Objectives Risk factors for thromboembolism in SLE are poorly understood. We hypothesized a possible role for protein C, based on its dual activity in inflammation and haemostasis and on the evidence of an association between acquired activated protein C (APC) resistance (APCR) and high-avidity anti-protein C antibodies (anti-PC) with a severe thrombotic phenotype in venous thrombosis APS patients. Methods In a cross-sectional study of 156 SLE patients, the presence and avidity of IgG anti-PC was established by in house-ELISA, and APCR to exogenous recombinant human APC (rhAPC) and Protac (which activates endogenous protein C) was assessed by thrombin generation-based assays. Associations with aPL profile, thrombotic history and disease activity (BILAG and SLEDAI-2K) were also established. Results Anti-PC were detected in 54.5% of patients and APCR in 59%. Anti-PC positivity was associated with APCR to both rhAPC (P <0.0001) and Protac (P =0.0001). High-avidity anti-PC, detected in 26.3% of SLE patients, were associated with APCR in patients with thrombosis only (P <0.05), and with the development of thrombosis over time (range: 0–52 years; P =0.014). High-avidity anti-PC levels correlated with SLEDAI-2K (P =0.033) and total BILAG (P =0.019); SLEDAI-2K correlated inversely with APCR to Protac (P =0.004). Conclusion Anti-PC occur in patients with SLE, independently of aPL profile, and are associated with APCR. High-avidity anti-PC are associated with thrombosis and with active disease and might prove a novel marker to monitor the risk of thrombosis and disease progression in SLE.

Proteus syndrome is an extremely rare disorder that manifests as an asymmetric, disproportionate overgrowth of any connective tissue, such as bone, fat or epidermal nevi, in a mosaic or patchy pattern. It has an estimated prevalence of less than 1/1,000,000 live births. The diagnosis can be difficult because the phenotypes of the patients are variable. Many individuals develop cutaneous capillary malformation and prominent varicosities (large and complex vascular malformations). Thus, Proteus syndrome patients are at risk of developing deep vein thrombosis and pulmonary embolism. The authors present the case of a patient with Proteus syndrome who was admitted because of pulmonary thromboembolism and presented hypertrophy of the left arm and left hemithorax.

Objectives: To characterize the frequency of thrombocytopenia in patients with SLE and determine its time of onset during the course of the disease, severity and impact on mortality. Methods:This was a single centre cohort analysis of 707 patients with SLE followed for up to 40 years. We reviewed the patients' clinical notes identifying the presence of thrombocytopenia, its time of onset and ascertained other clinical and serological features of the disease. Thrombocytopenia was classified as mild (100-149x10 9 /L), moderate (31-99x10 9 /L) or severe (≤30x10 9 /L platelets). It was also classified as asymptomatic, with minor bleeding or with major bleeding.Results: 22.9% of patients (n=162) had thrombocytopenia prior to or during the course of SLE. Twenty three patients (14.2%) had isolated ITP before the diagnosis of SLE. Median follow-up time was 19 years (IQR=13). Most patients (N=67, 41.4%) had mild thrombocytopenia. More than half the patients (n=98, 60.5%) developed asymptomatic thrombocytopenia and only 6 patients (3.7%) had major bleeding events in the context of thrombocytopenia. The development of severe thrombocytopenia anytime during the course of SLE was associated with an increased risk of death (HR=3.57, p=0.025). There was an increased risk of death for male patients (HR=3.41, p=0.036) who develop thrombocytopenia and for those who presented with concomitant haemolytic anaemia (HR=3.07, p=0.027). Conclusion:The presence of severe thrombocytopenia (platelets ≤30x10 9 ) in patients with SLE is associated with an increased risk of death, regardless of bleeding events. Male patients with SLE and thrombocytopenia had an increased mortality risk, as have those who develop concomitant thrombocytopenia and haemolytic anaemia.

Eosinophilic granuloma is a localized, non-invasive form of Langerhans cell histiocytosis. It usually develops in the long bones and is more frequent in children under the age of 10 years. It is very rare in adults.  We present the case of a young woman admitted to hospital for persistent refractory left parietal headache, later revealed to be caused by an eosinophilic granuloma.