Background and Aims

Antiphospholipid syndrome (APS) occurs in 1% of cases as catastrophic variant (CAPS), a highly lethal condition characterized by acute onset of multiple thromboses and/or microangiopathy. Aortic involvement is unusual and requires a high suspicion for diagnosis.

Methods

We describe the case of APS who developed acute aorto-iliac occlusion (Leriche syndrome) as CAPS debut. We also performed a review of the literature by Medline and EMBASE databases

Results

A 64-year-old male patient with primary APS history and previous arterial events was on treatment with only clopidogrel. He presented with aortic barrage so he underwent aorto-bifemoral by pass. At day 1 after surgery he developed thrombocytopenia, hemolytic anemia, livedo racemosa, cutaneous necrosis at lower limbs and alveolar hemorrhage. Antiphosholipid antibodies resulted positive at extremely high titer. Blood smear revealed schistocytes>6%. Skin biopsy documented thrombosis of dermal vessels. Treatment with methylprednisolone pulses, anticoagulation and plasma exchanges was started with full recovery.

Eight cases of Leriche syndrome in APS have been described so far: 5 primary forms (PAPS), 1 case associated with relapsing polychondritis. In 2 cases data were missing. In 3 cases aortic barrage represented APS onset manifestation, 4 cases were preceded by arterial thromboses, while none by venous thrombotic events. No proven evolution into CAPS was previously reported.

Conclusions

Aortic occlusion should be suspected in patients with APS and consistent clinical presentation, particularly when other cardiovascular risk factors or history of arterial thrombosis are present. CAPS is a life-threatening complication of surgery in patients with APS and must be systematically excluded in presence of symptoms.

Background and Aims

Eosinophilic granulomatosis with polyangiitis (EGPA), is a systemic disease characterized by late onset asthma, eosinophilia and vasculitis. About 20-30% of patients with EGPA displays inhalant allergies, while prevalence of food and drug allergy is unknown. Some authors hypothesized a role of allergen-specific immunotherapy (ASIT) as trigger of disease. Aim of the study is to establish the prevalence of allergies among EGPA patients and to determine whether atopy or ASIT could influence clinical expression of the disease.

Methods

Retrospective demographic and clinical data collection of EGPA patients in our center.

Results

Fifty-three (53) patients with definitive diagnosis of EGPA have been included in the analysis among which 25 (47.2%) with allergy history. Among allergic patients 15 (60%) resulted sensitized towards inhalants and among them 13 (86.7%) displayed multiple sensitization. Drug allergy affected 13 patients (52%), food 4 (16%). Thirteen subjects were eligible to ASIT and 7 underwent ASIT prior EGPA diagnosis with an average time-to-EGPA of 16.2 years. No statistically significant difference was found in terms of sex, age at diagnosis, positivity or specificity of anti-neutrophil cytoplasm antibodies (ANCA), eosinophil count at onset, clinical manifestations comparing allergic vs. non-allergic, ASIT vs. non-ASIT, ASIT vs. allergic, ASIT vs. eligible.

Conclusions

Among patients with EGPA allergies are highly prevalent, particularly towards inhalants and drugs, often with multiple sensitizations. The absence of correlation between inhalant ASIT exposure and variation in mode and time of EGPA onset doesn’t support the hypothesis of a its potential role in triggering the disease.