What this articles addresses, e.g., the differential diagnosis of CAPS, is something I brought up a long time ago, especially at the Galveston APS meeting a few years ago. In this regard, when talking about the longstanding CAPS registry, how do we know if all those patients actually had CAPS versus some of the other entities, e.g., HU syndrome, TTP, underlying infections, malignancies, HIT, etc? Antiphospholipid antibodies have been reported in the presence of infection, and I can easily imagine a patient in a critical care unit developing septic shock with positive antiphospholipid antibodies, not necessarily having CAPS in this setting.
Article Catastrophic APS in the Context of Other Thrombotic Microangiopathies
Emilio, these patients in a critical care unit who develop septic shock with positive antiphospholipid antibodies, do they also test positive for B2GPI and Lupus Anticoagulant?
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Thank you, Ricardo. If the lupus anticoagulant (LAC) and anti-B2GPI antibodies are positive, they will help in the diagnosis of course. However, often these sick ICU patients are placed on prophylactic or therapeutic heparin, and then, doing the LAC can be misleading, as you know, since the presence of anticoagulants gives rise to a false positive LAC. Dr. Silvia Pierangeli had a way to adjust the lab tests in these situations; however, most hospital labs won't do that, and they just report the LAC as is, often misleading to intensivists who are unaware of this problem, leading perhaps to a misdiagnosis of CAPS. Even flushing the line with a few drops of heparin is enough to throw off the LAC test. I think the patients most likely to have an accurate diagnosis of CAPS would be those well known lupus patients with known a priori positive antiphospholipid antibodies who for whatever reason when they decompensate and/or get infected, begin to thrombose in different areas. Otherwise, I think a number of other entities such as TTP, hemolytic uremic syndrome, DIC, and other consumptive coagulopathies may well be misdiagnosed as CAPS only because positive antiphospholipid antibodies are present. That's the problem I have with a CAPS diagnosis, and why I am somewhat skeptical of this diagnosis. After many years as a rheumatologist taking care of lupus patients, I may have seen maybe 1-2 patients with bona fide CAPS, if that. CAPS must be quite rare indeed.
This is quite debated and confusing to detect living CAPS. According to the citeria of APS, either LA and/or aCL and/or B2GP1 should be present (12 week apart 2 positive results). Clinically the patient should present thrombotic events (stroke, myocardial infarction, gangrene, pulmonary embolism or DVT) or recurrent miscarriages. However in case of CAPS, kidney and liver function might be disturbed. The major cause of CAPS death is multiple organ failure. APTT might be consistantly significantly high even after regulated warfarin dosages. Please share your experiences as well as this question was posted 1 year back.
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