Therapeutic options for refractory massive pleural effusion in systemic lupus erythematosus: A case study and review of the literature

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Objectives

Massive refractory pleural effusions are uncommon in patients with systemic lupus erythematosus. Describing such a patient, the literature was reviewed to report the various therapeutic options in such cases.

Methods

MEDLINE search using the terms “lupus” and “pleural effusion,” inclusion of cases with refractory massive effusions with emphasis on treatment.

Results

Only 10 such cases (including the patient described here) were reported in the English literature over the past 25 years. Those 10 patients suffered symptoms related to pleural effusion for a long period of time until resolution, ranging between 2 months to 2.5 years (median 6 months). During that period of time they underwent multiple fluid aspirations. Seven different types of therapy were reported in these case descriptions. They can be divided into 2 major groups: systemic therapy (immunosuppressive therapy, plasmapheresis, and intravenous immunoglobulin) and local therapy (intrapleural steroid injections, pleurodesis with talc or tetracycline, and pleurectomy). Pleurodesis with talc seemed to be the most effective treatment modality.

Conclusions

Due to the small number of reported patients, the best type of intervention is uncertain. When refractory pleural effusion is part of lupus exacerbation, the treatment of choice would be systemic, such as immunosuppressive therapy with high-dose steroids and cyclophosphamide. Intravenous immunoglobulin may also be considered. Local measures such as talc pleurodesis should be employed if systemic measures fail, or when pleural effusion is the only manifestation of lupus.

Section snippets

Methods

We describe a patient with recurrent massive pleural effusion, refractory to therapy with high-dose steroids and AZA. The English literature of the last 25 years was reviewed using a MEDLINE search with the terms “lupus” and “pleural effusion.” Cases with refractory (ie, not responsive to steroid therapy) massive effusions were included. The various therapeutic options in cases with recurrent massive pleural effusions refractory to steroid therapy are described in detail.

Report of a case

A 25-year-old woman with SLE was admitted with shortness of breath, gradually increasing in severity over the past few weeks. She had intermittent pleuritic-type left-sided chest pain. She was diagnosed with SLE 3 years previously, presenting with arthritis in the proximal interphalangeal (PIP) joints, knees, and ankles, skin rash on her face and extremities, severe myalgia, and fever. At that time, her ANA test (Euroimmune AG, Germany) was positive with a titer of 1:640, anti-dsDNA was

Discussion

This patient represents an uncommon scenario of SLE-related pleuritis associated with large pleural effusion, refractory to “first-line” immunosuppressive therapy with prednisone and AZA. Reviewing the English-language literature of the past 25 years, we found detailed descriptions of only 7 similar cases (8, 9, 10, 11, 12, 13, 14, 15) and a limited description of 2 other cases (16). All 10 cases are summarized in Table 1. The paucity of cases suggests that this phenomenon of refractory massive

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