmonary hypertension, vasculitis, pulmonary embolism, and diaphragmatic weakness. 
In review and literature and an analysis of 520 patients with SLE by Dubois and Tuffanelli,  pleuritis occurred in 45% of patients, and pleural effusion occurred in 30%. Pleurisy and pleural effusion were the initial manifestation in 3% and 1% of patients, respectively. However, in patients with late-onset (after the age of 50 years) SLE, pleuritis is even more common. In one study, pleuritis was the presenting manifestation in 27% of patients with late-onset SLE.
In this case, the patient presented with shortness of breath accompanied by fever and cough. Several diagnostic possibilities exist in such a case including pulmonary embolus, viral infection, parapneumonic effusion, tuberculosis, congestive heart failure, and collagen vascular disorders. There is clinical overlap between SLE and RA. The absence of anti-CCP antibodies and Rh factor and a simple cytological preparation revealed plenty of LE cells and led to the diagnosis of SLE, which was further confirmed by serum ANA, IF, and antibody detection tests. Oral steroid therapy was initiated, after which the bilateral pleural effusion dramatically improved. Serous effusions as a result of SLE tend to be more common in the chronic stage of the disease, and the presence of LE cells in an effusion is associated with the presence of active disease. ,
Pleural effusion due to lupus pleuritis is typically an exudate and may be unilateral or bilateral. The presence of LE cells in the pleural fluid is highly specific for SLE. LE cells are neutrophilic phagocytes rarely monocytes that contain intracytoplasmic hematoxylin bodies. The hematoxylin bodies or LE bodies are thought to be formed by the opsonization of cells by ANA typically found in SLE patients. These antibodies cause the denaturation of dead or injured cells, forming homogenous, oval-shaped bodies that are referred to as "hematoxylin bodies." These hematoxylin bodies are engulfed by neutrophils to create LE cells. The incubation of the pleural fluid at room temperature for several hours may enhance the LE cell phenomenon. 
LE cells are also seen in bone-marrow aspiration material, synovial fluid, cerebrospinal fluid, and pericardial fluid of SLE patients. The presence of LE cells in any of these specimens, in conjunction with the appropriate clinical picture and laboratory values, would contribute to the diagnosis of SLE. 
The prognosis of lupus serositis is generally good. Most patients with lupus pleuritis respond favorably to oral corticosteroids. The clinical and radiographic improvement is rapid and complete in most patients. Other immunosuppressive agents, such as azathioprine, may be used in patients whose response to steroids is slow or unsatisfactory.
| Conclusion|| |
In conclusion, although the identification of LE cells as one of the diagnostic criteria for SLE has been obsolete; however, as presented in this report and review of the literature, the in vivo finding of LE cells by cytopathology remains a significant finding consistent with the diagnosis of SLE that cannot be underestimated.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Park JY, Malik A, Dumoff KL, Gupta PK. Case report and review of lupus erythematosus cells in cytology fluids. Diagn Cytopathol 2007;35:806-9.
Sarwar A, Dellaripa PF, Beamis JF Jr. A 51-year-old man with fever, ulnar neuropathy, and bilateral pleural effusions. Lupus pleuritis. Chest 1999;116:1105-7.
Wan KS. Pleuritis and pleural effusion as the initial presentation of systemic lupus erythematous in a 23-year-old woman. Rheumatol Int 2008;28:1257-60.
Hepburn AL. The LE cell. Rheumatology (Oxford) 2001;40:826-7.
Dubois EL, Tuffanelli DL. Clinical manifestations of systemic lupus erythematosus. Computer analysis of 520 cases. JAMA 1964;190:104-11.
Icen M, Nicola PJ, Maradit-Kremers H, Crowson CS, Therneau TM, Matteson EL, et al.
Systemic lupus erythematosus features in rheumatoid arthritis and their effect on overall mortality. J Rheumatol 2009;36:50-7.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]