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In their report Yoo et al describe a 72 year old woman who presented with fever and symmetrical gangrene of the fingers and toes despite intact peripheral arterial pulses.1Klebsiella pneumoniae grew both from blood and liver biopsy aspirates. Treatment with antibiotics, prostacyclin, tissue plasminogen activator followed by warfarin and aspirin was beneficial and led to the regression of almost all the lesions. The authors state that “Although Amital et al reported that amputation could induce remission of the systemic illness, some reports have recommended that non-surgical management is preferable if there is an intact pulse in the affected limb”.
The term “catastrophic” antiphospholipid syndrome (CAPS) is used to define an accelerated form of antiphospholipid syndrome (APS) resulting in multiorgan failure. These patients have clinical evidence of multiple organ involvement, which develops within a short period of time, in addition to histopathological evidence of multiple small vessel thrombotic occlusion and high titres of antiphospholipid antibodies. The most common precipitating factor of CAPS is infection, appearing in 35% of patients.2
We previously described two patients with APS with severe leg infections who developed a fulminant course of CAPS with acute renal failure, adult respiratory distress syndrome, central nervous system involvement, and the emergence of gangrenous lesions in both legs. After conservative treatment failed both patients had both legs amputated and recovered.3 Recently, we encountered two patients with systemic lupus erythematosus and secondary CAPS who were less fortunate. Both had severe leg infections refractory to antibiotic, vasodilator, and anticoagulant treatment. Both refused bilateral leg amputation and after a short period died owing to multiple thrombi and multiorgan failure.
CAPS differs from APS by its systemic involvement and down hill progression ending in multiorgan failure and death. Kitchens reasoned that a “fibrinolytic shutdown” might be caused during infections as they induce a transitory increase in plasminogen activator inhibitor concentrations.4 This especially when another thrombophilic conditions is present may lead to a “thrombotic storm”.
We believe that the patient described by Yoo et al1 does not comply with the definitions of CAPS. Therefore their citation of our previous work, underlining the importance of eradicating the source of infection even at the cost of amputating both legs (in case the infection cannot be conservatively controlled), is inaccurate. The two unfortunate new cases we mentioned clearly demonstrate how critical these conditions may be and how essential it is to reach a decisive mode of action.
We thank Dr Amital and his colleagues for their valuable opinions about important guidelines for treatment of catastrophic antiphospholipid syndrome (CAPS). We think there are two points which were raised by Dr Amital and colleagues: one is whether our case is strictly consistent with the criteria of CAPS, the other is to decide between the surgical and non-surgical intervention in cases of gangrenous extremities.
CAPS was initially defined as an accelerated APS with multiorgan failure.1 This definition has been refined to be classified as the definite and probable category through the consensus workshop of the 10th International Congress on antiphospholipid antibodies (aPL) on 29 September 2002.2
According to these new preliminary criteria, the patient presented by us3 belongs to the category of probable CAPS: shock, acute respiratory distress syndrome, stuporous mentality, and liver failure as well as the gangrenous extremities were the initial manifestations shown simultaneously (we had to omit these findings from the initial manuscript owing to the length of letters allowed in this journal). The presence of aPL was confirmed on two occasions 6 weeks apart. However, histopathological confirmation was not obtained. Therefore, there is no doubt that the patient had probable CAPS.
As Asherson et al commented in the new preliminary criteria,2 the optimal management of CAPS is not yet firmly established. In fact, we agree with Dr Amital’s opinion that it is important to eradicate the infectious source even at the cost of amputation in case the infection is intractable.4 However, we do not think that amputation should be performed in all cases of CAPS. If peripheral arterial pulses are intact, as they were in our patient, non-surgical management may be initially preferred to allow the chance that the patient’s lesion may improve. In addition, the new treatment algorithm presented by Asherson et al also recommends medical management as an initial treatment (anticoagulation plus high doses of steroid plus intravenous immunoglobulin and plasma exchange).2 In our opinion, there is no absolute rule for the management of gangrenous condition in CAPS. We also think that the decision to amputate should be individualised and remains to be elucidated in the further consensus study.
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