Case Report

Systemic Lupus Erythematosus Presenting with

Recurrent Pleural Effusion without any Systemic
Manifestation
B Mitra*, P Sengupta*, K Saha*, N Sarkar**, J Pal***
Abstract
Pleural effusion can be the sole presenting manifestation in about 5 percent of cases with SLE.
We are reporting a case of SLE which presented with recurrent pelural effusion without other systemic
manifestation.

INTRODUCTION

ystemic lupus erythematosus (SLE) affects

predominantly women. Pleurisy is the most common
thoracic manifestation of SLE1 Clinically apparent
pleural effusions have been reported in up to 50 percent
of patients with SLE and in up to 93 percent of cases at
necropsy.2 Pleural effusion can be the sole presenting
manifestation in about 5 percent of cases with SLE.3 We
are reporting a case of SLE, which presented with
recurrent pleural effusion without other systemic
manifestation.

CASE
A 20 years girl, student nondiabetic nonhypertensive
was admitted in our institute in April 2004, with chief
complaint of progressive dyspnoea, for last 7 days,
without any pleuritic type of chest pain, productive
cough or hemoptysis. There was no history of palpitation,
PND, dependent edema or oliguria. There was a history
of low-grade intermittent fever for seven days without
any joint pain or dysuria.
In February 2004 she developed similar episodes of
dyspnoea, intermittent fever, weakness, loss of appetite,
lethargy, for which she was admitted in local hospital,
and diagnosed to have bilateral pleural effusion. At that
time there were bilateral palpable axillary lymph nodes,
one of which was biopsied, histopathology revealed:
reactive hyperplasia. Pleural biopsy was also done and
showed nonspecific reaction.
*Resident; **Professor; ***RMO; Department of Medicine,
Institute of Post Graduate Medical Education and Rresearch,
SSKM Hospital, Kolkata.
Received : 19.07.2004; Revised : 13.10.2004;
Re-Revised : 4.1.2005; Re-Re-Revised : 3.6.2005;
Accepted : 8.11.2005
JAPI VOL. 53 DECEMBER 2005

She was treated with four antitubercular drug (ATD),

(INH-300mg, Rifampicin 450mg, Ethambutol 800mg,
Pyrizinamide 1.5gm, her body weight was 61Kg) + oral
steroid (60mg/day) + inj ceftrioxone (2gm/day: for 7
days). As she was improved within 3 weeks she was
discharged with four drugs ATD with oral steroid. She
continued the ATD and steroid only for 3 weeks after
discharge, and thereafter she stopped all treatment by
herself without any medical consultation.
In July 2002 there was a similar episode of right sided
pleural effusion for which her family physician had
prescribed four drug ATD (IREZ) along with oral steroid
(60 mg/day). She improved clinico radiologicaly over 3
weeks, after which she stopped ATD medications
without any medical consultation.
On examination she was conscious, pulse was-100/
minute, blood pressure was 160/98 mm of Hg,
respiratory rate was 32/minute. Anaemia, jaundice,
clubbing, and neck glands were absent. Neck veins were
not engorged. Lymphoreticular system was normal. On
inspection of the respiratory system there was no chest
wall deformity or shoulder dropping. There were
bilateral intercostal fullness in infraaxillary region. The
movement of the chest wall was restricted bilaterally.
There was no venous prominence. On palpation trachea
was central in position, and percussion note was stony
dull (bilaterally). On auscultation there was diminished
vesicular breath sound bilaterally. Cardiovascular
system apex could not be localized properly, S1 and S2
were audible and there was no murmur or added
sounds. Other systems were normal.
On the day of admission her routine blood
examination was as follows:
Hemoglobin: 12 gm%, RBC 4.9 million/cumm, WBC
7500 (N 60% L 26% E2% B 0% M 22%), platelet count: 3

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lakhs/cumm, ESR: 80 mm/Hr, blood sugar 98mg%,

Urea-34mg%, creatinine 0.6mg%, CRP: 9mg/dl (normal
upto 5 mg/dl), serum lipid profile, T3, T4, TSH, liver
function test, serum LDH were within normal limits.
Urine: protein was-trace, no RBC, no cast was detected.24
hour urinary protein leak was 100mg/day, Sputum for
AFB: negative (3 consecutive days), sputum culture in
Bactec media: was negative. Pleural fluid Adenosine
Deaminase (ADA); 23U/L (normal limit; upto 40ng/dl).
Chest X-ray showed: bilateral massive pleural effusion,
Ultrasonography of chest was done which revealed
multiple fibrous septae in addition to bilateral pleural
effusion. Ultrasonography of abdomen showed no
abnormality. Routine ECG was normal.
Echocardiography showed, a mild pericardial effusion,
no chamber enlargement, no evidence of pulmonary
hypertension, valves were normal, no vegetation or clot
was detected. Pleural fluid Gram stain and culture was
negative for microorganism (three occasions). Mantoux
test was negative. ELISA for HIV 1 and 2 was negative.
Serum antinuclear antibody (ANA) was positive with
the titer value 1:160 (done in Hep-2 cell line). In
fluorescent microscopy the pattern of ANA was
homogeneous. Anti ds DNA was strongly positive in a
titer of 98ng/dl (Normal: upto 4ng/dl).
Pleural fluid routine examination done on various
occasions are given in Tables 1 and 2.
Pleural fluid ANA was positive in a titer of 1:320.
Serum ANA was 1:160 , so pleural/serum ANA ratio
was =2 (>1), pleural fluid LE phenomena not performed.
On the day of admission 500 ml plural fluid was
aspirated from right side of chest, next day 450 ml fluid
was aspirated from left chest, conservative treatment
(with IV antibiotic) was continued. (Fig. 1 shows chest
X-ray on admission, Fig. 2 shows chest X-ray after
thoracocentesis.) After getting all the reports, she was

put on oral prednisolone (1mg/Kg/day) 60 mg per day.

Within 14 days she improved clinically and her chest Xray showed significant radiological improvement (Fig.
3). Four weeks after institution of steroid her chest X-ray
showed near complete resolution of pleural fluid (Fig.
4).
Now she is on oral prednisolone, discharged from
the hospital, and under our follow-up.

Fig. 1 : Chest X-ray during admission in our institute, showing

bilateral pleural effusion, February 2004.

Table 1 : Pleural fluid examination prior to admission

Date
26/02/02
14/02/04
19/02/04
26/02/04

Pleural fluid cell

count (/cumm)
600
55
480
1960

Cell type
N80%
N70%
N78%
N75%

L20%
L30%
L22%
L25%

Protein
(mg%)

Sugar
(mg%)

Pleural fluid
LDH (U/L)

Serum LDH
(100-190 unit/L)

Pleural
fluid pH

2.5
4.7
4.5
5

80
112
123
95

254
274
301
312

148

7.441

152

Table 2 : Pleural fluid examination after admission

Date
07/04/04
16/04/04
19/04/04
29/04/04

Pleural fluid cell

count (/cumm)
1970
1523
490
200

Cell type
N80%
N78%
N68%
N60%

L20%
L22%
L32%
L40%

Protein
(mg%)

Sugar
(mg%)

Pleural fluid
LDH

Serum LDH

Pleural
fluid pH

5.1
4.8
3.7
3.1

123
103
100
98

267
278
312
267

150

7.388
7.322
7.411
7.344

149
147

(Normal pleural fluid protein: 1-2 gm /100ml 5, Normal serum LDH 100-190 U/L, Light criteria for exudative pleural effusion are:
pleural fluid protein/serum protein > 0.5, pleural fluid LDH/serum LDH >0.6, pleural fluid LDH more than two third of the normal
upper limit of the serum value)
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JAPI VOL. 53 DECEMBER 2005

Fig. 4 : Chest X-ray showing near complete resolution during

discharge.
Fig. 2 : Chest X-ray after therapeutic thoracocentesis, one day after
admission

Fig. 3 : Chest X-ray 14 day after initiation of steroid treatment.

JAPI VOL. 53 DECEMBER 2005

DISCUSSION
In SLE pleural effusion usually bilateral and small to
moderate in size, massive effusion leading to mediastinal
shift have also been recorded. 4 Immune complex
deposition in pleural microvasculature and activation
of complement plays an important role in pathogenesis
of pleurisy and pleural effusion.
The pathogenesis of pleural effusion in SLE differs
from that of RA, for example, immune complexes in RA
are thought to be produce locally in the pleura, whereas
immune complexes are derived from the circulation in
SLE. In addition, the concentration of soluble interleukin2 (IL-2) receptors in the pleural fluid in RA is significantly
higher than that in SLE. This suggests that a local T-cell
mediated immune reaction may be a more important
mechanism in rheumatoid pleurisy than in lupus.5 In
an autopsy study 54 of 58 patients (93%) in Ropes series
showed pleural involvement, in 33 patients fluid were
found in pleural space and adhesion seen in 63% cases.
Varing degree of microscopic change were found in 24%
cases, which consisted of accumulation of lymphocyte
and macrophage, pleural thicking, perivascular
fibrinoid necrosis with neutrophilic and mononuclear
infiltrates, fibrinous exudates and rare hematoxylin
bodies.
Several early studies revealed that reduced levels of
hemolytic complement, C1q, C4, C3 in pleural fluid from
lupus patient when compared to pleural effusions from
patients with cancer, heart failure, and other conditions.
This compliment level remained low even after

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adjustment for the total protein content of the pleural

fluid. However, low pleural fluid complement levels are
not specific for SLE and may occur in patients with RA
or empyema.
There is some evidence that low pleural fluid
compliment levels in SLE results from activation of
compliment cascade by immune complexes. Thus
conversions products that are generated by activation
of the complement cascade are present in SLE pleural
fluid, and immune complexes abound in SLE pleural
fluid. Furthermore perivascular deposition of
immunoglobulins and compliment components in the
parietal pleura have been found in patients with lupus
pleuritis. The nature of the immune complexes in SLE
pleural fluid is not clear, though they may well be DNAanti-DNA complexes.
Study conducted by Andrew BS, Arora NS, Shadforth
MF et al showed that in RA the process occurring in the
pleural space appears to be analogous to the joint whereas
the mechanism proposed to explain the pathogenesis of
malignant effusion includes 1) pleural implantation of
tumor cell, 2) obstruction of pleural lymphatic by tumor
cells, 3) increases pleural fluid protein which may impair
the absorption of the protein by visceral pleural
lymphatic and atelectasis leading to reduction of pleural
pressure. An immunopathogenic basis for malignant
pleural effusion has not been considered in there study.
They showed that the degree of immune complexes was
higher in the serum than in the pleural fluid in patients
with malignant diseases, the converse was true in
patients with connective tissue diseases. Activation of
C3 and properdin factor B was almost invariable in
pleural fluid from patients with connective tissue
diseases and bacterial infection.
None of the immunologic abnormalities that are
described in pleural fluid is diagnostic of lupus
pleuritis, thus the presence of immune complexes,
complement activation products, and immune deposit
in the parital pleurae all have been reported in pleurisy
associated with other rheumatic diseases, such as RA
as well as in non-rheumatic conditions, including cancer
and empyema. This suggests that an immune mediated

mechanism is a common pathway by which SLE and

other diseases cause pleurisy.
In SLE, the pleural fluid differential cell count varies
from predominantly polymorphoneuclear (PMN) to
mononuclear depending on the time of thoracocentesis
and onset of effusion. In SLE characteristics of pleural
fluid are : pH > 7.30, with glucose level >60mg/dl, and
LDH never exit more than 600 U/L. When pleural fluid
ANA >1:160 with pleural fluid / serum (PF/S) ANA
ratio is more than 1, the diagnosis of lupus is likely.6
In most of the patients, the pleural effusion resolves
with oral or intrapleural corticosteroid administration.
In our case the patient presented with recurrent
exudative pleural effusion, initially which was unilateral
and latter bilateral. The tempo of the disease was waxing
and waning in nature. When she was admitted in our
institute she had moderate bilateral pleural effusion with
mild pericardial effusion without any systemic
manifestations of SLE, but investigations (pleural fluid
and, serum ANA, serum anti dsDNA, pleural fluid cell
type, pH, LDH level) supported a diagnosis of lupus.
With steroid she responded dramatically. She will
need prolonged follow-up to ascertain whether she
develops other clinical features of lupus.

REFERENCES
1.

Orens JB, Matrinez FJ, Lynch JP III. Pleuropulmonary

manifestation of systemic lupus erythematosus. Rheum Dis
Clin North Am 1994;20:159-93.

2.

Keane MP, Lynch JP III. Pleuropulmonary manifestation of

systemic lupus erythematosus. Thorax 2000;55:159-66.

3.

Harvey AM, Shulman LE, Tumulty PA, et al. Systemic lupus

erythematosus: review of the literature and clinical analysis
of 138 cases. Medicine 1954;33:291-437.

4.

Boures D, Panagou P, Papandreou L, et al. Massive bilateral

pleural effusion as the only first presentation of systemic
lupus erythematosus. Respiration 1992; 59:173-5.

5.

Pettersson T, Soderbolom T, Nyberg P, et al. Pleural fluid

soluble Interleukin 2 receptors in rheumatoid arthritis and
systemic lupus erythematosus. J Rheumatol 1994;21:1820-4.

6.

Good JT Jr, King TE, Antony VB, et al. Lupus pleuritis,

clinical features and pleural fluid characteristics with special
reference to pleural fluid anti-nuclear antibody. Chest
1983;84:714-18.

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