Dear all About 5%-8% of cases with SCLS known as "chronic", or "active", In description they seem a distinct clinical picture, behave different in pattern, (chronic behavior, often no MGUS, more often trunk+face involved, however chronic invalidating). And combination of vast attacks and chronic pattern seems also to occur in some. Discussion is relevant whether it is the same disease, another disease, or a disease-variant. And still it is important not to mix-up with all other disease with edema due to other reasons. Last year an investigation was documented in which cardiac MRI of people with chronic active SCLS (N=6) had quite similar cardiac abnormalities (interpretation: intra cardiac extra cellular edema) compared with SCLS patients during acute attacks.However it was not found in people wo did not suffer of an attack in the months before nor having a chronic active form. see: http://www.jcmr-online.com/content/pdf/s12968-015-0181-6.pdf The article stated below also describes cardiac edema in SCLS. Clinical and Pathological Findings of a Fatal Systemic Capillary Leak Syndrome (Clarkson Disease) A Case Report Medicine

Volume 94, Number 9, March 2015 It still does't prove the disease mechanism is identical, but give hope of a possible extra diagnostic instrument in comparable cases, and maybe a clue in the search for a clue of SCLS. Hiltjo

Hello again Hiltjo, With my first and most severe attack, I had bedside echo- in ED. This demonstrated a Bezold-Jarisch syndrome. Basically an empty left ventricle due to acute hypovolemic shock, with a paradoxical severe bradycardia. (35 alternating 90). This may have been a reflex vagal effect, but may also be a conduction defect with myocardial oedema? Just food for thought. Importantly, the sequence bears out your highlight of the ' systemic' component. The pulmonary oedema was very severe and pre-dated any IV. There was no peripheral oedema until after the resorption phase. I wonder whether the tendency to central oedema (pulmonary, pericardial, cerebral) occurs in more physically active people? The more active physically, the more you 'pump' your extremities? This could explain a spectrum rather than two dustinct diseases. Of course this is confounded by the other cause of oedema..viz. overload. John