LETTER TO THE EDITOR

Generalized subcutaneous edema and polyserositis asunusual presentation in systemic lupus erythematosus

Dear Editor,

Systemic lupus erythematosus (SLE) is an autoimmune

multisystem disease with variable presentations. Poly-

serositis and subcutaneous edema are common mani-

festations of SLE. They are generally considered to be

associated with severe nephritic syndrome, constrictive

pericarditis, congestive heart failure, portal hyperten-

sion, malignancy, and/or pleural infection. However,

generalized subcutaneous edema and polyserositis as

main presentations of SLE, without the above-men-

tioned factors, is rare. Here we report an old woman

with generalized subcutaneous edema and polyserosi-

tis as the first manifestation. To the best of our knowl-

edge, such a case has only been reported once in the

literature.

A 68-year-old previously healthy woman was

referred to our clinic in April 2010 for a 2-month his-

tory of progressive edema starting in the lower limbs.

When admitted, she had severe generalized edema,

including face, abdomen and extremities. She had

gained 5 kg in weight over 2 months. She complained

of abdominal distension and dyspnea. A review of sys-

tems was negative for rheumatologic symptoms,

including malar rash, photosensitivity, arthritis, Ray-

naud’s phenomenon, myasthenia and fever. Vital signs

such as blood pressure, pulse and respiration were

normal.

Laboratory investigations showed increased erythro-

cyte sedimentation rate, 55 mm/h (normal value,

20 mm/h). Total protein and albumin were 6.7 g/dL

and 3.1 g/dL (normal range 4.6–8.3 and 3.5–5.2 g/dL),

respectively. The full blood count revealed hemoglobin

at 8.6 g/dL, platelet count 47 · 109/L. An antibody

screening showed antinuclear antibodies (ANA) >

1 : 1000 (normal < 1 : 100). Anti-cardiolipin antibod-

ies (ACL), anti-U1 ribonucleoprotein and anti-Ro/SSA

(Sjorgren’s syndrome antigen A) were positive. The

rheumatological profile revealed hypocomplementemia

with C3 at 0.734 mg/dL (normal 0.79–1.8 mg/dL).

Rheumatoid factor was positive. The urine analysis

showed protein urine (�), and a 24-h urinary protein

excretion was 0.93 g/24 h (2500 mL). Serum creatinine

and urea nitrogen were in normal ranges. Thyroid func-

tion was normal. A tuberculin skin test (Mantoux) was

used and the induration was 5 mm.

Computed tomography (CT) scan of the chest

revealed massive bilateral pleural effusion and pericar-

dial effusion, multiple lymph nodes in bilateral axil-

lary and mediastinal areas (Fig. 1a). CT scan of the

abdomen showed mild ascites and widespread subcu-

taneous edema (Fig. 1b). Echocardiography demon-

strated moderate to massive pericardial effusion. To

rule out lymphoma, a biopsy of the lymph node from

the left axillary area was performed. The pathology

showed inflammation.

Systemic lupus erythematosus was diagnosed based

on 5/11 American Rheumatism Association (ARA) cri-

teria for SLE (thrombocytopenia, polyserositis, nephri-

tis, ANA and ACL positivity). The patient was treated

by high dose methylprednisolone and cyclophospha-

mide. After this treatment, the patient’s condition

improved significantly: generalized edema disappeared

within 3 weeks. The patient remained without any

signs of lupus activity (Fig. 2).

DISCUSSION

Generalized subcutaneous edema in SLE is generally

considered to be associated with severe nephritic syn-

drome and congestive heart failure. However, these

factors were not found in our case except for mild pro-

teinuria and slightly low hypoalbuminemia, which

could not completely explain such severe anasarca.

The reason for general edema in this case still needs

further investigation. To the best of the authors’

knowledge, similar manifestations have only been

reported in one case. Aslan et al. described a case of a

13-year-old boy who presented with general edema

International Journal of Rheumatic Diseases 2011

ª 2011 The AuthorsInternational Journal of Rheumatic Diseasesª 2011 Asia Pacific League of Associations for Rheumatology and Blackwell Publishing Asia Pty Ltd

without contributing factors and bilateral massive

pleural effusion. SLE was diagnosed and he responded

well to corticosteroids with resolution of the pleural

effusions and edema.1

Polyserositis is also a common clinical manifesta-

tion of SLE. Simultaneous occurrence of serositis at

two or more sites in SLE was present in about 35% of

Chinese SLE patients.2 However, massive bilateral

pleural and pericardial effusions, as presenting mani-

festations, have rarely been reported. Differential diag-

noses of SLE-related pleural and pericardial effusion

includes nephrotic syndrome, heart failure, pulmonary

embolism, uremia, viral or bacterial infection, tubercu-

lous, myocardial infarction, neoplasms and trauma.3,4

However, none of these conditions were found in our

case. As generalized subcutaneous edema and polyse-

rositis were the main presentations in this case, both

being accumulations of excessive fluid outside the vas-

cular system, we speculated that the pathogenesis of

these two were same. So we made the following

assumptions. (i) Immune complex deposition in the

microvasculature and the change of vascular perme-

ability plays a role in pathogenesis of subcutaneous

edema and polyserositis. (ii) The patient had mild

hypoalbuminemia secondary to proteinuria and this

might also contribute to the development of edema

and effusions. In this case, polyserositis together with

generalized edema, are therefore probably reflections

of lupus activity rather than being causal.

Another interesting aspect in our patient is her age.

SLE is uncommon after the age of 50 years and may

be a specific subgroup.5–8 It has been reported that

late-onset SLE may have a more insidious start and

non-specific manifestations, with a higher occurrence

of serositis, pulmonary involvement; and a lower

occurrence of skin manifestations, photosensitivity,

arthritis and nephritis. Rheumatoid factor positivity

was more frequent.6 The clinical course is considered

to be more benign, with fewer degrees of disease activ-

ity.9 In our case, the patient’s condition seemed to be

consistent with the literature.

In conclusion, the possibility of SLE-related general-

ized edema and polyserositis should be kept in mind,

especially in elderly patients, even without other clini-

cal manifestations of the disease.

DISCLOSURE/FUNDING

The authors have nothing to disclose. No funding sup-

port was received for this study.Figure 2 After treatment, the patient’s condition improvedsignificantly and generalized edema had disappeared.

(a)

(b)

Figure 1 Computed tomography (CT) scan of the chestshowing massive bilateral pleural effusion and pericardialeffusion, multiple lymph nodes in bilateral axillary andmediastinal areas (a) and of the abdomen showing mildascites and widespread subcutaneous edema (b).

Letter to the Editor

2 International Journal of Rheumatic Diseases 2011

Le LU, Zhiming ZHAO and Hui CAI

Department of Integrative Medicine, Jingling Hospital,

Nanjing University School of Medicine, Nanjing, Jiangsu

Province, China

Correspondence: H. Cai,

email: njzy_caihui@163.com

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6 Font J, Pallareas L, Cervera R, et al. (1991) Systemic lupus

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(Baltimore) 83, 348–59.

8 Padovan M, Govoni M, Castellino G, et al. (2007) Late onset

systemic lupus erythematosus: no substantial differences

using different cut-off ages. Rheumatol Int 27, 735–41.

9 Formiga F, Moga I, Pac M, Mitjavila F, Rivera A, Pujol R

(1999) Mild presentation of systemic lupus erythematosus

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Letter to the Editor

International Journal of Rheumatic Diseases 2011 3