- [Sjögren's Syndrome: Early diagnosis and effective treatment]. [Journal Article]
- DMDtsch Med Wochenschr 2018; 143(24):1778-1782
- Primary Sjögren's syndrome (pSS) is an autoimmune disease affecting the salivary and lachrymal glands. The patients complain of symptoms of dry eyes and dry mouth, but up to 50 % may additionally dev...
Primary Sjögren's syndrome (pSS) is an autoimmune disease affecting the salivary and lachrymal glands. The patients complain of symptoms of dry eyes and dry mouth, but up to 50 % may additionally develop extraglandular manifestations such as arthritis, vasculitis, polyneuropathy, pulmonary fibrosis or interstitial nephritis. Most therapeutic studies on the glandular manifestations of pSS failed to meet their primary endpoints, possibly since many of the patients had already advanced and irreversible disease.In recent years several important advances have been made. The role of B and T lymphocytes in the pathogenesis of pSS has been identified. The disease activity scores ESSDAI and ESSPRI were developed and can now be used as endpoints in therapeutical studies. Since then, numerous promising new drugs, mostly affecting B and T lymphocytes, have been studied in clinical trials. New classification criteria have been proposed, which will be an important tool in making a diagnosis in an early disease stage, in which the glandular function may still be normal. It is very likely therefore, that we will soon have efficient therapies, which will be able to stop the disease progression in an early stage of pSS.
- Polyarteritis nodosa in case of familial Mediterranean fever. [Journal Article]
- TJTurk J Pediatr 2018; 60(3):326-330
- Gökçe İ, Altuntaş Ü, Filinte D, Alpay H. Polyarteritis nodosa in case of familial Mediterranean fever. Turk J Pediatr 2018; 60: 326-330. Familial Mediterranean fever (FMF) is an autosomal recessive d...
Gökçe İ, Altuntaş Ü, Filinte D, Alpay H. Polyarteritis nodosa in case of familial Mediterranean fever. Turk J Pediatr 2018; 60: 326-330. Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent self-limited attacks of fever accompanied by peritonitis, pleuritis, and arthritis. Protracted febrile myalgia syndrome (PFMS) is a rare form of vasculitic disease which is an uncommon dramatic manifestation of FMF, characterized by severe crippling myalgia and high fever. Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis affecting medium or small arteries. It is rarely observed in children, but its incidence increases in the presence of FMF. In this article we described a 14-year-old child diagnosed with FMF associated with PAN. Physicians should be aware of this possible association.
- Vascular Behçet's syndrome: an update. [Review]
- IEIntern Emerg Med 2018 Nov 29
- Behçet's syndrome (BS) is a complex vasculitis, characterised by peculiar histological, pathogenetic and clinical features. Superficial venous thrombosis (SVT) and deep vein thrombosis (DVT) are the ...
Behçet's syndrome (BS) is a complex vasculitis, characterised by peculiar histological, pathogenetic and clinical features. Superficial venous thrombosis (SVT) and deep vein thrombosis (DVT) are the most frequent vascular involvements, affecting altogether 15-40% of BS patients. Atypical thrombosis is also an important clinical feature of BS, involving the vascular districts of the inferior and superior vena cava, suprahepatic veins with Budd-Chiari syndrome, portal vein, cerebral sinuses and right ventricle. On the other hand, arterial involvement, although affecting only 3-5% of patients, represents a unique feature of BS, with aneurysms potentially affecting peripheral, visceral and pulmonary arteries. Vascular events in BS are promoted by inflammation, with neutrophils playing a key role in the pathogenesis of thrombotic events; in turn, coagulative components such as fibrinogen, thrombin, factor Xa and factor VIIa amplify the inflammatory cascade. Understanding the contribution of inflammatory and coagulation components in the pathogenesis of BS vascular events is crucial to define the most effective therapeutic strategy. Control of vascular thrombosis is achieved with immunosuppressants drugs rather than anticoagulants. In particular, use of azathioprine and cyclosporine in association with low-dose corticosteroids should be considered in DVT and SVT cases, while treatment with cyclophosphamide together with anti-TNF-α agents can be effectively used in arterial involvement. More recently, the anti-TNF-α drugs have also been reported as a valid alternative for the treatment also of venous events, especially DVT. An exception to the use of anticoagulant in BS could be represented by cerebral veins thrombosis. In this review, we will depict the main characteristics of the vascular involvement in BS, briefly describing histological and pathogenetic features, while focusing on the clinical and therapeutical approaches of the vascular manifestations of BS.
- A rare case report of polyangiitis overlap syndrome: granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis. [Journal Article]
- BPBMC Pulm Med 2018 Nov 29; 18(1):181
- CONCLUSIONS: To our knowledge, there have been very few cases reported of an overlap syndrome of GPA and EGPA. Identification of patients with a polyangiitis overlap syndrome of GPA and EGPA is imperative as prognosis, longitudinal management and treatment modalities may differ between these entities.
- Aging in Primary Systemic Vasculitis: Implications for Diagnosis, Clinical Manifestations, and Management. [Review]
- DADrugs Aging 2018 Nov 28
- Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) usually affect adults and older populations. The well recognized clinical (i.e., granulomatosis with polyangiitis [GPA], micr...
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) usually affect adults and older populations. The well recognized clinical (i.e., granulomatosis with polyangiitis [GPA], microscopic polyangiitis, and eosinophilic GPA) and serological phenotypes (i.e., anti-MPO-ANCA, anti-PR3-ANCA and ANCA negative) within AAV differ substantially for clinical, demographic, and epidemiological features, including age at presentation. Whether and how aging could contribute to the clinical expression of these disease phenotypes is intriguing and still overlooked. In addition, despite being predominantly a disease of the elderly, most of the studies analyzing drug interventions and the clinical trials on AAV explicitly excluded older patients, limiting the understanding of the disease in this subset of patients. In elderly patients induced with cyclophosphamide, a lower dose of treatment for patients aged 60 years or older and with reduced renal function has been recommended. Giant cell arteritis (GCA) and Takayasu's arteritis (TAK) are two primary systemic vasculitides involving large vessels that differ in age at presentation, with GCA patients being at least 10 years older than TAK patients. Different treatment approaches are effective in these conditions. However, a few authors have suggested that they might be considered expressions of the same clinical syndrome, rather than two different diseases. Novel insights into the role of senescence-related immunological and vascular processes might help to interpret the link between these two conditions. Overall, the impact of aging on all these vasculitides is complex and not easy to analyze. So far, few studies focusing on this topic have been published. We reviewed data on the clinical presentation, epidemiology, therapy, and disease- and treatment-related complications in patients affected by these vasculitides, highlighting the differences in young versus elderly subjects.
- An unusual case of post-cochlear implant performance degradation in a patient with suspected Cogan's syndrome. [Journal Article]
- CICochlear Implants Int 2018 Nov 24; :1-6
- Cogan's Syndrome is an autoimmune disorder that can affect the ear, eye and other organs. Although rare, Cogan's Syndrome is particularly relevant to the cochlear implant surgeon because the resultin...
Cogan's Syndrome is an autoimmune disorder that can affect the ear, eye and other organs. Although rare, Cogan's Syndrome is particularly relevant to the cochlear implant surgeon because the resulting hearing loss is often bilateral and the majority of cases progresses to profound levels where cochlear implantation may be indicated. There are many issues relating to this condition that concern the cochlear implant surgeon. Its rarity, lack of specific laboratory diagnostic tests and variability in the onset and types of manifestation relating to the ear, eye and other organs often pose diagnostic difficulties. Pre-operatively, the cochlear implant surgeon must anticipate and exclude the possibility of cochlear luminal obliteration and ossification. Although the post-implant hearing results are expected to be generally good in Cogan's Syndrome, the possibility of adverse hearing outcomes cannot be ruled out whether in the initial or subsequent post-operative period. The possible side effects of long-term immuno-suppressive therapy on the well-being of the cochlear implant are to be appreciated and managed. A case which posed much difficulty in management is presented and discussed to highlight some of these challenges.
- Extracellular traps in kidney disease. [Review]
- KIKidney Int 2018; 94(6):1087-1098
- During the past decade the formation of neutrophil extracellular traps (NETs) has been recognized as a unique modality of pathogen fixation (sticky extracellular chromatin) and pathogen killing (cyto...
During the past decade the formation of neutrophil extracellular traps (NETs) has been recognized as a unique modality of pathogen fixation (sticky extracellular chromatin) and pathogen killing (cytotoxic histones and proteases) during host defense, as well as collateral tissue damage. Numerous other triggers induce NET formation in multiple forms of sterile inflammation, including thrombosis, gout, obstruction of draining ducts, and trauma. Whether neutrophils always die along with NET release, and if they do die, how, remains under study and is most likely context dependent. In certain settings, neutrophils release NETs while undergoing regulated necrosis-for example, necroptosis. NETs and extracellular traps (ETs) released by macrophages also have been well documented in kidney diseases-for example, in various forms of acute kidney injury. Histones released from ETs and other sources are cytotoxic and elicit inflammation, contributing to necroinflammation of the early-injury phase of acute tubular necrosis in antineutrophil cytoplasmic antibody-related renal vasculitis, anti-glomerular basement membrane disease, lupus nephritis, and thrombotic microangiopathies. Finally, acute kidney injury-related releases of dying renal cells or ETs promote remote organ injuries-for example, acute respiratory distress syndrome. In this review, we summarize what is known about the release of ETs from neutrophils and macrophages in the kidney, the available experimental evidence, and ongoing discussions in the field.
- Significance of histological crescent formation in patients with IgA vasculitis (Henoch-Schönlein purpura)-related nephritis: a cohort in the adult Chinese population. [Journal Article]
- BNBMC Nephrol 2018 Nov 22; 19(1):334
- CONCLUSIONS: Adult IgAV-related nephritis patients with more crescents had more-severe renal manifestations and worse treatment responses, whereas the proportions of crescents were not associated with higher risks for ESRD or 50% decline in renal function. A more suitable pathological classification standard is needed to predict renal prognosis.
- Lymphocyte hydrogen sulfide production predicts intravenous immunoglobulin resistance in children with Kawasaki disease: A preliminary, single-center, case-control study. [Journal Article]
- MMedicine (Baltimore) 2018; 97(47):e13069
- The aim of the study was to identify whether lymphocyte hydrogen sulfide production is a potential biomarker to predict intravenous immunoglobulin (IVIG) resistance in children with Kawasaki disease ...
The aim of the study was to identify whether lymphocyte hydrogen sulfide production is a potential biomarker to predict intravenous immunoglobulin (IVIG) resistance in children with Kawasaki disease (KD).This preliminary, single-center, case-control study conducted between June 2016 and March 2018 in Shaanxi Provincial People's Hospital, 85 children (50 with KD and 35 healthy controls) were included. Laboratory biomarkers were collected from the medical records. All patients with KD received 1 g/kg/d IVIG for 2 days and 30-50 mg/kg/d oral aspirin. The aspirin dose was reduced from 3 to 5 mg/kg/d after body temperature normalized. Plasma hydrogen sulfide levels were detected using sulfide electrode. Lymphocyte hydrogen sulfide levels were detected using the human hydrogen sulfide ELISA kits at the acute stage.Of 50 patients with KD, 31 and 19 were diagnosed with complete KD (cKD) and incomplete KD (iKD), respectively. Eleven patients with KD were resistant to IVIG treatment. The laboratory biomarker findings and levels of plasma and lymphocyte hydrogen sulfide were significantly different between the patients with KD and control group (P < .001). Moreover, lymphocyte hydrogen sulfide production was significantly greater in IVIG-resistant patients than in the IVIG-responsive patients, both in cKD and iKD (P = .018 and P < .001 respectively). Receiver operating characteristic curve indicated that when the lymphocyte hydrogen sulfide production was >15.285 nmol/min/10 lymphocytes, the sensitivity and specificity for predicting IVIG resistance were 90.9% and 76.9%, respectively.Lymphocyte hydrogen sulfide production could serve as a predictor of the therapeutic efficacy of IVIG in children with KD.
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- Recurrent Cerebral Infarctions in Primary Sjögren Syndrome: A Case Report and Literature Review. [Journal Article]
- FNFront Neurol 2018; 9:865
- Recurrent cerebral infarctions are extremely rare in patients with primary Sjögren syndrome. We report a 66-year-old woman who was admitted to our hospital due to acute cerebral infarction with exace...
Recurrent cerebral infarctions are extremely rare in patients with primary Sjögren syndrome. We report a 66-year-old woman who was admitted to our hospital due to acute cerebral infarction with exacerbation of dysphagia and right-sided hemiplegia as the main symptoms. In the past 3 months, she had developed cerebral infarction twice, even though she had no risk factors for atherosclerosis. She was eventually diagnosed with primary Sjögren syndrome based on a long history of dryness of the eyes and mouth, positive anti-Ro(SSA) antibodies, and the findings of a labial salivary gland biopsy. The response to pulse methylprednisolone therapy after recurrent cerebral infarctions was poor. Thus we consider primary Sjögren syndrome patients with central nervous system involvement should be treated as soon as possible.