Dados do Trabalho

Title

SHRINKING LUNG SYNDROME- RARE MANIFESTATION IN SYSTEMIC LUPUS ERITEMATOSUS: CASE REPORT

Background

Systemic lupus erythematosus (SLE) is an autoimmune disease that can present with manifestations in the lung, causing pleuritis, interstitial disease, pulmonary hypertension or pneumonitis. Rarely, dyspnea, chest pain, and changes in lung function tests will appear without major changes in lung parenchyma on chest tomography. When this occurs in the individual with SLE, this presentation is called Shrinking Lung Syndrome.

Case report

Males, 77 years old, with SLE (Criteria SLICC: FAN reagent, hemolytic anemia, lymphopenia and antiphospholipid antibody present) and laboratory SAAF: anticardiolipin IGM positive and B2 glycoprotein I IGM positive), using AAS 100 mg / day, azathioprine 150 mg / day, presented dyspnea on moderate exertion, without cough, fever or edema in the lower limbs, for 1 year and 6 months. Angio CT of thorax: no evidence of pulmonary thromboembolism, with presence of elevation of the right diaphragmatic dome.
Transthoracic echocardiogram: ejection fraction of 70% PSAP: 37mmHg, with mild grade tricuspid insufficiency and mild degree aortic root dilatation. Plethysmography: Restrictive respiratory disorder in moderate degree, with reduced diffusion in a marked degree. Chest CT: Signs suggestive of bilateral diaphragmatic paralysis accompanied by restrictive atelectasis of the adjacent parenchyma, without stigmas of interstitial disease. In this case, corticotherapy was started, still recent for an evaluation.

Conclusion

While this pulmonary presentation is rare in patients with SLE, it is important to be investigated in individuals with symptoms of dyspnea, without changes such as: anemia, heart failure or interstitial disease. Although there is no specific treatment, xanthine, beta-agonists, corticosteroids and immunosuppressants can be initiated empirically.