Lupus Management After Failure of First-Line Treatment Save

Janet Pope, MD, MPH, FRCPC

Jun 18, 2015 8:47 am

There are no detailed recommendations agreed upon by SLE experts on what treatment to use after first and second (and often third) line treatment has failed.

In an attempt to fill in the knowledge gap for SLE treatment, several SLE experts were polled. Using cases that were organ-based, experts were asked to write what they would do first, then second, then third, etc., assuming all treatment was available, tolerated and enough time was given to determine the treatment was not successful.

This is in press in Arthritis Care & Research ("Treatment algorithms in systemic lupus erythematosus," by Muangchan C, et al). What was striking is that some organs had very good agreement despite lack of RCT data (myocarditis, ILD, vasculitis) whereas other treatment strategies had some RCT data but poor agreement (APS, ITP). Also rituximab treatment that is not approved in SLE (with negative RCT data) was frequently in the treatment algorithm. The take home message is that there is a need to continue developing treatment strategies in SLE that are both evidence and eminence based with the knowledge that the options used and doses of steroids will vary with the organ involved and the severity/activity of the patient. These are artificial scenarios in that many patients may have more than one organ involved with disease activity and may be partial responders to treatment or have side effects or comorbidities so treatment cannot be increased, but the consensus in various organs can help guide treatment particularly for those who are not expert in SLE treatment.

Treatment algorithm for organ system involvements by systemic lupus erythematosus

Organ involvement	Treatment options				Ancillary therapy	% Agreement (median)
Organ involvement	1st-line or Induction	2nd-line or Failure of induction	3rd-line	Maintenance	Ancillary therapy	% Agreement (median)
1. Constitutional symptoms	GC, HCQ, IMM or combinations	MMF	Switching to RTX or BLM	N/A	N/A	60
2. Widespread DLE	HCQ ± GC	Adding AZA or switching antimalarial	Switching AZA to MMF or MTX	N/A	Sun screening + Topical steroids or Topical tacrolimus	70
3. Uncomplicated digital/ cutaneous vasculitis	GC ± HCQ ± MTX	AZA or MMF	Switching to IV CYC	N/A	N/A	80
4. Gangrenous digital/ cutaneous vasculitis	GC + IV CYC	Adding RTX or PLAX	N/A	AZA or MMF	PGA	90
5. Non-erosive, non-deforming polyarthritis	HCQ ± GC	Adding MTX	Adding RTX	N/A	NSAIDs	80
6. Lupus pericarditis	GC ± HCQ	Adding MMF or AZA or MTX	Adding BLM or RTX	N/A	Pericardiocentesis ± window	75
7. Lupus myocarditis	GC + IV CYC ± HCQ	Adding RTX or BLM or IVIG	N/A	MMF	N/A	90
8. Lupus ILD	GC + MMF or IV CYC	Adding RTX or IVIG	N/A	AZA or MMF	N/A	90
9. Lupus PAH	GC + IV CYC or MMF + ERA	Adding RTX, and PDE5i	Adding PGA	MMF	N/A	80
10. Lupus thrombocytopenia	GC ± HCQ	Adding AZA or MMF	Adding RTX or IV CYC or IVIG	N/A	Splenectomy	50
11. Lupus- APS- venous thrombosis	Anticoagulation (Warfarin or Low molecular weight heparin) ± HCQ	Direct thrombin inhibitor	N/A	N/A	N/A	60
12. Lupus- APS- arterial thrombosis	Anticoagulation (Warfarin or Low molecular weight heparin) ± HCQ	Adding ASA or Dipyridamole or PAI	N/A	N/A	N/A	70
13. Lupus mononeuritis multiplex	GC + IV CYC	Adding RTX or IVIG or PLAX	N/A	AZA or MMF	N/A	75
14. CNS lupus	GC + IV CYC	Adding RTX or IVIG or PLAX	N/A	AZA or MMF	N/A	60
15. LN type III / IV	GC + MMF	IV CYC	Adding RTX	MMF	HCQ, ACEI	70
16. LN type V	GC + MMF	Switching to AZA or IV CYC or RTX	N/A	MMF	HCQ, ACEI	80

Abbreviation: systemic glucocorticoids: GC; hydroxychloroquine: HCQ; methotrexate: MTX; immunomodulators: IMM i.e. HCQ or low dose weekly MTX; azathioprine: AZA; mycophenolate mofetil: MMF; cyclophosphamide: CYC; rituximab: RTX; belimumab: BLM; plasmapheresis: PLAX; intravenous immunoglobulins: IVIG; endothelin receptor antagonist: ERA; phosphodiesterase-5 inhibitor: PDE5i; prostanoids / prostaglandins analogues: PGA; platelets aggregation inhibitor: PAI; angiotensin converting enzyme inhibitor: ACEI; NSAIDs: nonsteroidal anti-inflammatory drugs; not available: N/A

Disclosures

The author has no conflicts of interest to disclose related to this subject

Janet Pope, MD, MPH, FRCPC

(1 Posts)

Dr. Janet Pope is a Professor of Medicine in the Division of Rheumatology at the University of Western Ontario (UWO), Schulich School of Medicine, London, Ontario, Canada. She is the Division Head in Rheumatology at St. Joseph's Health Centre, London. Her research includes studies in scleroderma, SLE and RA, including outcome measurements, clinical trials and disease manifestations. She has published over 450 peer-reviewed articles, 15 chapters, 500 abstracts and several Cochrane meta-analysis reviews. Mentoring of research students and trainees numbers more than 125. She has received the Distinguished Investigator Award from the Canadian Rheumatology Association, Rheumatologist of the Year from the Ontario Rheumatology Association, Department of Medicine Research Achievement Award, and the Dean’s Award of Excellence in Research. She has been inducted into the Canadian Academy of Health Sciences.

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