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  1. Article ; Online: Differentiating Activity From Damage-The Morphological Challenge of Morphea.

    Saracino, Amanda M / Nikpour, Mandana

    JAMA dermatology

    2023  Volume 159, Issue 3, Page(s) 249–251

    MeSH term(s) Humans ; Scleroderma, Localized/diagnosis ; Skin ; Quality of Life
    Language English
    Publishing date 2023-02-08
    Publishing country United States
    Document type Editorial ; Comment
    ZDB-ID 2701761-8
    ISSN 2168-6084 ; 2168-6068
    ISSN (online) 2168-6084
    ISSN 2168-6068
    DOI 10.1001/jamadermatol.2022.6364
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  2. Article ; Online: Response to: 'Differentiating disease activity from damage in systemic sclerosis: it's still early days!' by Jain and Sharma.

    Nikpour, Mandana

    Annals of the rheumatic diseases

    2019  Volume 79, Issue 8, Page(s) e99

    MeSH term(s) Humans ; Scleroderma, Localized ; Scleroderma, Systemic
    Language English
    Publishing date 2019-05-22
    Publishing country England
    Document type Journal Article ; Comment
    ZDB-ID 7090-7
    ISSN 1468-2060 ; 0003-4967
    ISSN (online) 1468-2060
    ISSN 0003-4967
    DOI 10.1136/annrheumdis-2019-215639
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  3. Article ; Online: Composite measures to assess disease damage, disease severity and treatment response in systemic sclerosis clinical trials.

    Ross, Laura / Nikpour, Mandana

    Current opinion in rheumatology

    2023  Volume 35, Issue 6, Page(s) 334–340

    Abstract: Purpose of review: The aim of this study was to summarize the composite outcome measures available to assess disease damage, severity and treatment response in systemic sclerosis (SSc).: Recent findings: There have been many recent efforts to ... ...

    Abstract Purpose of review: The aim of this study was to summarize the composite outcome measures available to assess disease damage, severity and treatment response in systemic sclerosis (SSc).
    Recent findings: There have been many recent efforts to rigorously develop and validate composite outcome measures to assess the totality of the impact of SSc on patients. All initiatives have used both consensus and data-driven methods. Recently developed are a SSc-specific damage index, a patient-reported disease impact questionnaire (ScleroID) and a treatment response index (ACR-CRISS).
    Summary: Multiorgan, composite measures are being increasingly applied to assess treatment efficacy in clinical trials. Although a fully validated, disease-specific composite measure is not yet available, there has been significant recent progress towards developing measures of treatment response, damage and overall impact of SSc for application in randomized controlled trials.
    Language English
    Publishing date 2023-08-29
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1045317-9
    ISSN 1531-6963 ; 1040-8711
    ISSN (online) 1531-6963
    ISSN 1040-8711
    DOI 10.1097/BOR.0000000000000971
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    Jg. 1995 - 2021: Lesesall (2.OG)
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  4. Article ; Online: Reply to Letter to the Editor: Sclerosis terminology in the spotlight.

    Saracino, Amanda M / Jerjen, Rebekka / Nikpour, Mandana

    Journal of the American Academy of Dermatology

    2023  Volume 89, Issue 4, Page(s) e173–e174

    MeSH term(s) Humans ; Sclerosis ; Terminology as Topic
    Language English
    Publishing date 2023-06-13
    Publishing country United States
    Document type Letter ; Comment
    ZDB-ID 603641-7
    ISSN 1097-6787 ; 0190-9622
    ISSN (online) 1097-6787
    ISSN 0190-9622
    DOI 10.1016/j.jaad.2023.06.009
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  5. Article ; Online: Sicca: an important manifestation of damage in systemic sclerosis (SSc) and SSc-overlap syndromes. Response to 'Do the salivary glands of patients with systemic sclerosis show ultrasonographic modifications suggestive of Sjögren's syndrome?' by Coudrec

    Nikpour, Mandana

    Annals of the rheumatic diseases

    2019  Volume 79, Issue 10, Page(s) e138

    MeSH term(s) Autoimmune Diseases ; Connective Tissue Diseases ; Humans ; Salivary Glands/diagnostic imaging ; Scleroderma, Systemic/complications ; Scleroderma, Systemic/diagnostic imaging ; Sjogren's Syndrome/diagnostic imaging
    Language English
    Publishing date 2019-06-12
    Publishing country England
    Document type Journal Article ; Comment
    ZDB-ID 7090-7
    ISSN 1468-2060 ; 0003-4967
    ISSN (online) 1468-2060
    ISSN 0003-4967
    DOI 10.1136/annrheumdis-2019-215797
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  6. Article ; Online: Diagnosing and characterizing inflammatory myopathies at an Australian tertiary public hospital: Resource utilization and direct healthcare costs.

    Huang, Victoria / Ciciriello, Sabina / Nikpour, Mandana / Oon, Shereen / Day, Jessica

    International journal of rheumatic diseases

    2024  Volume 27, Issue 4, Page(s) e15153

    Abstract: Aim: To determine the direct health service costs and resource utilization associated with diagnosing and characterizing idiopathic inflammatory myopathies (IIMs), and to assess for limitations and diagnostic delay in current practice.: Methods: A ... ...

    Abstract Aim: To determine the direct health service costs and resource utilization associated with diagnosing and characterizing idiopathic inflammatory myopathies (IIMs), and to assess for limitations and diagnostic delay in current practice.
    Methods: A retrospective, single-center cohort analysis of all patients diagnosed with IIMs between January 2012 and December 2021 in a large tertiary public hospital was conducted. Demographics, resource utilization and costs associated with diagnosing IIM and characterizing disease manifestations were identified using the hospital's electronic medical record and Health Intelligence Unit, and the Medicare Benefits Schedule.
    Results: Thirty-eight IIM patients were identified. IIM subtypes included dermatomyositis (34.2%), inclusion body myositis (18.4%), immune-mediated necrotizing myopathy (18.4%), polymyositis (15.8%), and anti-synthetase syndrome (13.2%). The median time from symptom onset to diagnosis was 212 days (IQR: 118-722), while the median time from hospital presentation to diagnosis was 30 days (8-120). Seventy-six percent of patients required emergent hospitalization during their diagnosis, with a median length of stay of 8 days (4-15). The average total cost of diagnosing IIM was $15 618 AUD (STD: 11331) per patient. Fifty percent of patients underwent both MRI and EMG to identify affected muscles, 10% underwent both pan-CT and PET-CT for malignancy detection, and 5% underwent both open surgical and percutaneous muscle biopsies. Autoimmune serology was unnecessarily repeated in 37% of patients.
    Conclusion: The diagnosis of IIMs requires substantial and costly resource use; however, our study has identified potential limitations in current practice and highlighted the need for streamlined diagnostic algorithms to improve patient outcomes and reduce healthcare-related economic burden.
    MeSH term(s) Humans ; Retrospective Studies ; Myositis/diagnosis ; Myositis/economics ; Myositis/therapy ; Male ; Female ; Middle Aged ; Tertiary Care Centers/economics ; Hospitals, Public/economics ; Hospital Costs ; Aged ; Adult ; Health Resources/statistics & numerical data ; Health Resources/economics ; Health Care Costs ; Delayed Diagnosis/economics ; Predictive Value of Tests ; Time Factors ; Australia
    Language English
    Publishing date 2024-04-25
    Publishing country England
    Document type Journal Article
    ZDB-ID 2426924-4
    ISSN 1756-185X ; 1756-1841
    ISSN (online) 1756-185X
    ISSN 1756-1841
    DOI 10.1111/1756-185X.15153
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    Zs.A 6098: Show issues Location:
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  7. Article ; Online: Endobronchial obstruction in connective tissue diseases: an uncommon but life threatening complication: two case reports.

    Shah, Rushab / Lim, Lisa / Nikpour, Mandana

    Journal of medical case reports

    2023  Volume 17, Issue 1, Page(s) 329

    Abstract: Background: Granulomatosis with polyangiitis and relapsing polychondritis are rare, multisystemic and potentially life-threatening connective tissue diseases. We present two cases of severe endobronchial obstruction in the aforementioned conditions and ... ...

    Abstract Background: Granulomatosis with polyangiitis and relapsing polychondritis are rare, multisystemic and potentially life-threatening connective tissue diseases. We present two cases of severe endobronchial obstruction in the aforementioned conditions and discuss difficulties with detection and treatment. Despite differing underlying pathophysiologies, endobronchial disease is a less frequently reported but serious complication of both conditions.
    Case presentation: Case 1, a 31-year-old South Asian woman with relapsing polychondritis, required partial tracheal resection and reconstruction in combination with immunosuppressive therapy to achieve respiratory recovery following collapse of her right main bronchus and a stricture in her left main bronchus. Case 2, a 22-year-old Caucasian male with granulomatosis with polyangiitis, underwent surgical resection of an endobronchial growth causing occlusion of his right main bronchus. Although his respiratory status was initially stabilised with increased immunosuppression, he continues to have disease progression in spite of this.
    Conclusions: Our cases highlight the importance of a multidisciplinary approach combining immunosuppression with supportive care and judicious use of surgical interventions in select cases. A further review of the literature shows endobronchial obstruction is potentially under-reported due to overlap in connective tissue disease symptomatology and there is no consensus on best practice.
    MeSH term(s) Female ; Humans ; Male ; Adult ; Young Adult ; Polychondritis, Relapsing/complications ; Polychondritis, Relapsing/surgery ; Granulomatosis with Polyangiitis/complications ; Bronchial Diseases/surgery ; Bronchial Diseases/complications ; Airway Obstruction/etiology ; Airway Obstruction/surgery ; Bronchi/diagnostic imaging
    Language English
    Publishing date 2023-08-02
    Publishing country England
    Document type Review ; Case Reports ; Journal Article
    ZDB-ID 2269805-X
    ISSN 1752-1947 ; 1752-1947
    ISSN (online) 1752-1947
    ISSN 1752-1947
    DOI 10.1186/s13256-023-04058-x
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  8. Article ; Online: Response to: 'Remission in systemic lupus erythematosus: durable remission is rare' by Wilhelm et al.

    Nikpour, Mandana

    Annals of the rheumatic diseases

    2016  Volume 75, Issue 12, Page(s) e79

    MeSH term(s) Humans ; Lupus Erythematosus, Systemic ; Severity of Illness Index
    Language English
    Publishing date 2016
    Publishing country England
    Document type Letter ; Research Support, Non-U.S. Gov't ; Comment
    ZDB-ID 7090-7
    ISSN 1468-2060 ; 0003-4967
    ISSN (online) 1468-2060
    ISSN 0003-4967
    DOI 10.1136/annrheumdis-2016-210560
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  9. Article ; Online: Gastrointestinal Tract Considerations Part I: How Should a Rheumatologist Best Manage Common Upper Gastrointestinal Tract Complaints in Systemic Sclerosis?

    Quinlivan, Alannah / McMahan, Zsuzsanna H / Lee, Eun Bong / Nikpour, Mandana

    Rheumatic diseases clinics of North America

    2023  Volume 49, Issue 2, Page(s) 295–318

    Abstract: The upper gastrointestinal (GI) tract is frequently involved in systemic sclerosis (SSc) and may impact quality of life, physical function and survival. Although we are currently very proactive in terms of screening for heart and lung involvement, ... ...

    Abstract The upper gastrointestinal (GI) tract is frequently involved in systemic sclerosis (SSc) and may impact quality of life, physical function and survival. Although we are currently very proactive in terms of screening for heart and lung involvement, patients with SSc are not routinely screened for GI involvement. This review details the available investigations for common upper GI symptoms in SSc, including dysphagia, reflux and bloating and provides advice as to how to integrate these investigations into current clinical care.
    MeSH term(s) Humans ; Rheumatologists ; Quality of Life ; Scleroderma, Systemic/complications ; Gastrointestinal Diseases/diagnosis ; Gastrointestinal Diseases/etiology ; Gastrointestinal Diseases/therapy ; Gastrointestinal Tract ; Upper Gastrointestinal Tract
    Language English
    Publishing date 2023-02-28
    Publishing country United States
    Document type Journal Article ; Review ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ZDB-ID 92118-x
    ISSN 1558-3163 ; 0889-857X
    ISSN (online) 1558-3163
    ISSN 0889-857X
    DOI 10.1016/j.rdc.2023.01.006
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  10. Article ; Online: Endobronchial obstruction in connective tissue diseases

    Rushab Shah / Lisa Lim / Mandana Nikpour

    Journal of Medical Case Reports, Vol 17, Iss 1, Pp 1-

    an uncommon but life threatening complication: two case reports

    2023  Volume 4

    Abstract: Abstract Background Granulomatosis with polyangiitis and relapsing polychondritis are rare, multisystemic and potentially life-threatening connective tissue diseases. We present two cases of severe endobronchial obstruction in the aforementioned ... ...

    Abstract Abstract Background Granulomatosis with polyangiitis and relapsing polychondritis are rare, multisystemic and potentially life-threatening connective tissue diseases. We present two cases of severe endobronchial obstruction in the aforementioned conditions and discuss difficulties with detection and treatment. Despite differing underlying pathophysiologies, endobronchial disease is a less frequently reported but serious complication of both conditions. Case presentation Case 1, a 31-year-old South Asian woman with relapsing polychondritis, required partial tracheal resection and reconstruction in combination with immunosuppressive therapy to achieve respiratory recovery following collapse of her right main bronchus and a stricture in her left main bronchus. Case 2, a 22-year-old Caucasian male with granulomatosis with polyangiitis, underwent surgical resection of an endobronchial growth causing occlusion of his right main bronchus. Although his respiratory status was initially stabilised with increased immunosuppression, he continues to have disease progression in spite of this. Conclusions Our cases highlight the importance of a multidisciplinary approach combining immunosuppression with supportive care and judicious use of surgical interventions in select cases. A further review of the literature shows endobronchial obstruction is potentially under-reported due to overlap in connective tissue disease symptomatology and there is no consensus on best practice.
    Keywords Airway obstruction ; Relapsing polychondritis ; Granulomatosis with polyangiitis ; Medicine ; R
    Subject code 610
    Language English
    Publishing date 2023-08-01T00:00:00Z
    Publisher BMC
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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