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Chronic systemic capillary leak syndrome: a case responding to prednisolone treatment

Systemic capillary leak syndrome (SCLS) was first described in 1960 (1). It is a rare condition of unknown aetiology characterized by recurrent episodes of acutely developed hypovolaemic shock due to fast leakage of plasma to the extravascular compartments. It is accompanied by haemoconcentration, hypoalbuminaemia, oedema, and frequently a monoclonal gammopathy (2, 3). Treatment has primarily been symptomatic with terbutaline and theofyllamine (2). Only a few patients with chronic SCLS have been described (4 6). These patients had chronic weight gain, pleural and pericardial effusions, mild hypoalbuminaemia, haemoconcentration, and an M-component. We describe the case of a 45-year-old previously healthy farmer who presented with arthralgia, night sweats, feeling of uneasiness, and fatigue. He had weight gain of 25 kg and developed generalized oedema over a period of 3 weeks. Without a specific diagnosis he was prescribed 40 mg prednisolone daily at his local hospital. He had a remarkable response, losing 5 kg the first day. Over a period of 2 months, the prednisolone was tapered to 5 mg leading to a relapse of symptoms and a weight gain of 9 kg in 6 days. Prednisolone was increased to 40 mg followed by tapering to 15 mg without relapses. He was referred to the university hospital after 9 months disease duration. Prednisolone was again reduced to 5 10 mg followed by a prompt relapse of symptoms, including oedema and weight gain of approximately 1.5 kg/day. Three years after the start of symptoms, he still needed prednisolone 10 mg/day, and azathioprine 150 mg/day was added after the first year to reduce the prednisolone dose. Terbutaline treatment was tried during the course, but had to be abandoned after a few days because of tachycardia and uneasiness. Physical examination showed oedema of the extremities but was otherwise normal. Computed tomography (CT) scan of the abdomen revealed ascites. Albumin was slightly decreased to 34 g/L (normal range 40 51 g/L). Blood pressure was 130/90 mmHg. He had normal/negative C-reactive protein, haemoglobin, plasma M-component, urine light chains, liver enzymes, hepatitis serology, creatinine clearance, urinary protein excretion, anti-nuclear antibodies, rheumatoid factor, anti-cyclic citrullinated peptide (anti-CCP) antibodies, anti-neutrophil cytoplasmic antibody (ANCA), and C3, C4 and C1 esterase inhibitors. A bone scan, spirometry, diffusion capacity, and bone marrow biopsy were also normal. He had negative tests for several infectious diseases. However, 13 months after debut he had a positive test for Q-fever, although previous tests had been inconclusive. A confirmative test in Marseille was positive as well. He was treated with doxycycline for 9 months, with no effect on his symptoms. He had a left bundle branch block and asynchroneous contraction as visualized by echocardiography. Ejection fraction was initially normal, but decreased to 35% during the course of the disease with New York Heart Association (NYHA) class I. Coronary angiography and biopsies from the right atrium were normal without signs of ischaemic heart disease, amyloidosis, or sarcoidosis. For heart disease he was treated with beta blocker, angiotensin-converting enzyme (ACE) inhibitor, and loop diuretics without any effect on his symptoms or weight gain. Extensive investigations revealed no kidney failure, liver failure, or lung disease including pulmonary hypertension. The presence of oedema, chronic weight gain, and hypoalbuminaemia suggested a case of chronic SCLS. No M-component was found, but this is not essential for the diagnosis (2). A diagnosis of Q-fever was made after 13 months. In the beginning, tests for this disease were inconclusive. However, it was possible that Q-fever led to chronic SCLS in our patient. There have been no previous published reports on this possible causality. The recurrent oedema could not be explained by the mild heart disease because oedema and weight gain were only relieved by prednisolone treatment. Previously described cases of chronic SCLS also had a good response to prednisolone treatment, but it is unclear whether it can be discontinued (4 6). Evidence is scarce, but it might be that, by blocking cytokines, prednisolone elicits its effect in chronic SCLS (7). 400 Letters