José Delgado Alves

Lupus or syphilis? That is the question!

A 47-year-old man presented with fever, a maculopapular rash of the palms and soles, muscular weakness, weight loss, faecal incontinence, urinary retention and mental confusion with 1u2005month of evolution. Neurological examination revealed paraparesis and tactile hypoesthesia with distal predominance, and no sensory level. Laboratory investigations revealed a venereal disease research laboratory (VDRL) titre of 1/4 and Treponema pallidum haemagluttin antigen (TPHA) of 1/640, positive anti-nuclear antibodies of 1/640 and nephrotic proteinuria (3.6u2005g/24u2005h). Lumbar puncture excluded neurosyphilis, due to the absence of TPHA and VDRL. The diagnosis of systemic lupus erythematosus (SLE) was established and even though transverse myelitis as a rare presentation of SLE has a poor outcome, the patient improved with cyclophosphamide, high-dose corticosteroids and hydroxychloroquine. A diagnosis of secondary syphilis was also established and the patient was treated with intramuscular benzathine penicillin G.

PS1:13 Systemic lupus erithematosus with positive anticentromere antibodies – a different clinical subgroup?

Introduction Anticentromere antibodies (ACA) are one of the most specific systemic sclerosis (SSc)-related antibodies. The presence of ACA has also been identified in other autoimmune diseases, mainly in SSc overlap syndromes with Sjogren syndrome (SjS), primary biliary cirrhosis and rarely in patients with systemic lupus erythematosus (SLE). Purpose To evaluate the prevalence and clinical significance of ACA in a cohort of SLE patients. Methods Retrospective analysis of all ACA positive SLE patients (ACA +SLE), from a cohort of 270 consecutive SLE patients fulfilling the 2012 SLICC Criteria and/or 1997 ACR Criteria, of a single referral centre, between 2010–2016. Comparative analysis was made with a representative group of 63 consecutive SLE patients without ACA (ACA-veSLE). Data were obtained by medical records review. Results From 270 SLE patients, 10 (3.7%) were ACA+. All ACA +SLE patients were female. The age at the time of diagnosis was not different between the groups (40.9±16.6 years ACA +SLEu2009vs 37.8±16.2 years ACA-veSLE), but ACA +SLE patients had longer disease duration (15.2±17.3 years vs 9.5±8.8 years, p=0.002, respectively). ACA +SLE patients had significantly more Raynaud’s phenomenon (RP) (p=0.028), but none had a capillaroscopy SSc pattern. Sicca symptoms were also more frequent in ACA +SLE (p=0.013), with only 1 patient with a positive anti-SSA antibody. None of these patients fulfilled criteria for SjS. Prevalence of arthritis, oral ulcers, alopecia, cutaneous lupus, serositis, neurologic, renal and hematologic involvement was not significantly different between the two groups. Hypocomplementemia at any time of the disease course was more frequent in ACA-veSLE (p=0.016). Antiphospholipid antibodies were less frequently positive in ACA+SLE patients (20% vs 46%, p=0.1), and none fulfilled criteria for antiphospholipid syndrome (APS) (21% of ACA-veSLE patients with APS). Apart from RP, SSc-associated clinical characteristics (skin thickening, digital ulcers, telangiectasia, pulmonary arterial hypertension, interstitial lung disease, gastroesophageal reflux and calcinosis) were not present in any of the ACA+SLE patients. Conclusions ACA +SLE patients do not constitute a different clinical subgroup regarding organ involvement, but can associate with a lower probability of concomitant APS. Moreover, although highly specific of SSc, ACA can be identified in SLE patients without SSc overlap, and should not hamper the diagnosis of SLE.

Relevance of antiphospholipid antibodies in multiple sclerosis: A systematic review and meta analysis.

BACKGROUNDnThe relationship between antiphospholipid antibodies (aPL) and multiple sclerosis (MS) is unclear.nnnOBJECTIVESnTo evaluate a link between aPL and MS.nnnMETHODSnEMBASE and PubMed search to August 2016; Petos odds ratio (OR) meta-analysis.nnnRESULTSnThe pooled prevalence of participants positive for IgG and IgM anticardiolipin (aCL) from 12 case-control studies was superior in MS than controls (6.8% vs 1.8%, p = 0.01 and 8.58% vs 2.18%, p = 0.001) with medium and high heterogeneities respectively (I2 = 48.55% and 68.13%). The pooled prevalence of participants positive for IgG anti-beta2glycoprotein-I (aβ2GPI) from seven case-control studies was lower in MS than controls (0.93% vs 4.02%) with high heterogeneity (I2 = 53.92%) though the pooled prevalence of participants positive for IgM aβ2GPI was similar (7.24% vs 6.13%) with high heterogeneity (I2 = 52.85%). Five cohorts compared IgG/IgM aCL and IgM aβ2GPI in stable/remission vs active/relapsing MS: the pooled prevalence of IgG aCL was similar in active/relapsing and stable/remission MS (19% vs 18.9%) but the pooled prevalence of IgM aCL was higher in active than in stable MS (36.9% vs 21%, p < 0.0001) as well as that of IgM β2GPI (40.5% vs 3.2%, p < 0.0001) with no heterogeneity.nnnCONCLUSIONnData from case-control studies do not support a link between IgG/IgM aPL and MS. Data from cohort studies comparing active vs stable MS indicate a strong link between aPL of IgM isotype and active/relapsing MS but in the absence of aPL titres to comment upon this may either represent an epiphenomenon of active neuro-inflammation or natural autoantibodies devoid of pathogenic potential. Data expressed as frequency of aPL positive participants rather than average titres preclude further assumptions.

IgA-dominant postinfectious glomerulonephritis induced by methicillin-sensitive Staphylococcus aureus.

A 56-year-old man with alcohol-associated cirrhosis, arterial hypertension and diabetes, presented with a 1-month history of fever, lumbar back pain and lower limb weakness. MRI revealed a spinal epidural abscess extending from the cervical to the dorsolumbar spine. A methicillin-sensitive Staphylococcus aureus strain was isolated on blood cultures. Meropenem was initially started with no response, and then changed to vancomycin. During treatment, the patient’s condition progressed with anasarca and renal failure with nephrotic-range proteinuria. The renal biopsy showed a membranoproliferative glomerulonephritis with IgA deposition. After completing 2u2005months of antibiotic therapy the patient recovered from the neurological deficits, with a complete resolution of the abscess and partial recovery of renal function and proteinuria.

Refractory Wegener's granulomatosis presenting with alveolar haemorrhage, treated with rituximab

A previously healthy 57-year-old man presented with epistaxis, crusted nasal ulcers and weight loss. CT scan of the sinuses revealed an inflammatory swelling of the ethmoid and maxillary sinuses (figure 1) and CT scan of the lungs showed two pulmonary nodular lesions in both upper lobes. Laboratory investigation showed an elevated erythrocyte sedimentation rate (97u2005mm/h) and a positive anti-PR3-antineutrophil cytoplasmic antibody.nnnnFigurexa01 nCT scan of the sinuses showing an inflammatory swelling of the ethmoid and …

Xanthogranulomatous pyelonephritis presenting as palmoplantar keratoderma.

A 60-year-old woman with type 1 diabetes mellitus and a history of repeated urinary tract infections due to Proteus mirabillis presented with diffuse palmoplantar hyperkeratosis of 4u2005months duration (figure 1) and acute retrosternal pain. An ECG showed a diffuse concave ST segmental elevation and the echocardiogram showed a mild pericardial effusion, suggestive of pericarditis. Laboratory analysis showed negative troponin, leucocytosis, elevated erythrocyte sedimentation rate (110u2005mm/h) and C reactive protein (10u2005mg/dL). A CT scan revealed a hydronephrotic, non-functioning left kidney with an inflammatory infiltrate in …

Atherogenesis and Vascular Disease in SLE

SLE is the classical model of a chronic multi-systemic immune-mediated inflammatory disease. It affects mainly young women, a subgroup of the general population usually free of cardiovascular risk. Although survival rates have improved dramatically, mainly due to early diagnosis, improved treatment, and better management of complications, death rates for patients with SLE remain 3 to 5 times higher than in the general population (Haque & Bruce, 2009). Nevertheless, whilst the 5-year survival of SLE was below 50% in the 1950s, it is nowadays above 90% (Nikpour et al., 2005). Atherosclerosis in SLE is a highly complex process with autoimmunity, local and systemic inflammation, and endothelial dysfunction playing critical roles in its initiation and propagation. In the particular case of SLE, the extremely intricate immune system deregulation involving all types of immune cells up to an increased autoantibody production seems to play a major role for the accelerated atheroma formation found in these patients. Cardiovascular events are now the major cause of morbidity and mortality in SLE. The acceptance of the importance of vascular risk in this context came from the description of a bimodal mortality pattern (Urowitz et al., 1976), with the early peak (within 1 year of diagnosis) as a consequence of active lupus and its complications, and the later peak (more than 5 years after diagnosis) mainly attributable to atherosclerosis. SLE is now considered to be a coronary heart disease-risk equivalent, mainly due to accelerated atherosclerosis (Aranow & Ginzler, 2000; Bjornadal et al., 2004; Manzi et al., 1997; Esdaile et al., 2001; Fischer et al. 2004; Roman et al., 2003; Ward, 1999). This can be especially relevant in young women, where up to a 50-fold increase in cardiovascular risk over age and gender-matched controls has been reported (Manzi et al., 1997). In fact, the majority of those women were aged less than 55 years at the time of their first cardiac event. Framingham risk factors do not explain entirely the atherosclerotic burden found in patients with SLE. Furthermore, traditional cardiovascular risk factors seem to be less important predictors of cardiovascular events than the activity of lupus (Esdaile et al., 2001). (see table 1). The direct relation between conventional and SLE-related risk factors and the actual incidence of events has not been easy to establish for different reasons: most patients with