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https://www.readbyqxmd.com/read/28098669/eye-problems-in-a-woman-with-churg-strauss-syndrome
#1
Benjamin D Pierce, Carrlene B Donald, Anthony P Mendez
Churg-Strauss syndrome is a rare, systemic vasculitis of unknown cause. Ocular involvement is a rare but established complication and can lead to vision damage or blindness if not treated promptly. Treatment of ocular manifestations corresponds with systemic treatment of the disease and consists primarily of corticosteroids.
February 2017: JAAPA: Official Journal of the American Academy of Physician Assistants
https://www.readbyqxmd.com/read/28097447/mannose-binding-lectin-mbl-codon-54-rs1800450-polymorphism-predisposes-towards-medium-vessel-vasculitis-in-patients-with-systemic-lupus-erythematosus
#2
Vir Singh Negi, Panneer Devaraju, Durga Prasanna Misra, Vikramraj K Jain, Jignesh Babulal Usdadiya, Paul T Antony, Reena Gulati
Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with multiple etiological factors. Mannose-binding lectin (MBL) plays a key role in innate immunity by activating antibody-independent lectin complement pathway, opsonisation, phagocytosis, and immune complex (IC) clearance. Genetic polymorphisms in the promoter and coding regions of MBL gene affect the circulatory levels and biological activity of MBL. Defects in MBL can lead to defective opsonisation and, hence, hamper clearance of apoptotic debris, the persistence of which can drive autoantibody formation in lupus...
January 17, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28094303/genetic-polymorphisms-of-rs3077-and-rs9277535-in-hla-dp-associated-with-systemic-lupus-erythematosus-in-a-chinese-population
#3
Junlong Zhang, Wenli Zhan, Bin Yang, Anning Tian, Lin Chen, Yun Liao, Yongkang Wu, Bei Cai, Lanlan Wang
Although the SLE risk gene loci of HLA-DR and HLA-DQ within the major histocompatibility complex (MHC) region has been gradually revealed by recent Genome-Wide Association studies (GWAS), the association of HLA-DP polymorphisms with SLE was minimally reported. Considering that the variants in rs3077 and rs9277535 in the HLA-DP region could influence the immune response by affecting antigen presentation of HLA class II molecules to CD4(+) T cells, the present study aimed to explore the role of HLA-DP polymorphisms in SLE...
January 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28094164/disorders-characterized-by-predominant-or-exclusive-dermal-inflammation
#4
Mark R Wick
Some cutaneous inflammatory disorders are typified by a predominant or exclusive localization in the dermis. They can be further subdivided by the principal cell types into lymphocytic, neutrophilic, and eosinophilic infiltrates, and mixtures of them are also seen in a proportion of cases. This review considers such conditions. Included among the lymphoid lesions are viral exanthems, pigmented purpuras, gyrate erythemas, polymorphous light eruption, lupus tumidus, and cutaneous lymphoid hyperplasia. Neutrophilic infiltrates are represented by infections, Sweet syndrome, pyoderma gangrenosum, and hidradenitis suppurativa, as well as a group of so-called "autoinflammatory" dermatitides comprising polymorphonuclear leukocytes...
December 14, 2016: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28094068/-large-vessel-vasculitis-with-myelodysplastic-syndrome-a-rare-association
#5
J Galland, H Kawski, J-F Guichard, F Maurier
INTRODUCTION: The vasculitis can be the consequence of malignancy: most often hematologic rather than solid tumors. The association between large vessels vasculitis and myelodysplastic syndrome is rare. CASE REPORT: A 55-year-old man experienced asthenia, fever, polyarthritis and inflammatory syndrome. Haematological investigations found a type 2 refractory anemia with excess blasts (RAEB-2) with discovery of severe anemia (Hb: 7,8g/dl) and thrombopenia (platelets: 40,000/mm(3))...
January 13, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28092221/tuberculosis-mimicking-primary-systemic-vasculitis-not-to-be-missed
#6
Ankit Jain, Durga Prasanna Misra, A Ramesh, Debdatta Basu, Vikramraj K Jain, Vir Singh Negi
Infections are an important differential diagnosis in patients presenting with features of systemic vasculitis. We report a young lady with constitutional features, leg ulcers, digital gangrene and absent peripheral pulses with cervical adenopathy. Chest imaging revealed multiple necrotic lung lesions and involvement of left subclavian artery at its origin from the aorta, Histopathology from cervical lymph nodes showed multiple caseated lymph nodes, which in the context of a positive Mantoux test led us to diagnose tuberculosis and institute appropriate therapy...
January 1, 2017: Tropical Doctor
https://www.readbyqxmd.com/read/28089971/incidence-of-malignancy-prior-to-antineutrophil-cytoplasmic-antibody-associated-vasculitis-compared-to-the-general-population
#7
Emma E van Daalen, Chinar Rahmattulla, Ron Wolterbeek, Jan A Bruijn, Ingeborg M Bajema
OBJECTIVE: Previous studies have reported an increased malignancy risk preceding antineutrophil cytoplasmic antibody-associated vasculitis (AAV), suggesting common pathogenic pathways in these 2 entities. However, the study results were conflicting and often limited to patients with granulomatosis with polyangiitis (GPA). Here, we study the malignancy risk prior to AAV diagnosis [either GPA or microscopic polyangiitis (MPA)] to elaborate on the putative association between malignancy and AAV...
January 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28089164/low-median-nerve-palsy-as-initial-manifestation-of-churg-strauss-syndrome
#8
Young Hak Roh, Young Do Koh, Jung Ho Noh, Hyun Sik Gong, Goo Hyun Baek
Anterior interosseous nerve (AIN) syndrome is typically characterized by forearm pain and partial or complete dysfunction of the AIN-innervated muscles. Although the exact etiology and pathophysiology of the disorder remain unclear, AIN syndrome is increasingly thought to be an inflammatory condition of the nerve rather than a compressive neuropathy because the symptoms often resolve spontaneously following prolonged observation. However, peripheral neuropathy can be 1 of the first symptoms of systemic vasculitis that needs early systemic immunotherapy to prevent extensive nerve damage...
January 11, 2017: Journal of Hand Surgery
https://www.readbyqxmd.com/read/28088210/endothelial-function-in-children-with-a-history-of-henoch-schonlein-purpura
#9
Yonatan Butbul Aviel, Lotem Dafna, Giora Pilar, Riva Brik
BACKGROUND: Although Henoch-Schonlein purpura (HSP) is the most common form of systemic vasculitis in children, the long term effect of HSP on endothelial function is still not clear. The aim of our study was to evaluate the long term effect of HSP on endothelial function in children and adolescents. METHODS: This research was an observational prospective study. The study group comprised of 19 children diagnosed with HSP. The minimum interval between the diagnosis with HSP and endothelial testing was 5 months...
January 14, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28087591/clinical-trials-in-antineutrophil-cytoplasmic-antibody-associated-vasculitis-what-we-have-learnt-so-far-and-what-we-still-have-to-learn
#10
Divi Cornec, Emilie Cornec-Le Gall, Ulrich Specks
The prognosis of the antineutrophil cytoplasmic antibody associated vasculitides (AAV), microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA), has been fundamentally improved over the last five decades by the use of glucocorticoids and immunosuppressants, turning them from consistently fatal diseases into chronic conditions. The long-term course is now largely determined by the frequency of disease flares and by accruing damage caused by disease activity and treatment-related comorbidities...
January 12, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28087351/central-nervous-system-vasculitis-in-adults-an-update
#11
REVIEW
Lívia Almeida Dutra, Alexandre Wagner Silva de Souza, Gabriela Grinberg-Dias, Orlando Graziani Povoas Barsottini, Simone Appenzeller
Primary central nervous system vasculitis (PCNSV) is a challenging diagnosis due to broad clinical manifestations and variable specificity and sensitivity of laboratory and imaging diagnostic tools. Differential diagnosis include reversible cerebral vasoconstriction syndrome (RCVS), secondary vasculitis of the CNS and other noninflammatory vasculopathies. Brain biopsy is essential for definitive diagnosis and to exclude mimickers. Recent data show data large-vessel PCNSV present worse prognosis when compared to small-vessel PCNSV...
January 10, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28079860/erratum
#12
Georgi Tchernev, Anastasiya A Chikoeva, Marco Tana, Claudio Tana
In the article "Transcriptional blood signatures of sarcoidosis, sarcoid-like reactions and tubercolosis and their diagnostic implications" which appeared in Volume 33, Issue 3 (2016) of Sarcoidosis, vasculitis and diffuse lung diseases, the title was given incorrectly. The correct title is "Transcriptional blood signatures of sarcoidosis, sarcoid-like reactions and tuberculosis and their diagnostic implications".
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28079834/intestinal-brucellosis-associated-with-celiac-artery-and-superior-mesenteric-artery-stenosis-and-with-ileum-mucosa-and-submucosa-thickening-a-case-report
#13
Miaoqian Wang, Qingli Zhu, Qian Yang, Wenbo Li, Xinning Wang, Wei Liu, Baotong Zhou, Zhenghong Li, Hong Yang
RATIONALE: Brucellosis is a multisystem infection found worldwide that has a broad range of characteristics, which range from acute fever and hepatomegaly to chronic infections that most commonly affect the central nervous system, cardiovascular system, or skeletal system. Gastrointestinal and splanchnic artery involvements in brucellosis are relatively uncommon. PATIENT CONCERNS: We report a case of brucellosis in an adolescent presenting as intermittent abdominal pain, diarrhea, and fever, with intestinal tract involvement...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28079801/acute-necrotizing-retinal-vasculitis-as-onset-of-systemic-lupus-erythematosus-a-case-report
#14
Simeon Monov, Ruska Hristova, Rositza Dacheva, Reni Toncheva, Russka Shumnalieva, Viara Shoumnalieva-Ivanova, Daniela Monova
RATIONALE: Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by autoantibody production, complement activation, and deposition of immune complexes in tissues and organs. SLE can involve any region of the visual system. Although ocular manifestations are not part of the classification criteria for SLE, they can be observed in up to one-third of the patients with SLE. They are rarely reported at the time of disease onset. Retinal vasculitis is usually associated with active generalized disease...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28079316/clinical-characteristics-and-treatment-response-of-peripheral-neuropathy-in-the-presence-of-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome-experience-at-a-single-tertiary-center
#15
Hye Jin Cho, Sehyo Yune, Jin Myoung Seok, Eun Bin Cho, Ju Hong Min, Yeon Lim Seo, Byung Jae Lee, Byoung Joon Kim, Dong Chull Choi
BACKGROUND AND PURPOSE: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic small-vessel vasculitis accompanied by asthma, eosinophilia, and eosinophilic inflammation of various tissues including the peripheral nerves. This study investigated the clinical course and long-term outcomes of peripheral neuropathy in patients with EGPA. METHODS: Seventy-one patients with physician-diagnosed EGPA were identified at Samsung Medical Center between January 1995 and April 2014...
January 2017: Journal of Clinical Neurology
https://www.readbyqxmd.com/read/28077689/giant-cell-arteritis-and-vascular-disease-risk-factors-and-outcomes-a-cohort-study-using-uk-clinical-practice-research-datalink
#16
Lin Li, Tuhina Neogi, Susan Jick
OBJECTIVE: To evaluate the associations between GCA and vascular diseases and other comorbidities in patients with GCA compared with non-vasculitis patients. METHODS: Using the UK-based Clinical Practice Research Datalink we identified 9778 newly diagnosed GCA patients in 1990-2014, and up to 10 non-vasculitis patients randomly matched to each case on age, sex, practice and years of history before cohort entry. We compared the distributions of 9 different pre-existing vascular diseases and 11 other comorbidities, and risks of incident vascular diseases and other comorbidities after cohort entry between GCA and non-vasculitis patients...
January 11, 2017: Rheumatology
https://www.readbyqxmd.com/read/28076879/igm-antibodies-to-proteinase-3-in-granulomatosis-with-polyangiitis-and-microscopic-polyangiitis
#17
J M Clain, A M Hummel, J H Stone, F C Fervenza, G S Hoffman, C G M Kallenberg, C A Langford, W J McCune, P A Merkel, P A Monach, P Seo, R F Spiera, E W St Clair, S R Ytterberg, And U Specks
Antineutrophil cytoplasmic antibodies (ANCA) appear to play an important role in the pathogenesis of ANCA-associated vasculitis (AAV). Yet, ANCA alone are not sufficient to generate disease, and some evidence suggests that infectious triggers may serve as inciting events for AAV disease activity. Antibodies of the IgM isotype often serve as markers of recent infection, and IgM ANCA have previously been identified in patients with AAV, though the frequency and clinical relevance of IgM ANCA is not well established...
January 11, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28076570/bilateral-retinal-vasculitis-in-a-patient-with-lichen-planus
#18
Seyhan Dikci, Oğuzhan Genç, Turgut Yılmaz, Penpe Gül Fırat
Lichen planus (LP) is an autoimmune, inflammatory disease of unknown etiology that commonly affects the skin and mucous membranes. Retinal vasculitis is a group of vision-threatening disorders, in which autoimmunity is thought to play a role in pathogenesis. We present the case of a patient who was diagnosed with retinal vasculitis and who was followed up for mucosal LP. LP has not been reported as a cause of retinal vasculitis in the literature. We believe that the retinal vasculitis in this case was related to LP because cellular immunity plays a role in the pathogenesis of both entities...
November 2016: Arquivos Brasileiros de Oftalmologia
https://www.readbyqxmd.com/read/28076021/radiologic-assessment-of-native-renal-vasculature-a-multimodality-review
#19
Sayf Al-Katib, Monisha Shetty, Syed Mohammad A Jafri, Syed Zafar H Jafri
A wide range of clinically important anatomic variants and pathologic conditions may affect the renal vasculature, and radiologists have a pivotal role in the diagnosis and management of these processes. Because many of these entities may not be suspected clinically, renal artery and vein assessment is an essential application of all imaging modalities. An understanding of the normal vascular anatomy is essential for recognizing clinically important anatomic variants. An understanding of the protocols used to optimize imaging modalities also is necessary...
January 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28074992/-antineutrophil-cytoplasmic-antibody-anca-associated-renal-vasculitis-and-pregnancy-report-of-one-case
#20
Jorge Vega, Gonzalo P Méndez
ANCA mediated vasculitis mainly occur between the fourth and fifth decade of life; therefore, it is very uncommon to see pregnant patients with the disease. Vasculitis may affect significantly the course of pregnancy; in turn pregnancy can change the course of vasculitis. We report a 20 years old woman with ANCA-mediated renal vasculitis lasting 10 years who consulted with a pregnancy of 15 weeks. She was in remission and had amenorrhea attributed to ovarian toxicity due to cyclophosphamide. Pregnancy had an uneventful course with spontaneous delivery at the 37th week, giving birth to a healthy newborn...
October 2016: Revista Médica de Chile
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