The relationship between antiphospholipid antibodies (aPL) and autoimmune haemolytic anaemia (AIHA) has never been systematically addressed. The aim of this study is to assess the link between aPL and AIHA in adult systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). This study performed an EMBASE/PubMed search from inception to June 2019 and meta-analysis using Peto’s odds ratios. The pooled prevalence (PP) of IgG/IgM anticardiolipin (aCL) and lupus anticoagulant (LA) was greater in AIHA +ve than AIHA −ve patients (34.7% vs. 27.6%, p = 0.03; 33.3% vs. 21.8%, p < 0.0001; 20.9% vs. 8.3%, p = 0.01). The PP of AIHA was greater in: (1) IgG and IgM aCL +ve than −ve patients (21.8% vs. 11.1%, p = 0.001 and 18.7% vs. 6.3%, p < 0.0001), (2) in SLE related APS than in primary APS patients (22.8% vs. 3.9% p < 0.0001), (3) in APS +ve than APS −ve SLE patients (23.2% vs. 8.4%, p = 0.01), and (4) in thrombotic APS than non-thrombotic APS/SLE patients (26.8% vs. 10%, p = 0.03). The PP of IgG/IgM aCL and LA was greater in DAT +ve than DAT −ve patients (42.4% vs. 12.8%, p < 0.0001; 26.2% vs. 12.8%, p = 0.03 and 29.2% vs. 15.7%, p = 0.004 respectively). It was found that AIHA prevalence is maximal in SLE with aPL/APS, low-moderate in SLE without aPL and minimal in PAPS. Moreover, AIHA is rightly included among the classification criteria for SLE but not for APS/aPL. The significance of an isolated DAT positivity remains unclear in this setting

Multiple sclerosis (MS) and antiphospholipid syndrome (APS) share common clinical, laboratory and radiological features. We reviewed all the English papers on MS and APS published in the literature from 1965 to 2014 using PubMed and Google Scholar. We found that APS can mimic antiphospholipid antibodies (aPL)-positive MS in many ways in its clinical presentation. Nevertheless, APS diagnosis, clinical manifestations and management differ from those of MS. aPL were found in MS patients with titers ranging from 2% to 88%. The distribution and volume of lesions on magnetic resonance imaging (MRI) may help to differentiate MS from primary APS. In addition, atypical MS presentation can guide physicians toward an alternative diagnosis, especially when features of thrombosis and/or history of connective tissue disease are present. In that case, an anticoagulation trial could be worthwhile.

Eosinophilic fasciitis (EF) is a rare connective-tissue disorder that is characterised by subacute onset of erythema, oedema, and induration of the skin and soft tissues of the limbs and trunk. Although several triggers have been hypothesised to be associated with EF, the aetiology of eosinophilic fasciitis (EF) is still unclear, and several treatment regimens have been proposed to treat this disease. In this article, we report a case of a 72-year-old gentleman with multiple comorbidities who presented to the clinic for diffuse skin thickening present on his forearms, thighs, legs bilaterally, and over the pelvis. The patient was diagnosed with EF and failed multiple treatment regimens including prednisone, methotrexate, rituximab, but finally responded and was maintained on tocilizumab. In this article, we will review the current understanding of EF, diagnostic approach, popular treatments and review other cases of EF in which tocilizumab was used.

Background: Behçet’s Disease (BD) is a complex vasculitis affecting multiple organ systems, with Neuro-Behçet’s Disease (NBD) representing a rare yet severe manifestation. Data on NBD are limited, particularly in Middle Eastern populations. Methods: This retrospective observational study, spanning from 2000 to 2021, involved 262 BD patients at a tertiary medical center in Lebanon. NBD was diagnosed based on International Consensus Recommendation diagnostic criteria. Clinical data, including demographics, manifestations, inflammatory blood markers, genetics, and treatments, were collected. The modified Rankin Scale (mRS) was used to assess disease severity. Results: Among the cohort, 27 (10.3%) had NBD, with headaches, weakness, and dizziness as the most common presenting symptoms. The prevalence of NBD was similar across genders, which differs from some regional studies. HLA-B51 positivity was found in 50 out of 60 (83.3%) tested BD patients. Parenchymal NBD cases exhibited greater disease severity than non-parenchymal cases, with female patients experiencing a more severe course compared to males. Elevated inflammatory markers (CRP and ESR) were more common in patients with severe NBD. Corticosteroids and colchicine were the most commonly used therapies overall, while patients with better disease severity were more frequently prescribed methotrexate, mycophenolate, cyclophosphamide, adalimumab, and rituximab. An analysis of disease progression showed that at presentation, 57.1% (n = 12) of NBD patients had mild to moderate disability, which increased to 76.2% (n = 16) at the last follow-up, including 10 patients who showed an improvement in their mRS score. Conclusions: This study provides valuable insights into the prevalence and clinical characteristics of NBD in a Middle Eastern population. These findings enhance our understanding of NBD in the Middle East, highlighting the need for further research to improve diagnosis and management.

Autoimmunity and the overall state of chronic inflammation are associated with many extra- articular manifestations.(1) Rheumatoid vasculitis is probably the rarest yet the most severe extraarticular manifestation and is usually observed in patients with long-standing, uncontrolled seropositive disease.(2) In this article, we describe the case of a 61-year-old female patient diagnosed with seropositive long-standing rheumatoid arthritis presented to our hospital recently and episodic fluctuations of altered level of consciousness of one week duration, initially diagnosed to have bacterial meningitis based on cerebrospinal fluid analysis with superimposed pneumonia and started on antibiotics with no improvement. Patient is known to have multiple rheumatoid nodules in her chest, followed-up by serial computed tomography scans. Magnetic resonance imaging (MRI) of the brain upon admission showed multiple confluent high fluid- attenuated inversion recovery (FLAIR) signals in the periventricular white matter, hippocampal gyrus and peritrigonal regions of both cerebral hemispheres (Figure 1). In light of the patient’s known diagnosis of rheumatoid arthritis, these findings were compatible with an inflammatory process consistent with rheumatoid vasculitis. Repeated cerebrospinal fluid analysis after antibiotics treatment indicated increased number of inflammatory cells, mainly lymphocytes and monocytes. Open lung biopsy revealed active chronic interstitial pneumonitis (Figure 2). It was then decided to start our patient on steroids: intravenous methylprednisolone 40 mg push twice daily and antibiotics were discontinued with the diagnosis of rheumatoid cerebral vasculitis. She was then discharged on prednisone 40 mg orally once daily. Repeated MRI of the brain one month later displayed significant decrease in the high FLAIR signals. Repeated lumbar puncture and cerebrospinal fluid analysis three months later demonstrated mature lymphocytes and occasional monocytes. Decision was to proceed with rituximab infusions as a steroid-sparing agent. Repeated MRI and cerebrospinal fluid studies three months after first cycle of rituximab showed significant improvement. Cerebral vasculitis is a rare complication of rheumatoid arthritis with frequency ranging between 1% and 8%.(3) The damage in the endothelium may trigger thrombus formation which might result in ischemia or hemorrhage in the area served by the vessel that can be seen on the macroscopic level.(4) Rheumatoid vasculitis is most commonly seen in seropositive females with destructive and erosive rheumatoid arthritis associated with subcutaneous nodules and other extraarticular manifestation.(4) A biopsy is required for a definitive diagnosis of cerebral vasculitis. However, it is not always feasible and mostly based on cerebrospinal fluid and cerebral imaging studies. Cerebrospinal fluid would demonstrate lymphocytosis and protein elevation which implies an underlying inflammation. There are no clear guidelines when it comes to treatment of cerebral vasculitis in rheumatoid arthritis. However, glucocorticoids have been commonly used such as pulse methylprednisolone.(5) In a study carried out by Puéchal et al.,(6) complete remission of cerebral rheumatoid vasculitis was observed in 12 out of 17 patients receiving rituximab. Our patient had significant response on intravenous methylprednisolone and rituximab that was shown by repeated MRI of the brain.

Subjective loss of response immediately prior to routine TNFi therapy can occur in axial spondyloarthritis (axSpA). We investigated clinical outcomes in patients taking the first 3 licenced TNFis and correlated this with recurrence of MRI bone marrow oedema (MRI-BMO). Proof-of-concept study including axSpA patients established on etanercept (ETA), adalimumab (ADA) or infliximab (IFX) reporting symptom deterioration prior to next dose. MRI/clinical data were collected prior to scheduled dose (v1), 4 days post-dose (v2) and at the time of patient-reported symptom return (v3). MRI spine/sacroiliac joints utilising 3 T were scored using the semi-quantitative Leeds MRI scoring system. 113 clinical assessments and MRIs were performed in 38 participants (ADA = 16, ETA = 12, IFX = 10), mean age 42.1 years ± 24.4(2SD, n = 38), 71.1% male (n = 27/38), 69.7% HLA-B27 positive (n = 23/33). At v1 all patients had high disease activity [ASDAS-CRP = 3 (2.7-3.7)] and 57.9% had MRI-BMO (number of MRI-BMO: ETA = 26, ADA = 59, IFX = 28). Improved clinical responses were seen at v2 [ASDAS-CRP -0.41(-0.81-0.30), p= 0.018; BASDAI -0.58(-2.2-0.52), p= 0.024]. Despite just a 4-day interval between v1 and v2, a numerical reduction in MRI-BMO lesions between v1/v2 was observed (ETA=-6, ADA=-10, IFX=-3). By v3 comparatively fewer new BMO lesions were detected in the ETA and ADA groups compared with IFX (ETA=-1, ADA = +3, IFX = +8), although the numbers were too small to enable testing for statistical significance. Short-lived fluctuations in MRI-BMO were commoner with longer-acting agents and corresponded with subjective loss of clinical response before next scheduled TNFi dose. Larger studies are need to confirm the possible pathogenic implications of this phenomenon.