Case Presentation: A 66 year old female with a past medical history of hypertension, heart failure and atrial fibrillation presented with severe lumbar back pain that began 2 weeks prior. She was writhing in pain and described the pain as throbbing, rating it a 10/10. The pain radiated down her thighs but spared her calves and feet. She was afebrile with a BP of 93/67 mmHg, and heart rate was 111 bpm with a regular rhythm. A CT of the lumbar spine revealed multilevel degenerative disease with central disc herniation at L4-L5 resulting in severe central stenosis. On day 2, she developed AKI and epigastric pain. Despite aggressive hydration and albumin infusion, her blood pressure remained low. Over the next few days, her kidney function worsened and labs revealed fulminant liver failure. Due to hyponatremia, hyperkalemia and hypoglycemia she was placed on stress dose steroids. She developed bilateral lower extremity edema, swelling of the face, and severe metabolic acidosis. Despite aggressive management, she developed acute hypoxic respiratory failure and hypotension with a blood pressure of 66/41 mmHg, despite vasopressor support. She expired ten days after admission.
Discussion: Systemic capillary leak syndrome (SCLS), also known as Clarkson’s disease, is a rare and potentially fatal condition with less than 300 cases documented. The pathogenesis is weakness of the vascular membrane resulting in seepage of proteins and fluid from intravascular spaces into interstitial spaces. The clinical course is characterized by the triad of hypotension, hypoalbuminemia and hemoconcentration. The main challenge in management of systemic capillary leak syndrome is the diagnosis rather than the treatment. It can be difficult to distinguish from sepsis, anaphylaxis, toxic shock syndrome and drug reactions. Several theories have been proposed to explain the pathogenesis. These include abnormalities in VEG-F and angiopoietin-2, endothelial cell apoptosis, the involvement of IL-2, and inflammatory mediators such as leukotrienes or TNF-alpha. Three phases have been identified during an attack of SCLS. The first phase consists of prodromal symptoms, which can manifest as fatigue, edema, abdominal pain and myalgias of the extremities. The fluid extravasation phase develops between 1-4 days later when the classic triad presents. The final phase, the recovery phase, which can occur quickly with extravasated fluids being recruited into intravascular spaces and result in pulmonary edema. Prodromal symptoms and their triggers have not been clearly defined. Symptoms such as intense headache and abdominal pain have been reported. This case is unusual because of the presenting prodromal symptom of severe lumbar pain which has not been reported prior to the onset of systemic capillary leak syndrome. The patient did eventually develop severe abdominal pain during the extravasation phase of the syndrome. The response to treatment has significant variation and generally depends on how early the diagnosis has been made. Patients with a delay in diagnosis or serious complications such as rhabdomyolysis, renal failure, pericardial effusions, pleural effusions and multi-organ failure have a very poor prognosis as was the case with our patient.
Conclusions: SCLS is a rare disease, and prodromal symptoms can easily be missed by clinicians because the condition can easily resemble septic shock. The patient presentation may be severe and an opportunity may be missed to provide early support and avoid the development of serious complications.