Pulmonology

Vasculitis

Questions discussed in this category

How would you manage a patient with ANCA associated vasculitis who has recurrent flare including pulmonary hemorrhage, persistent MPO antibody positivity while on maintenance rituximab and avacopan regimen even after induction rituximab therapy?

1 Answer available

How would you approach the workup and management of isolated inflammatory subglottic stenosis in a young previously healthy patient that is steroid responsive with a completely negative serologic autoimmune workup?

Would you push for biopsy before deciding on treatment?

1 Answer available

What is your approach to management of pulmonary fibrosis in patients with a history of microscopic polyangiitis who do not have other active organ involvement?

Patient is with past history of glomerulonephritis in remission after rituximab for the past 2 years. +MPO/P-ANCA

1 Answer available

How would you approach a pulmonary-renal PR3+ ANCA vasculitis patient who has persistent re-narrowing of mainstem bronchus after several dilatation and stenting procedures, with other anca features well-controlled on rituximab & avacopan?

5 Answers available

Is there a subset of ANCA vasculitis patients for which you would try plasma exchange?

2 Answers available

Are there patients with granulomatosis with polyangiitis on maintenance rituximab therapy for whom you do not co-administer glucocorticoid therapy?

1 Answer available

Would you stop azathioprine in a patient with ANCA vasculitis who has been in long term remission on azathioprine, but has a new diagnosis of lung cancer requiring initiation of immunotherapy?

2 Answers available

Do you extend the duration of maintenance therapy past 24 months for patients with ANCA glomerulonephritis who have multiple organ involvement?

2 Answers available

What else do you consider in the differential diagnosis for pulmonary-renal syndromes if there is low clinical and serologic evidence of AAV, Goodpasture's or other rheumatologic disease (SLE, RA, APS, Scleroderma)?

2 Answers available

Is there a role for monitoring serum ANCAs to assess ANCA associated vasculitis disease activity?

This question is part of a collaboration with RheumMadness and is in reference to Pathogenic ANCA (RAVE Trial).

2 Answers available

What is your second line therapy for patients with EGPA with mainly pulmonary and sinonasal features who did not respond to mepolizumab 300 mg/month and still require high doses of steroid?

2 Answers available

Would you advise a patient with quiescent vasculitis who flared with the first Covid mRNA vaccine to obtain an additional dose?

Would you suggest switching to a viral vector vaccine, such as J&J?

2 Answers available

What is your preferred regimen for remission induction and maintenance in EGPA with cardiac involvement?

1 Answer available

How do you approach patients with ANCA vasculitis who had partial response, but continue to have disease activity on q 6 month maintenance rituximab and remain on steroids?

Would you change rituximab maintenance dose or schedule?

1 Answer available

How do you choose between oral and IV cyclophosphamide for the treatment of ANCA-associated vasculitis?

1 Answer available

How do you treat an AAV patient with recurrent bronchial stenosis that has not responded to rituximab induction therapy and dilatations?

1 Answer available

Are there AAV patients for whom you would consider combination therapy with cyclophosphamide and rituximab?

1 Answer available

What is considered an adequate treatment period to assess response of AAV induction therapy with rituximab or cyclophosphamide?

1 Answer available

Which EGPA patients are most likely to benefit from treatment with anti-IL-5 agents such as mepolizumab?

1 Answer available

Which ANCA vasculitis patients are better candidates for cyclophosphamide rather than rituximab?

2 Answers available

How does your treatment algorithm differ for drug-induced ANCA vasculitis compared to non drug-induced ANCA vasculitis in cases with severe/organ-threatening manifestations?

1 Answer available

Are there certain subsets of ANCA vasculitis patients for whom you would consider life long maintenance therapy?

2 Answers available

What clinical factors do you use to determine timing of on-demand dosing of rituximab in ANCA vasculitis maintenance therapy?

1 Answer available

Do you recommend on-demand versus fixed-schedule dosing for rituximab maintenance in ANCA vasculitis?

If you use both, how do you decide which to use for a particular patient?

2 Answers available

When do you stop immunosuppressants in patients with GPA?

1 Answer available

Papers discussed in this category

N Engl J Med, 2020 Feb 13

Plasma Exchange and Glucocorticoids in Severe ANCA-Associated Vasculitis.

The New England journal of medicine, 2010-07-15

Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis.

Medicine (Baltimore),

The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort.

The New England journal of medicine, 2010-07-15

Rituximab versus cyclophosphamide for ANCA-associated vasculitis.

Ann. Rheum. Dis., 2018 Apr 25

Comparison of individually tailored versus fixed-schedule rituximab regimen to maintain ANCA-associated vasculitis remission: results of a multicentre, randomised controlled, phase III trial (MAINRITSAN2).

Ann. Rheum. Dis., 2017 May 25

Randomised controlled trial of prolonged treatment in the remission phase of ANCA-associated vasculitis.

Ann. Rheum. Dis., 2018 May 03

Long-term efficacy of remission-maintenance regimens for ANCA-associated vasculitides.

The New England journal of medicine, 2017-05-18

Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis.

Kidney international reports, 2018-03

Combination Therapy With Rituximab and Cyclophosphamide for Remission Induction in ANCA Vasculitis.

Rheumatology (Oxford),

A novel glucocorticoid-free maintenance regimen for anti-neutrophil cytoplasm antibody-associated vasculitis.

Ann Intern Med,

Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial.

Ann Rheum Dis, 2011 Nov 29

Pulse versus daily oral cyclophosphamide for induction of remission in ANCA-associated vasculitis: long-term follow-up.

RMD Open, 2021 Sep

Early experience of COVID-19 vaccination in adults with systemic rheumatic diseases: results from the COVID-19 Global Rheumatology Alliance Vaccine Survey.

Annals of the rheumatic diseases, 2024 Jan 11

Rituximab as maintenance therapy for ANCA-associated vasculitides: pooled analysis and long-term outcome of 277 patients included in the MAINRITSAN trials.

Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases, 2012-08

Cyclophosphamide without corticosteroids for treatment of severe systemic necrotizing vasculitis.

Current medical research and opinion, 2016-12

Corticosteroids: the knee jerk response.

Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association, 2015-05

Risk of lymphoma in patients with inflammatory bowel disease treated with azathioprine and 6-mercaptopurine: a meta-analysis.

J Immunother Cancer, 2021 Jul

Timing of steroid initiation and response rates to immune checkpoint inhibitors in metastatic cancer.

American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons, 2016 Jul 07

Azathioprine and Risk of Skin Cancer in Organ Transplant Recipients: Systematic Review and Meta-Analysis.

Arthritis & rheumatology (Hoboken, N.J.), 2019 Oct 21

Safety and Efficacy of Immune Checkpoint Inhibitors in Patients With Cancer and Preexisting Autoimmune Disease: A Nationwide, Multicenter Cohort Study.

European journal of cancer (Oxford, England : 1990), 2023 Dec 28

Effects of an immunosuppressive therapy on the efficacy of immune checkpoint inhibition in metastatic melanoma - An analysis of the prospective skin cancer registry ADOREG.

Arthritis and rheumatism, 2006 Mar

Association of chronic inflammation, not its treatment, with increased lymphoma risk in rheumatoid arthritis.

Current opinion in pulmonary medicine, 2023 Jun 30

Interstitial lung disease in patients with anti-neutrophil cytoplasm antibody-associated vasculitis: an update on pathogenesis and treatment.

Rheumatology (Oxford, England), 2023 Feb 01

Interstitial lung disease in ANCA-associated vasculitis: associated factors, radiographic features and mortality.

JAMA otolaryngology-- head & neck surgery, 2020 Jan 01

Comparative Treatment Outcomes for Patients With Idiopathic Subglottic Stenosis.

Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery, 2024 Mar 14

Medical Maintenance Therapy Following Laser Excision in Patients With Granulomatosis With Polyangiitis (GPA)-Associated Subglottic Stenosis.

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