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Article ; Online: Vasculitic neuropathy-related disability, pain, quality of life, and autonomic symptoms: A survey of 312 patients.

Collins, Michael P / Hadden, Robert D M / Luqmani, Raashid A

2024

Abstract: Objectives: To assess self-reported symptoms of neuropathy, disability, pain, health-related quality of life (HR-QOL), and autonomic dysfunction in patients with vasculitis.: Methods: Patients with vasculitis (with or without neuropathy) were invited ...

Abstract	Objectives: To assess self-reported symptoms of neuropathy, disability, pain, health-related quality of life (HR-QOL), and autonomic dysfunction in patients with vasculitis. Methods: Patients with vasculitis (with or without neuropathy) were invited by Vasculitis UK to complete an anonymous online survey. Results: 312 patients (71% female) responded. Median age was 61-70 years. Median duration of vasculitis was 4 years (<2 months to > 15 years). Vasculitic types included granulomatosis with polyangiitis (34%), unspecified ANCA-associated vasculitis (13%), microscopic polyangiitis (11%), eosinophilic granulomatosis with polyangiitis (11%), giant cell arteritis (10%), non-systemic vasculitic neuropathy (2%), and other (19%). Many patients reported foot/hand symptoms suggestive of neuropathy, including numbness (64%), pain (54%), or weakness (40%). 242 patients (78%) met our definition of probable vasculitic neuropathy: diagnosis of neuropathy by vasculitis team OR numbness OR weakness in feet/hands. Only 52% had been formally diagnosed with neuropathy. Compared with 70 patients without neuropathy, neuropathy patients had greater disability measured by the inflammatory Rasch-built Overall Disability Scale (centile mean 63.1 (SD 17.3) vs 75.2 (16.7); p< 0.0001), Inflammatory Neuropathy Cause and Treatment scale (median 2 (IQR 1-4) vs 0.5 (0-2); p< 0.0001), and modified Rankin scale (median 2 (IQR 1-3) vs 2 (1-2); p= 0.0002); greater pain on an 11-point rating scale (mean 4.6 (SD 2.6) vs 3.5 (2.8); p= 0.0009); and poorer HR-QOL on the EQ5D-3L (summary index mean 0.58 (SD 0.29) vs 0.69 (0.28); p<0.0001). Two-thirds reported autonomic symptoms (not associated with neuropathy). Conclusion: Neuropathy is common and associated with significant disability, pain, and impaired HR-QOL in patients with systemic vasculitis.
Language	English
Publishing date	2024-01-30
Publishing country	England
Document type	Journal Article
ZDB-ID	1464822-2
ISSN	1462-0332 ; 1462-0324
ISSN (online)	1462-0332
ISSN	1462-0324
DOI	10.1093/rheumatology/keae038
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Article: Vasculitis: an update.

Luqmani, Raashid A

British journal of hospital medicine (London, England : 2005)

2014 Volume 75, Issue 8, Page(s) 432–439

Abstract: The systemic vasculitides are uncommon but serious diseases. Early recognition can be difficult because they mimic many conditions. Aggressive immunosuppression is toxic but effective; a targeted approach with biological agents may improve the outcome. ...

Abstract	The systemic vasculitides are uncommon but serious diseases. Early recognition can be difficult because they mimic many conditions. Aggressive immunosuppression is toxic but effective; a targeted approach with biological agents may improve the outcome.
MeSH term(s)	Anti-Inflammatory Agents/therapeutic use ; Diagnosis, Differential ; Humans ; Immunologic Factors/therapeutic use ; Prognosis ; Vasculitis/diagnosis ; Vasculitis/etiology ; Vasculitis/therapy
Chemical Substances	Anti-Inflammatory Agents ; Immunologic Factors
Language	English
Publishing date	2014-08
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Review
ISSN	1750-8460
ISSN	1750-8460
DOI	10.12968/hmed.2014.75.8.432
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Article: Discontinuation of therapies in vasculitis.

Luqmani, Raashid A

Clinical and experimental rheumatology

2013 Volume 31, Issue 4 Suppl 78, Page(s) S93–7

Abstract: For most patients with vasculitis, treatment will result in prevention of mortality and also lead to clinical remission. This increased survival is of course most welcome, but the burden of surviving an episode of acute vasculitis consists of the effects ...

Abstract	For most patients with vasculitis, treatment will result in prevention of mortality and also lead to clinical remission. This increased survival is of course most welcome, but the burden of surviving an episode of acute vasculitis consists of the effects of the disease as well as the adverse events from treatment. Therefore, we have begun to explore the possibility of withdrawing treatment in order to avoid long-term medication toxicities. Whilst this will reduce short-term side effects, if withdrawal leads to subsequent uncontrolled flares of disease, the need for additional therapy may outweigh any benefit from a drug-free holiday. For very mild forms of vasculitis, such as isolated skin vasculitis, the best option may be to avoid treatment altogether. In those patients with vasculitis secondary to an identifiable agent such as drug toxicity or an infectious organism, discontinuing the offending drug or treating the infection will usually resolve or cure the vasculitis. In patients with localised vasculitis, surgical removal of the affected area can be curative. Other forms of vasculitis have a self-limited duration, after which there does not appear to be any clinical evidence of disease, such as is the case for the majority of patients with giant cell arteritis. By contrast, in many forms of vasculitis, especially those associated with the presence of anti-neutrophil cytoplasm antibody (ANCA), relapse occurs in at least half the patients. Where glucocorticoid therapy is used for any length of time, in doses of >5 mg/day, side effects are almost universal. Adding a concomitant agent in the attempt to shorten the course and/or reduce the dose of glucocorticoid treatment may be effective, but can also result in toxicity from the alternative agent, and leaves the patient on immunosuppressive therapy. More toxic therapy, such as cyclophosphamide, usually is administered only for a limited time or cumulative amount, in order to achieve induction of remission or flare in severe disease. The advent of targeted biologic therapy offers the opportunity to provide more effective, less toxic and perhaps more long-lasting control of disease. Rituximab in small-vessel vasculitis can result in long-lasting control of disease, for 18 months or more, from a single course of treatment. Suppression of the interleukin-6 pathway may be effective in large-vessel vasculitis. Unfortunately, none of these therapies is capable of 'cure' for the majority of patients. Therefore, discontinuation of therapy remains unachievable for most patients with vasculitis, at least in the first few years of disease. Short courses of intensive, aggressive therapy are followed by the use of maintenance treatment. Long-term follow-up studies are required to determine the potential benefit of early, more effective control of vasculitis.
MeSH term(s)	Anti-Inflammatory Agents/administration & dosage ; Anti-Inflammatory Agents/adverse effects ; Biological Products/administration & dosage ; Drug Administration Schedule ; Drug Therapy, Combination ; Glucocorticoids/administration & dosage ; Humans ; Patient Selection ; Recurrence ; Remission Induction ; Risk Factors ; Time Factors ; Treatment Outcome ; Vasculitis/diagnosis ; Vasculitis/drug therapy ; Vasculitis/immunology
Chemical Substances	Anti-Inflammatory Agents ; Biological Products ; Glucocorticoids
Language	English
Publishing date	2013-07
Publishing country	Italy
Document type	Journal Article
ZDB-ID	605886-3
ISSN	1593-098X ; 0392-856X
ISSN (online)	1593-098X
ISSN	0392-856X
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Article: Can we identify patients with microscopic polyangiitis who are at risk of relapse?

Luqmani, Raashid A

The Journal of rheumatology

2012 Volume 39, Issue 3, Page(s) 455–457

MeSH term(s)	Asian Continental Ancestry Group ; Cyclophosphamide/therapeutic use ; Female ; Humans ; Immunosuppressive Agents/therapeutic use ; Male ; Microscopic Polyangiitis/drug therapy ; Microscopic Polyangiitis/epidemiology ; Prednisolone/therapeutic use
Chemical Substances	Immunosuppressive Agents ; Cyclophosphamide (8N3DW7272P) ; Prednisolone (9PHQ9Y1OLM)
Language	English
Publishing date	2012-03
Publishing country	Canada
Document type	Comment ; Editorial
ZDB-ID	194928-7
ISSN	1499-2752 ; 0315-162X
ISSN (online)	1499-2752
ISSN	0315-162X
DOI	10.3899/jrheum.111602
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Article: Treat-to-target in vasculitis: is this a sensible approach?

Luqmani, Raashid A

Clinical and experimental rheumatology

2012 Volume 30, Issue 4 Suppl 73, Page(s) S149–53

Abstract: It is possible to achieve substantial initial control of systemic vasculitis in the majority of patients. However, the 'target' has shifted considerably over the last 20-30 years from keeping patients alive to maintaining good quality disease control, ... ...

Abstract	It is possible to achieve substantial initial control of systemic vasculitis in the majority of patients. However, the 'target' has shifted considerably over the last 20-30 years from keeping patients alive to maintaining good quality disease control, avoiding the development of comorbidities - either as a result of disease or treatment, and also preventing relapses. This expansion of potential targets that can be achieved in systemic vasculitis has arisen because we have more effective therapies, but more importantly we have developed a framework within which targets can be created reproducibly. In other words we have much clearer definitions of what constitutes clinical disease activity, relapse, remission and morbidity. These targets are based on simple clinical evaluation, limited laboratory assessments of patients that can be undertaken by any secondary care facility. As a result of this they remain at a clinical level and may not address the most important targets, which are curing disease and that would be the aspiration to move towards. The first step towards that is to move from clinically-based targets towards mechanistic targets based primarily around the pathophysiological drivers of disease. That in turn may lead to identification of specific targets that can turn off disease. The systemic vasculitides are heterogeneous and although for ANCA-associated vasculitis in the short term treatments are similar, the development of clear understanding of mechanisms and new targets may bring with it the promise of much more focused therapies that will address only individual targets and therefore personalize therapy for each individual condition and patient.
MeSH term(s)	Antibodies, Antineutrophil Cytoplasmic/blood ; Antibodies, Monoclonal, Murine-Derived/therapeutic use ; Antirheumatic Agents/therapeutic use ; Cyclophosphamide/therapeutic use ; Humans ; Patient Care Planning ; Recurrence ; Rituximab ; Secondary Prevention/methods ; Severity of Illness Index ; Systemic Vasculitis/drug therapy ; Systemic Vasculitis/physiopathology
Chemical Substances	Antibodies, Antineutrophil Cytoplasmic ; Antibodies, Monoclonal, Murine-Derived ; Antirheumatic Agents ; Rituximab (4F4X42SYQ6) ; Cyclophosphamide (8N3DW7272P)
Language	English
Publishing date	2012-07
Publishing country	Italy
Document type	Journal Article
ZDB-ID	605886-3
ISSN	1593-098X ; 0392-856X
ISSN (online)	1593-098X
ISSN	0392-856X
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Article ; Online: Response to: Correspondence on '2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis' by Joanna C Robson

Grayson, Peter C / Robson, Joanna C / Suppiah, Ravi / Ponte, Cristina / Watts, Richard A / Luqmani, Raashid A / Merkel, Peter A

Annals of the rheumatic diseases

2022 Volume 82, Issue 8, Page(s) e198

MeSH term(s)	Humans ; United States ; Granulomatosis with Polyangiitis ; Microscopic Polyangiitis ; Rheumatology ; Polyarteritis Nodosa
Language	English
Publishing date	2022-03-22
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-2022-222362
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Article ; Online: Reply.

Ponte, Cristina / Grayson, Peter C / Gribbons, Katherine Bates / Robson, Joanna C / Suppiah, Ravi / Judge, Andrew / Watts, Richard A / Luqmani, Raashid A / Merkel, Peter A

Arthritis & rheumatology (Hoboken, N.J.)

2023 Volume 75, Issue 6, Page(s) 1077–1080

Language	English
Publishing date	2023-03-21
Publishing country	United States
Document type	Letter ; Comment
ZDB-ID	2756371-6
ISSN	2326-5205 ; 2326-5191
ISSN (online)	2326-5205
ISSN	2326-5191
DOI	10.1002/art.42448
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Article ; Online: Association Between HLA-DPB1 and Antineutrophil Cytoplasmic Antibody-Associated Vasculitis in Children.

Gibson, Kristen M / Drögemöller, Britt I / Foell, Dirk / Benseler, Susanne M / Graham, Jinko / Hancock, Robert E W / Luqmani, Raashid A / Cabral, David A / Brown, Kelly L / Ross, Colin J

Arthritis & rheumatology (Hoboken, N.J.)

2023 Volume 75, Issue 6, Page(s) 1048–1057

Abstract: Objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare, life-threatening inflammation of blood vessels that can affect both adults and children. Compared to adult-onset disease, AAV is especially rare in children, ... ...

Abstract	Objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare, life-threatening inflammation of blood vessels that can affect both adults and children. Compared to adult-onset disease, AAV is especially rare in children, with an annual prevalence of 0.5-6.4 cases per million children. The etiology of AAV remains largely unknown, and both environmental and genetic factors are likely involved. The present study was undertaken to explore the genetic susceptibility factors recently identified in adult patients, including HLA-DP and HLA-DQ, in pediatric patients. Methods: We performed a genome-wide association study of pediatric AAV in patients of European ancestry (n = 63 AAV cases, n = 315 population-matched controls). Results: We identified a significant genetic association between pediatric AAV and the HLA-DPB104:01 allele (P = 1.5 × 10 Conclusion:* The HLA-DPB1 locus showed an association with pediatric AAV, as similarly shown previously in adult AAV. Despite the difference in the age of onset, these findings suggest that childhood- and adult-onset vasculitis share a common genetic predisposition. The identification of genetic variants contributing to AAV is an important step to improved classification tools and treatment strategies.
MeSH term(s)	Adult ; Humans ; Child ; Antibodies, Antineutrophil Cytoplasmic ; Genome-Wide Association Study ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/genetics ; HLA-DP beta-Chains/genetics ; Genetic Predisposition to Disease ; Peroxidase
Chemical Substances	Antibodies, Antineutrophil Cytoplasmic ; HLA-DPB1 antigen ; HLA-DP beta-Chains ; Peroxidase (EC 1.11.1.7)
Language	English
Publishing date	2023-03-30
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2756371-6
ISSN	2326-5205 ; 2326-5191
ISSN (online)	2326-5205
ISSN	2326-5191
DOI	10.1002/art.42423
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Article ; Online: Autoantibodies Against Lysosome Associated Membrane Protein-2 (LAMP-2) in Pediatric Chronic Primary Systemic Vasculitis.

Gibson, Kristen M / Kain, Renate / Luqmani, Raashid A / Ross, Colin J / Cabral, David A / Brown, Kelly L

Frontiers in immunology

2021 Volume 11, Page(s) 624758

Abstract: Background: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a small vessel vasculitis in adults and children that commonly affects the kidneys. Although the frequent antigenic, and presumed pathogenic, targets of ANCA in AAV ... ...

Abstract	Background: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a small vessel vasculitis in adults and children that commonly affects the kidneys. Although the frequent antigenic, and presumed pathogenic, targets of ANCA in AAV are proteinase-3 (PR3) and myeloperoxidase (MPO), ANCA against lysosome associated membrane protein-2 (LAMP-2), a lesser known ANCA antigen that is expressed on the glomerular endothelium, are present in some adults with AAV-associated renal disease. LAMP-2-ANCA has not been assessed in children with chronic systemic vasculitis, and, if present, would be a potentially valuable biomarker given that treatment decisions for these pediatric patients at diagnosis are largely informed by kidney function. Methods: A custom ELISA, using commercially available reagents, was designed to detect autoantibodies to human LAMP-2 in serum. Sera obtained from 51 pediatric patients at the time of diagnosis of chronic primary systemic vasculitis (predominantly AAV) were screened. LAMP-2-ANCA titers were evaluated for correlation with clinical metrics of disease activity (pediatric vasculitis activity score [pVAS], C-reactive protein [CRP] concentration, and erythrocyte sedimentation rate [ESR]), MPO- and PR3-ANCA titers, and renal function (glomerular filtration rate [GFR], renal-specific pVAS, and serum creatinine concentration). Results: LAMP-2-ANCA (>1,000 ng/ml) were detected in 35% (n = 18) of pediatric systemic vasculitis patients, of which, 10 (20% of all patients) were found to have high positive titers (>1,500 ng/ml). Undetectable or negative titres (<500 ng/ml) were identified in 12% (n = 6) of patients, those with titers between 500 and 1,000 ng/ml were considered low with unknown clinical relevance (53%, n = 27). Although LAMP-2-ANCA titers did not significantly differ between patients with AAV versus ANCA-negative vasculitis, only AAV patients had high concentrations (>1,500 ng/ml) of LAMP-2-ANCA. LAMP-2-ANCA titers did not correlate with measures of disease activity (pVAS, CRP, or ESR) at the time of diagnosis. In contrast, for patients with 12-month post diagnosis follow-up, a negative correlation was observed between the change in GFR (from diagnosis to 12-month follow-up) and LAMP-2-ANCA titer at diagnosis. Conclusions: Moderate to high LAMP-2-ANCA titers were detected in 35% (18/51) of children with chronic systemic vasculitis affecting small-to-medium vessels. Although the highest concentrations of LAMP-2-ANCA in this population were observed in individuals positive for classic ANCA (MPO- or PR3-ANCA), similar to previous reports on adult patients, LAMP-2-ANCA titers do not correlate with classic ANCA titers or with overall disease activity at diagnosis. Renal disease is a common manifestation in systemic small-medium vessel vasculitis (both in adults and children, though more severe in children) and our preliminary data suggest LAMP-2-ANCA at diagnosis may be a risk factor for more severe renal disease.
MeSH term(s)	Adolescent ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/blood ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/immunology ; Autoantibodies/blood ; Autoantibodies/immunology ; Child ; Child, Preschool ; Chronic Disease ; Female ; Humans ; Infant ; Lysosomal-Associated Membrane Protein 2/blood ; Lysosomal-Associated Membrane Protein 2/immunology ; Male
Chemical Substances	Autoantibodies ; LAMP2 protein, human ; Lysosomal-Associated Membrane Protein 2
Language	English
Publishing date	2021-02-03
Publishing country	Switzerland
Document type	Clinical Trial ; Journal Article ; Multicenter Study ; Research Support, Non-U.S. Gov't
ZDB-ID	2606827-8
ISSN	1664-3224 ; 1664-3224
ISSN (online)	1664-3224
ISSN	1664-3224
DOI	10.3389/fimmu.2020.624758
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Article ; Online: Reply.

Micheletti, Robert G / Fuxench, Zelma Chiesa / Craven, Anthea / Luqmani, Raashid A / Watts, Richard A / Merkel, Peter A

Arthritis & rheumatology (Hoboken, N.J.)

2021 Volume 73, Issue 6, Page(s) 1089

Language	English
Publishing date	2021-03-30
Publishing country	United States
Document type	Letter ; Comment
ZDB-ID	2756371-6
ISSN	2326-5205 ; 2326-5191
ISSN (online)	2326-5205
ISSN	2326-5191
DOI	10.1002/art.41661
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