LUPUS NEPHRITIS:Clinical Pearls

DR. ABDELAZEIM ELHEFNY MDPROF. OF RHEUMATOLOGY & IMMUNOLOGY

AIN SHAMS UNIVERSITY

LUPUS NEPHRITIS

•Kidney involvement in SLE can range from mild

to severe and occurs in 50%-70% of patients with lupus.

• Despite advances in therapy, morbidity and mortalityremain high.

• In some studies, lupus nephritis leads to ESRD in 17%-25%of patients and also is associated with increased risk for CVevents.

Cervera el al., Medicine (Baltimore). 2003;82(5):299-308.

A prolonged exposure of C1q epitopes to the immune system could lead to an autoimmune response against itself.

?

for exact diagnosis; classification & guiding therapy of LN, but LN is not a renal biopsy diagnosis.

MANIFESTATIONS OF LN

Total score = 15

confirmed in

• Systolic BP goal between 110 - 129 mm Hg may be beneficial in patients with urine protein

excretion >1.0 g/d.

• Systolic BP <110 mm Hg may be associated with a higher risk for kidney disease progression.

Relative Risk of CKD

Hydroxychloroquinemay protect

against

Onset of LN

Relapses ofLN

ESRD

Vascular thrombo

sis

PGA= Physician/pt global assessment

• There was no evidence of additional benefit of

25(OH)D beyond a level of 40 ng/ml.

LUPUS & Pregnancy

Never MMF, stop 3 month before conception,If taking MMF shift to AZA & wait for 3 month to start peg

For 6 months

mg

Ruiz-Irastorza, et al., Autoimmunity Reviews. 2014: 13; 206-214.

Imperial College London Lupus center

RITUXILUP protocol

Steroid avoiding regimen:

Used since 1.1.2006 in all new/relapsing LN who are not already on steroids and do not have RPGN/ cerebral lupus.

Established as our first line treatment protocol:• MP 500mg IV + rituximab 1g – d1 & d15

• MMF – start at 500 mg bid & titrate to trough levels 1.4 – 2.4 mg/l

NO ORAL STEROIDS.

RITUXILUP

• Rituxilup regimen leads to remission, preservation of renal function & minimal oral steroid use in a significant proportion of patients.

• Relapses were only in patients with class IV or V disease, majority responded to retreatment, again with no oral steroids.

• Flares were not uncommon but did not predict poor outcomes.

• Poor outcomes were predicted by baseline creatinine > 1.4 mg/dl or a failure to achieve partial renal remission (PR) at 6 months.

• The minimal use of oral steroids in the majority would be expected to have long term benefits in terms of CV risk & reduced side effects.

Less treatment failure & less renal flare.

1.4 – 2.4 mg/l

The Future?

• Multi-target therapy: adding biologics or CNI

to MMF.

• Tracking disease activity without renal

biopsy.

• Preventing renal fibrosis.

CONCLUSION I note that the present and the future are brighter for lupus nephritis.

Previously, LN was the primary cause of death in SLE. Today, we realize that it is not as common as thought even a few years ago, yet it still does affect around 50% of patients.

To our patients: make sure to take your hydroxychloroquine every day, take vitamin D regularly to keep your blood 25-OH vitamin D level 40 ng/mL, and use your sunscreen religiously every day (even if you don’t go outside) in the hopes of decreasing your chances of getting nephritis.

See your rheumatologist at least every few months & give a urine sample to look for LN, even if you feel perfectly fine (remember that most people with nephritis do not have any symptoms at all) in the hopes of catching lupus nephritis in the earliest stages possible.

In the near future, we will have newer and better therapies for LN and will hopefully be able to use much lower doses of steroids in its treatment; while we await the discovery of a cure for this disease.