CASE REPORT
Shrinking lung syndrome in systemic lupus erythematosus*
CÍNTIA ANDRADE COSTA, DÁLVARO OLIVEIRA DE CASTRO JR.,
SÉRGIO JEZLER
(TE SBPT), MITTERMAYER SANTIAGO
Systemic lupus erythematosus (SLE) may involve the respiratory tract in several ways, such as through pleuritis, pneumonitis, interstitial disease or pulmonary hypertension. In rare cases, SLE patients present a syndrome characterized by dyspnea, chest pain and abnormalities in pulmonary function testing, although there may be no evidence of major parenchymal lung disease on computed tomography scans. This condition has come to be known as shrinking lung syndrome. We report a case that meets these diagnostic criteria, emphasizing the pathogenesis proposed, as well as the therapeutic options available.
Key words: Lupus Erythematosus/Systemic, Respiratory System, Shrinking Lung Syndrome
* S t u d y c a r r i e d o u t i n t h e R h e u m a t o l o g y D e p a r t m e n t o f t h e H o s p i t a l S a n t a I z a b e l ( H S I ) / E s c o l a B a h i a n a d e M e d i c i n a e S a úd e P úb l i c a ( E B M S P )
C o r r e s p o n d e n c e t o : M i t t e r m a y e r S a n t i a g o . N úc l e o d e R e u m a t o l o g i a d o H o s p i t a l S a n t a I z a b e l : P r a ç a A l m e i d a C o u t o 5 0 0 , N a z a r é , S a l v a d o r , B a h i a . C E P 4 0 0 0 0 - 0 0 0 . E - m a i l : m i t t e r @ s v n . c o m . b r
S u b m i t t e d : 1 2 M a y 2 0 0 3 . A c c e p t e d , a f t e r r e v i s i o n : 3 S e p t e m b e r 2 0 0 3 .
Abbreviations used in this paper:
SLE — Systemic lupus erythematosus SLS — Shrinking lung syndrome
INTRODUCTION
Systemic lupus erythematosus (SLE) is a diffuse disease that affects conjunctive tissues and presents innumerable clinical manifestations. More than 50% of patients present respiratory involvement at some stage of the progression of the disease. It may affect the upper airways, pleura, parenchyma and pulmonary vessels. A rare manifestation of the disease is called shrinking lung syndrome (SLS), characterized by the dysfunction of respiratory muscles, especially the diaphragm, by mechanisms not yet well defined, resulting in dyspnea.1
We report a case of SLE with respiratory manifestations, whose investigation led to a diagnosis of SLS. We discuss pathogenic, diagnostic, and therapeutic aspects of this syndrome. We also conducted a review of the literature on this topic.
CASE REPORT
positive results for lens epithelial cells, negative results for native anti-DNA and rheumatoid factor, and normal results for C3 and C4. Testing for anticardiolipin antibodies using ELISA was negative, as was testing for anti-Ro/SSA antibodies using counter-electrophoresis. Chest X-ray revealed elevated hemidiaphragm, reduced lung fields, and laminar atelectasis (Figure 1). Atelectasis was confirmed by high-resolution computer tomography, which showed no interstitial involvement (Figure 2). Arterial blood gas tests were normal and spirometry revealed moderate restrictive ventilatory distress, with forced vital capacity of 58%, maximal inspiratory pressure at —40 cm H2O and maximal expiratory pressure at +60 cm H2O. Echocardiogram revealed mild pericardial effusion, mitral
regurgitation and mild pulmonary hypertension (38 mmHg). The patient was treated with 20 mg of prednisone combined with pulse therapy cyclophosphamide (1 g/month) for 6 months. Although there was partial improvement of dyspnea, radiographic and spirometric characteristics remained unchanged (Table 1).
DISCUSSION
The appearance of SLS is a very rare clinical condition that is classically described in patients diagnosed with SLE2 and seldom seen in patients suffering from other diseases3. It
is characterized by dyspnea, chest pain upon deep breathing, and disproportionality between alterations seen in pulmonary function test results (which revealed restrictive, occasionally severe, respiratory distress) and radiographic findings (which showed only unilateral or bilateral elevated hemidiaphragm and an absence of parenchymal involvement). All of these characteristics were present in our patient, and a diagnosis of SLS was therefore made.
Using MEDLINE, Warrington et al.2 reviewed data in the literature published between
1965 and 1977 and found 49 well-documented cases of SLE-related SLS. Mean age of patients was 40, and the proportion of females and males was 5.5 to 1. Curiously, 20% of the reported cases presented some evidence of myopathy. Using the same database, we reviewed the literature from 1977 until the present and identified another 11 cases of SLS in patients with SLE.1 , 4 , 5 , 6 , 7. If we include another case reported in a non-indexed journal8
and the present case, the total number of reported cases comes to 62.
The pathogenesis of SLS is under debate. Some suggest that it results primarily from a dysfunction of the diaphragm muscles9, but other authors do not accept this hypothesis1 0.
Other hypotheses include diaphragmatic paralysis secondary to phrenic nerve injury6,
diffuse fibrosis in the diaphragm1 1 and limited chest wall expansion1 0. It has been
proposed, although not very convincingly, that autoantibodies such as anti-phospholipids1 2
and anti-Ro/SSA7 antibodies participate in SLS pathogenesis. In the present case, tests for
these antibodies were negative.
Another complicating factor is that treatment for SLS has not been well defined. In the cases described in the literature, several pharmacological agents have been used to treat the condition. These include corticosteroids1 3 , 1 4, immunosuppressants1, inhaled
beta-agonists (which have a positive inotropic on beta-receptors in the diaphragm muscle)1 5,
xanthines1 6, and even digitalis (based on the argument that diaphragm responds to this
drug similarly to the heart muscle)8.
In the present case, clinical improvement was achieved by increasing the prednisone dosage from 5 mg to 20 mg, although there was no improvement in spirometric or radiographic parameters. The use of cyclophosphamide was an attempt to revert pulmonary hypertension, as was suggested in a recent study1 7. As a matter of fact, a second
echocardiogram performed after the sixth month of pulse therapy revealed no signs of pulmonary hypertension. Therefore, the clinical improvement seen may be attributable to the reduction in pulmonary hypertension and not to reversion of the SLS.
of xanthines, beta-agonists, corticosteroids, or even immunosuppressants may be beneficial.
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TABLE 1
Evolution of pulmonary function (simple spirometry) before and after treatment
Before After
FVC (liters) 1.27 1.35
FVC (%) 56 58
FEV1 (liters) 1.13 1.25
FEV1 (%) 56 62
FEV1/FVC (%) 88 92
