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  1. Article: A case of acute generalized exanthematous pustulosis associated with polyarteritis nodosa, responding to systemic steroids.

    Alkhachroum, Ayham M / Kazzaz, Nayef

    Journal of community hospital internal medicine perspectives

    2015  Volume 5, Issue 2, Page(s) 26645

    Abstract: A patient with a known biopsy of polyarteritis nodosa diagnosis presented with cyclic fevers, acute kidney injury, and progression of rash from macular to pustular, worsening despite being on antibiotics, without evidence of infection on multiple ... ...

    Abstract A patient with a known biopsy of polyarteritis nodosa diagnosis presented with cyclic fevers, acute kidney injury, and progression of rash from macular to pustular, worsening despite being on antibiotics, without evidence of infection on multiple cultures. The patient had a pathological diagnosis from a skin biopsy of acute generalized exanthematous pustulosis syndrome, with a total resolution of rash, fevers, and acute kidney injury on treatment with pulse steroids.
    Language English
    Publishing date 2015-04-01
    Publishing country United States
    Document type Case Reports
    ZDB-ID 2616884-4
    ISSN 2000-9666
    ISSN 2000-9666
    DOI 10.3402/jchimp.v5.26645
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: A case of acute generalized exanthematous pustulosis associated with polyarteritis nodosa, responding to systemic steroids

    Ayham M. Alkhachroum / Nayef Kazzaz

    Journal of Community Hospital Internal Medicine Perspectives, Vol 5, Iss 2, Pp 1-

    2015  Volume 5

    Abstract: A patient with a known biopsy of polyarteritis nodosa diagnosis presented with cyclic fevers, acute kidney injury, and progression of rash from macular to pustular, worsening despite being on antibiotics, without evidence of infection on multiple ... ...

    Abstract A patient with a known biopsy of polyarteritis nodosa diagnosis presented with cyclic fevers, acute kidney injury, and progression of rash from macular to pustular, worsening despite being on antibiotics, without evidence of infection on multiple cultures. The patient had a pathological diagnosis from a skin biopsy of acute generalized exanthematous pustulosis syndrome, with a total resolution of rash, fevers, and acute kidney injury on treatment with pulse steroids.
    Keywords AGEP ; PAN ; Systemic Steroids ; Skin Lesions ; Pustules ; Internal medicine ; RC31-1245
    Language English
    Publishing date 2015-04-01T00:00:00Z
    Publisher Taylor & Francis Group
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  3. Article: Intercellular Interactions as Regulators of NETosis.

    Kazzaz, Nayef M / Sule, Gautam / Knight, Jason S

    Frontiers in immunology

    2016  Volume 7, Page(s) 453

    Abstract: Neutrophil extracellular traps (NETs) are chromatin-derived webs extruded from neutrophils in response to either infection or sterile stimulation with chemicals, cytokines, or microbial products. The vast majority of studies have characterized NET ... ...

    Abstract Neutrophil extracellular traps (NETs) are chromatin-derived webs extruded from neutrophils in response to either infection or sterile stimulation with chemicals, cytokines, or microbial products. The vast majority of studies have characterized NET release (also called NETosis) in pure neutrophil cultures
    Language English
    Publishing date 2016
    Publishing country Switzerland
    Document type Review ; Journal Article
    ZDB-ID 2606827-8
    ISSN 1664-3224
    ISSN 1664-3224
    DOI 10.3389/fimmu.2016.00453
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article: Do neutrophil extracellular traps contribute to the heightened risk of thrombosis in inflammatory diseases?

    Rao, Ashish N / Kazzaz, Nayef M / Knight, Jason S

    World journal of cardiology

    2015  Volume 7, Issue 12, Page(s) 829–842

    Abstract: Thrombotic events, both arterial and venous, are a major health concern worldwide. Further, autoimmune diseases, such as systemic lupus erythematosus, anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, and antiphospholipid syndrome, ... ...

    Abstract Thrombotic events, both arterial and venous, are a major health concern worldwide. Further, autoimmune diseases, such as systemic lupus erythematosus, anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, and antiphospholipid syndrome, predispose to thrombosis, and thereby push the risk for these morbid events even higher. In recent years, neutrophils have been identified as important players in both arterial and venous thrombosis. Specifically, chromatin-based structures called neutrophil extracellular traps (NETs) play a key role in activating the coagulation cascade, recruiting platelets, and serving as scaffolding upon which the thrombus can be assembled. At the same time, neutrophils and NETs are emerging as important mediators of pathogenic inflammation in the aforementioned autoimmune diseases. Here, we first review the general role of NETs in thrombosis. We then posit that exaggerated NET release contributes to the prothrombotic diatheses of systemic lupus erythematosus, ANCA-associated vasculitis, and antiphospholipid syndrome.
    Language English
    Publishing date 2015-10-23
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 2573665-6
    ISSN 1949-8462
    ISSN 1949-8462
    DOI 10.4330/wjc.v7.i12.829
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: A 37-Year-Old Man With Primary Antiphospholipid Syndrome Presenting With Respiratory Distress and Worsening Toe Ischemia.

    Kazzaz, Nayef M / Wilson, Allecia M / Kado, Ruba / Barnes, Geoffrey D / Knight, Jason S

    Arthritis care & research

    2017  Volume 69, Issue 8, Page(s) 1253–1259

    MeSH term(s) Adult ; Antiphospholipid Syndrome/complications ; Antiphospholipid Syndrome/diagnosis ; Antiphospholipid Syndrome/therapy ; Coronary Artery Disease/diagnosis ; Coronary Artery Disease/etiology ; Coronary Artery Disease/therapy ; Disease Progression ; Dyspnea/diagnosis ; Dyspnea/etiology ; Dyspnea/therapy ; Fatal Outcome ; Hemorrhage/diagnosis ; Hemorrhage/etiology ; Hemorrhage/therapy ; Humans ; Ischemia/diagnosis ; Ischemia/etiology ; Ischemia/therapy ; Lung Diseases/diagnosis ; Lung Diseases/etiology ; Lung Diseases/therapy ; Male ; Toes/blood supply
    Language English
    Publishing date 2017-08-29
    Publishing country United States
    Document type Case Reports ; Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ZDB-ID 645059-3
    ISSN 2151-4658 ; 0893-7524 ; 2151-464X
    ISSN (online) 2151-4658
    ISSN 0893-7524 ; 2151-464X
    DOI 10.1002/acr.23168
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Treatment of catastrophic antiphospholipid syndrome.

    Kazzaz, Nayef M / McCune, W Joseph / Knight, Jason S

    Current opinion in rheumatology

    2016  Volume 28, Issue 3, Page(s) 218–227

    Abstract: Purpose of review: Catastrophic antiphospholipid syndrome (CAPS) is a severe manifestation of antiphospholipid syndrome (APS). Although affecting only 1% of patients with APS, the condition is frequently fatal if not recognized and treated early. Here, ... ...

    Abstract Purpose of review: Catastrophic antiphospholipid syndrome (CAPS) is a severe manifestation of antiphospholipid syndrome (APS). Although affecting only 1% of patients with APS, the condition is frequently fatal if not recognized and treated early. Here, we will review the current approach to diagnosis and treatment of CAPS.
    Recent findings: Data from the international 'CAPS registry', spearheaded by the European Forum on Antiphospholipid Antibodies, have improved our understanding of at-risk patients, typical clinical features, and precipitating diagnoses. Current guidelines also continue to support the role of anticoagulants and glucocorticoids as foundation therapy in all patients. Finally, new basic science and case series suggest that novel therapies, such as rituximab and eculizumab, warrant further study.
    Summary: Attention to associated diagnoses, such as infection and systemic lupus erythematosus (SLE), is critical at the time of diagnosis. All patients should be treated with anticoagulants, corticosteroids, and possibly plasma exchange. In patients with SLE, cyclophosphamide should be considered. In refractory or relapsing cases, new therapies, such as rituximab and possibly eculizumab, may be options, but need further study.
    MeSH term(s) Anticoagulants/therapeutic use ; Antiphospholipid Syndrome/therapy ; Catastrophic Illness/therapy ; Disease Management ; Glucocorticoids/therapeutic use ; Humans ; Plasma Exchange/methods ; Practice Guidelines as Topic ; Recurrence
    Chemical Substances Anticoagulants ; Glucocorticoids
    Language English
    Publishing date 2016-05
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 1045317-9
    ISSN 1531-6963 ; 1040-8711
    ISSN (online) 1531-6963
    ISSN 1040-8711
    DOI 10.1097/BOR.0000000000000269
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article: Systemic lupus erythematosus complicated by diffuse alveolar haemorrhage: risk factors, therapy and survival.

    Kazzaz, Nayef M / Coit, Patrick / Lewis, Emily E / McCune, W Joseph / Sawalha, Amr H / Knight, Jason S

    Lupus science & medicine

    2015  Volume 2, Issue 1, Page(s) e000117

    Abstract: Objectives: While diffuse alveolar haemorrhage (DAH) is recognised as a life-threatening complication of systemic lupus erythematosus (SLE), little is known about its risk factors and response to treatment. We describe 22 cases of DAH in a US lupus ... ...

    Abstract Objectives: While diffuse alveolar haemorrhage (DAH) is recognised as a life-threatening complication of systemic lupus erythematosus (SLE), little is known about its risk factors and response to treatment. We describe 22 cases of DAH in a US lupus cohort of approximately 1000 patients, and compare them to 66 controls from the same outpatient cohort.
    Methods: We captured variables pertaining to diagnoses of SLE and secondary antiphospholipid syndrome (APS), and analysed them by univariate testing. Those variables with p values <0.05 were then further considered in a multivariate model. Kaplan-Meier curves were constructed for each group, and survival was analysed by Log-rank test.
    Results: Of the 22 patients with DAH, 59% were diagnosed with DAH within 5 years of lupus diagnosis. By univariate testing, several manifestations of SLE and APS were more common in patients with DAH, including history of thrombocytopenia, cardiac valve disease, low C3, leucopenia, neuropsychiatric features, haemolysis, arterial thrombosis, lupus anticoagulant, secondary APS and low C4. On multivariate analysis, history of thrombocytopenia and low C3 were maintained as independent risk factors. Importantly, only two patients had platelet counts <50 000/µL at the time of the DAH episode, arguing that DAH was not simply a haemorrhagic complication of thrombocytopenia. All patients were treated with increased immunosuppression, including various combinations of corticosteroids, plasmapheresis, cyclophosphamide, rituximab and mycophenolate mofetil. Notably, all patients in the cohort survived their initial episode of DAH. While the patients with DAH did well in the short-term, their long-term survival was significantly worse than controls. Several of the deaths were attributable to thrombotic complications after recovering from DAH.
    Conclusions: To the best of our knowledge, this is the largest case-control study of lupus DAH to date. History of thrombocytopenia was strongly predictive of DAH (OR ∼40). A number of APS manifestations correlated with DAH by univariate analysis, and deserve further consideration in larger studies.
    Language English
    Publishing date 2015
    Publishing country England
    Document type Journal Article
    ZDB-ID 2779620-6
    ISSN 2053-8790
    ISSN 2053-8790
    DOI 10.1136/lupus-2015-000117
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: Endothelial progenitor dysfunction associates with a type I interferon signature in primary antiphospholipid syndrome.

    Grenn, Robert C / Yalavarthi, Srilakshmi / Gandhi, Alex A / Kazzaz, Nayef M / Núñez-Álvarez, Carlos / Hernández-Ramírez, Diego / Cabral, Antonio R / McCune, W Joseph / Bockenstedt, Paula L / Knight, Jason S

    Annals of the rheumatic diseases

    2017  Volume 76, Issue 2, Page(s) 450–457

    Abstract: Objectives: Patients with antiphospholipid syndrome (APS) are at risk for subclinical endothelial injury, as well as accelerated atherosclerosis. In the related disease systemic lupus erythematosus, there is a well-established defect in circulating ... ...

    Abstract Objectives: Patients with antiphospholipid syndrome (APS) are at risk for subclinical endothelial injury, as well as accelerated atherosclerosis. In the related disease systemic lupus erythematosus, there is a well-established defect in circulating endothelial progenitors, which leads to an accrual of endothelial damage over time. This defect has been at least partially attributed to exaggerated expression of type I interferons (IFNs). We sought to determine whether these pathways are important in APS.
    Methods: We studied 68 patients with primary APS. Endothelial progenitors were assessed by flow cytometry and functional assay. Type I IFN activity was determined by a well-accepted bioassay, while peripheral blood mononuclear cells were scored for expression of IFN-responsive genes.
    Results: Endothelial progenitors from patients with APS demonstrated a marked defect in the ability to differentiate into endothelial cells, a phenotype which could be mimicked by treating control progenitors with APS sera. Elevated type I IFN activity was detected in the circulation of patients with APS (a finding that was then replicated in an independent cohort). While IgG depletion from APS sera did not rescue endothelial progenitor function, the dysfunction was successfully reversed by a type I IFN receptor-neutralising antibody.
    Conclusions: We describe, for the first time to our knowledge, an IFN signature in primary APS and show that this promotes impaired endothelial progenitor function. This work opens the door to novel approaches that may mitigate vascular damage in APS, such as anti-IFN drugs.
    MeSH term(s) Adult ; Aged ; Antibodies, Neutralizing/pharmacology ; Antiphospholipid Syndrome/immunology ; Antiphospholipid Syndrome/physiopathology ; Case-Control Studies ; Cell Differentiation/drug effects ; Cell Differentiation/physiology ; Endothelial Progenitor Cells/drug effects ; Endothelial Progenitor Cells/physiology ; Female ; Flow Cytometry ; Humans ; Interferon Type I/immunology ; Interferon-alpha/immunology ; Leukocytes, Mononuclear/immunology ; Male ; Middle Aged ; Receptor, Interferon alpha-beta/antagonists & inhibitors ; Young Adult
    Chemical Substances Antibodies, Neutralizing ; IFNAR2 protein, human ; Interferon Type I ; Interferon-alpha ; Receptor, Interferon alpha-beta (156986-95-7)
    Language English
    Publishing date 2017-02
    Publishing country England
    Document type Journal Article
    ZDB-ID 7090-7
    ISSN 1468-2060 ; 0003-4967
    ISSN (online) 1468-2060
    ISSN 0003-4967
    DOI 10.1136/annrheumdis-2016-209442
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article: Cardiovascular complications of systemic lupus erythematosus: impact of risk factors and therapeutic efficacy-a tertiary centre experience in an Appalachian state.

    McVeigh, Elise Danielle / Batool, Amna / Stromberg, Arnold / Abdel-Latif, Ahmed / Kazzaz, Nayef Mohammed

    Lupus science & medicine

    2021  Volume 8, Issue 1

    Abstract: ... Appalachian community was reported at 33 kg/m: Methods: We identified 20 UKHS patients having both a lupus ...

    Abstract Objectives: Cardiovascular complications became a notable cause of morbidity and mortality in patients with lupus as therapeutic advancements became more efficient at managing other complications. The Appalachian community in Kentucky has a higher prevalence of traditional cardiovascular risk factors, predisposing them to cardiovascular events. Namely, the mean body mass index of the members of the Kentucky Appalachian community was reported at 33 kg/m
    Methods: We identified 20 UKHS patients having both a lupus diagnosis and experienced at least one cardiovascular event. We chose three controls matched for birth-year ±5 years to each case. In a case-control design, we analysed lupus manifestations, cardiovascular risk factors and immunosuppressive therapies. We collected Systemic Lupus Erythematosus Disease Activity Index 2000 disease activity index during the cardiovascular event.
    Results: We identified 308 patients with lupus from among all University of Kentucky Health System patients. 20 (6.5%) of such patients with lupus were confirmed to cardiovascular complication. Of those 20, 7 (35%) had experienced myocardial infarction, 10 (50%) had experienced stroke and 4 (20%) had peripheral ischaemia. Tobacco use and male gender were the only traditional cardiovascular risk factors higher in the cases group. Hydroxychloroquine and steroids were less utilised in the cases than in the controls (70% vs 100% in hydroxychloroquine, 30% vs 82% in steroids). Venous thrombosis was found to be significantly higher in the cases. On multivariate analysis, venous thrombosis remained significant.
    Conclusion: Despite tobacco use partially explaining the increased risk of cardiovascular disease among the cases group, the higher prevalence of venous thrombosis in the cases group suggests lupus as a potential additional risk factor of cardiovascular morbidity among patients with lupus in this Appalachian community.
    MeSH term(s) Appalachian Region ; Cardiovascular Diseases/etiology ; Female ; Humans ; Hydroxychloroquine ; Kentucky/epidemiology ; Lupus Erythematosus, Systemic/complications ; Male ; Risk Factors
    Chemical Substances Hydroxychloroquine (4QWG6N8QKH)
    Language English
    Publishing date 2021-05-11
    Publishing country England
    Document type Journal Article ; Research Support, N.I.H., Extramural
    ZDB-ID 2779620-6
    ISSN 2053-8790
    ISSN 2053-8790
    DOI 10.1136/lupus-2020-000467
    Database MEDical Literature Analysis and Retrieval System OnLINE

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