NP24 Nephrology

Parenchymal Kidney Diseases

Essential Med Notes 2015

Systemic Lupus Erythematosus

lupus nephritis can present as any of the glomerular syndromes
nephrotic syndrome with an active sediment is most common presentation
GN caused by immune complex deposition in capillary loops and mesangium with resulting
renal injury
serum complement levels are usually low during periods of active renal disease
children and males with SLE are more likely to develop nephritis
SLE Classification
Class I

Class II

Class III

Class IV

Class V

Class VI

Minimal
mesangial
lupus nephritis

Mesangial
proliferative
lupus nephritis

Focal
lupus nephritis

Diffuse
lupus nephritis

Membranous
lupus nephritis

Advanced sclerotic
lupus nephritis

Treatment

Treatment

Treatment

Treatment

Treatment

Class I and II do not

need treatment directed
at renal lesions

Lowest possible
dose of steroids
and observation

Steroids
Steroids
+
(controversial)
cytotoxic drugs
(consider dialysis or renal
transplant with severe disease)

ESRD
planning

Figure 15. International Society of Nephrology/Renal Pathology Society classification of

lupus nephritis 2003

Henoch-Schnlein Purpura
seen more commonly in children
purpura on buttocks and legs, abdominal pain, arthralgia, and fever
glomeruli show varying degrees of mesangial hypercellularity
IgA and C3 staining of mesangium
usually benign, self-limiting course, 10% progress to CKD
Goodpastures Disease
antibodies against type IV collagen present in lungs and GBM
more common in 3rd and 6th decades of life, males slightly more affected than females
present with RPGN type I and hemoptysis/dyspnea
pulmonary hemorrhage more common in smokers and males
treat with plasma exchange, cyclophosphamide, prednisone
ANCA-Associated Vasculitis (e.g. Granulomatosis with Polyangiitis and Microscopic
Polyangiitis [formerly Wegeners Granulomatosis])
PR3-ANCA (c-ANCA) most commonly associated with the clinical picture of granulomatosis
with polyangiitis (previously called Wegener's granulomatosis)
MPO-ANCA (p-ANCA) most commonly associated with the clinical picture of microscopic
polyangiitis
renal involvement very common
focal segmental necrotizing RPGN with no immune staining
may be indolent or fulminant in progression
vasculitis and granulomas rarely seen on renal biopsy
treating typically involves cyclophosphamide and prednisone
Cryoglobulinemia
cryoglobulins: monoclonal IgM and polyclonal IgG
presents as purpura, fever, Raynauds phenomenon, and arthralgias
at least 50% of patients have hepatitis C
renal disease seen in 40% of patients (isolated proteinuria/hematuria progressing to nephritic
syndrome)
most patients have decreased serum complement (C4 initially)
treat hepatitis C, plasmapheresis
overall prognosis: 75% renal recovery
Shunt Nephritis
immune-complex mediated nephritis associated with chronically infected ventriculoatrial
shunts inserted for treatment of hydrocephalus
presents as acute nephritic syndrome with decreased serum complement
nephrotic range proteinuria in 25% of patients

EULAR Recommendations for the Management

of Systemic Lupus Erythematosus (SLE)
Ann Rheum Dis 2008;67:195-205
Lupus Nephritis Recommendations
Monitoring: Renal biopsy, urine sediment analysis,
proteinuria, and kidney function may have
independent predictive ability for clinical outcome in
therapy of lupus nephritis but need to be interpreted
in conjunction. Changes in immunological tests
(anti-dsDNA, serum C3) have only limited ability to
predict the response to treatment and may be used
only as supplemental information.
Treatment: In patients with proliferative lupus
nephritis, glucocorticoids in combination with
immunosuppressive agents are effective against
progression to end-stage renal disease. Longterm efficacy has been demonstrated only for
cyclophosphamide-based regimens, which are
also associated with considerable adverse effects.
In short- and medium-term trials, mycophenolate
mofetil has demonstrated at least similar efficacy
compared with pulse cyclophosphamide and a
more favorable toxicity profile: failure to respond by
6 mo should evoke discussions for intensification
of therapy. Flares following remission are not
uncommon and require diligent follow-up.
End-Stage Renal Disease: Dialysis and
transplantation in SLE have long-term patient
and graft-survival rates comparable with those
observed in non-diabetic non-SLE patients, with
transplantation being the method of choice.