A Very Rare Cause of Pleuritic Chest Pain: Bilateral Pleuritis as a First Sign of Familial Mediterranean Fever

The familial Mediterranean fever (FMF), also called recurrent polyserositis, is characterized by reccurrent episodes of serositis at pleura, peritoneum, and synovial membrane and fever. We present a patient with recurrent bilateral pleural effusion due to serositis attacks as a first sign of FMF. A 59-year-old Turkish man suffered from recurrent pleuritic chest pain due to pleural effusion and atelectasis. The etiology was not found, and his symptoms were spontaneously recovered during several weeks. The pleuritic chest pain was associated with abdominal pain in the last attack. The gene mutation analysis revealed the homozygosity of FMF (F479L) gene mutation in both our patient and his grandchild. After the colchicine treatment, the attack has not developed. In conclusion, recurrent pleural effusion and pleuritic chest pain may be the first signs of the FMF.


Introduction
e familial Mediterranean fever (FMF), an autosomal recessive condition, affects more than one hundred thousand people worldwide and, as such, is the most common of the hereditary periodic fevers [1]. e FMF has affected mainly Mediterranean populations including non-Ashkenazi Jews, Arabs, Turks, and Armenians. It is characterized chie�y by short and periodic attacks of fever and serositis involving the pleura, peritoneum, synovial membrane, and tunica vaginalis [2]. Pulmonary involvements of FMF because of in�ammation of pleura were reported by 30�40% of patients. ey are usually present with unilateral pleuritis and fever [3,4]. We present a patient with recurrent bilateral pleural effusion due to serositis attacks as a �rst sign of FMF.

Case Presentation
A 59-year-old Turkish man was admitted to the hospital with pleuritic chest pain on right hemithorax, dyspnea, cough, and fever (38.5 ∘ C). Physical examination showed the decreased breath sound and pleural frotman with auscultation. e chest radiography also showed pleural effusion with linear atelectasis on right side lung ( Figure 1). orax CT was performed and this showed more right sided bilateral pleural effusion and linear atelectasis ( Figure  2).
Laboratory �ndings demonstrated the leukocytosis (favor to polymorphonuclear cell) and increased erythrocyte sedimentation rate (69 mm/h). e patient was treated with antibiotic (cefuroxime and clarithromycin) for pneumonia and pleuritis but did not improve. e lung ventilationperfusion scintigraphy was performed for pulmonary thromboembolism, and it de�ned the match perfussion defect. e complaints of patient were recovered by reducing aer several weeks. Aer the �rst attack, he suffered from recurrent symptoms as in �rst attack within four years. In 2008, the patient was admitted to hospital with chest and abdominal pain. Chest radiography showed pleural effusion and atelectasis. When we evaluated the �ndings of patient comparing with previous �ndings, these symptoms may be caused from FMF. Colchicine was started for treatment as dose of 1 mg/day. His symptoms were improved. Colchicine dose was increased to 1.5 mg/day. Aer the treatment, attack has not developed ( Figure 3).  Family history was asked to con�rm the FMF. �e learned that his grandchild has recurrent abdominal pain and suspected for FMF. MEFV (the gene responsible for FMF) gene mutation was studied to con�rm the diagnosis. e gene mutation analysis revealed the homozygosity of FMF (F479L) gene mutation in both our patient and his grandchild.

Discussion
FMF, also called reccurrent polyserositis, was characterized with reccurrent episodes of serositis at pleura, peritoneum, and synovial membrane and fever [5,6]. e initial attack usually occurs before twenty years old and is typically dominated by peritoneal symptoms and signs. e initial attack is characterized by pleuritic chest pain and fever in fewer than 10% of patients like our case, but approximately 40% have an attack of febrile pleurisy during the course of their disease. ere were no symptoms except pleuritic chest pain in our patient. Livneh and Langevitz described the diagnostic criteria of FMF [7]. According to these criteria, unilateral pleuritis is one of the major criteria of FMF. But, bilateral pleuritic attack was the �rst sign of our patients. According to F 3: Northwest radiography showing the normal chest radiograph aer the treatment.
our knowledge, this case is the �rst case report with bilateral pleuritis and the �rst sign of FMF. Chest radiographs during the acute pleuritic attacks show the elevation of the ipsilateral diaphragm and frequently small pleural effusions. In our patient, the chest radiography showed unilateral pleural effusion, but computed tomography revealed the bilateral pleural effusion. e attacks are reccurrent, with irregular intervals of days to months between the attacks. Because the administration of colchicine decreases the frequency of the attacks [8], there were no attacks in our patients aer the colchicine treatment.
In conclusion, recurrent pleural effusion and pleuritic chest pain may be the �rst signs of the FMF. Also, FMF attacks may be one of the reasons of the bilateral plural effusions. For patients who have unexplained bilateral pleural effusion and chest pain with fever, FMF should be keep in mind, especially in the Mediterranean region.
�on�ic� of �n�eres�s ere is no actual or potential con�ict of interests for us, the authors.