A Case Report of Successful Long-term Relapse Control by Protein-A Immunoadsorption in an...

A Case Report of Successful Long-term Relapse Controlby Protein-A Immunoadsorption in an

Immunosuppressive-treated Patient With End-stage RenalDisease Due to Wegener’s Granulomatosis

Michael Koch, Matthias Kohnle, and Rudolf Trapp

Center of Nephrology, Mettmann, Germany

Abstract: A long-term female hemodialysis patient withend-stage renal disease due to Wegener’s granulomatosis(WG) experienced a severe relapse when immunosuppres-sive therapy was switched from prednisone and cyclophos-phamide to azathioprine maintenance therapy. Ten coursesof protein A immunoadsorption therapy and switchingimmunosuppressive therapy to mycophenolate mofetil

have proved to be very successful and free of side effects.The patient has fully recovered from all clinical WGsymptoms and is still in remission ten months after thetreatment. Key Words: End-stage renal disease, Immuno-suppressive therapy, Protein A immunoadsorption,Relapse, Wegener’s granulomatosis.

Wegener’s granulomatosis (WG) is a form ofsmall- and medium-sized vessel vasculitis ofunknown etiology that affects mainly the upperairways, lungs, kidneys, and other organs. Due to itsend-organ damage, it can be a serious disease with atypical relapsing–remitting course that requires long-term treatment with immunosuppressive therapy (1).It is named after Friedrich Wegener, who describedthe disease in 1936 (2). Organ inflammatory damageis mediated by cytoplasmic-staining antineutrophil-cytoplasmic-antibodies (cANCA), and their detec-tion is a component of the diagnosis, as well as clinicalsigns and symptoms, and histopathological biopsyabnormalities. We describe the case of a youngfemale patient with end-stage renal disease (ESRD)and WG undergoing long-term hemodialysis, whoseclinical and serological symptoms could be controlledusing long-term immunosuppressive therapy withprednisone and oral and intravenous pulse cyclo-phosphamide, but who developed serious, fulminant

symptoms after being switched to azathioprine,which, however, could be successfully treated byprotein A immunoadsorption (IA). The remarkableaspect of this case is that although under long-termhemodialytic and immunosuppressive therapy, herWG became active and fulminant after switching toazathioprine. However, protein A IA therapy, used inlieu of conventional plasma separation and known asa procedure being carried out without serious sideeffects in a significant number of treatments to date(3), proved very effective and free of side-effects inour case as well. The patient completely recoveredfrom all WG symptoms and is still in remission tenmonths after this treatment.

CASE REPORT

A 31-year-old female patient (height 173 cm,weight67 kg) presented in October 2001 with a long historyof the usual symptoms of WG (see Table 1). Ourpatient exhibited involvement of the lung andkidneys, and despite effective long-term immunosup-pression therapy (lymphocyte count < 1000/mm3)with prednisone and oral and intravenous pulse cyclo-phosphamide, she developed severe symptoms asso-ciated with frailness, weakness, and the worsening of

Received February 2008; revised June 2008.Address correspondence and reprint requests to Dr Michael

Koch, Gartenstraße 8, 40822 Mettmann, Germany. Tel: +49 2104979 960; Fax: +49 2104 979 9671; Email: [email protected]

Therapeutic Apheresis and Dialysis 13(2):150–156doi: 10.1111/j.1744-9987.2009.00670.x© 2009 The AuthorsJournal compilation © 2009 International Society for Apheresis

150

mailto:[email protected]



TAB

LE

1.M

edic

alhi

stor

yof

the

patie

nt(b

orn

1970

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nJu

ne19

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Wegener’s Granulomatosis Relapse Control 151

© 2009 The AuthorsJournal compilation © 2009 International Society for Apheresis Ther Apher Dial, Vol. 13, No. 2, 2009

TAB

LE

1.C

ontin

ued

Dat

eC

linic

alL

abor

ator

yD

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osti

csT

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py

Oct

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Seco

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lar

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ong-

term

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rine

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tmen

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r20

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int

pain

,myo

path

ycA

NC

A50

RU

/mL

N/A

Pre

dnis

one

incr

ease

dto

75m

g/da

y

M Koch et al.152

© 2009 The AuthorsJournal compilation © 2009 International Society for ApheresisTher Apher Dial, Vol. 13, No. 2, 2009

TAB

LE

1.C

ontin

ued

Dat

eC

linic

alL

abor

ator

yD

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osti

csT

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py

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ober

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r20

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curr

ent

join

tpa

inof

PIP

3-D

III

righ

t,bi

late

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join

tscA

NC

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RU

/mL

N/A

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eth

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nce

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nd50

mg/

day

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e11

Oct

2003

plus

sulf

amet

hoxa

zole

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met

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im;

pred

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ne50

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ise

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ctio

n;cy

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hosp

ham

ide

50m

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ery

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ginn

ing

15N

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03

Mar

ch–A

ugus

t20

04N

osy

mpt

oms

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CA

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U/m

LN

/AC

yclo

phos

pham

ide

50m

g/da

y,th

enev

ery

2da

ys50

mg

unti

lDec

2004

,th

en50

mg/

day,

begi

nnin

g18

Feb

2005

agai

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ery

seco

ndda

y50

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unti

l22

Apr

,the

n50

mg/

day;

pred

niso

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scon

tinu

edon

14M

ar20

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ast

dose

was

2.5

mg/

day

Mar

ch–A

ugus

t20

05N

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mpt

oms

cAN

CA

92R

U/m

LN

/AA

pr–A

ug20

05:5

0m

gcy

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ide/

day;

inA

ug20

05:

swit

chfr

omcy

clop

hosp

ham

ide

toaz

athi

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ne50

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day,

then

75m

g/da

y

Mar

ch–N

ovem

ber

2006

No

sym

ptom

sN

/AN

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mg

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hiop

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/day

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ary

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ptom

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573

RU

/mL

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of23

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2007

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mg/

day

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ith

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Jun

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est

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t;W

G,W

egen

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ulom

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is.



her overall health, without typical organ involvement(e.g. hemoptysis) after being switched to azathioprinemaintenance therapy. Concentrations of the mostcommon biomarker of WG (cANCA bound to theproteinase-3 [PR3] target) ranged between 0 and 92relative units (RU) per millimeter between October2001 and December 2006 (see Table 1), but signifi-cantly increased (without complement depletion)starting August 2005 under azathioprine therapy,reaching its peak in May 2007 (see Fig. 1) and includ-ing significant deterioration of her overall generalhealth.

Due to the severe worsening of the patient’s clini-cal status, our patient was subjected to ten sessions ofprotein A IA therapy—which, in contrast to conven-tional plasma exchange, selectively removes immu-noglobulins and immune complexes from plasma—between May and June 2007 using protein A columns(Immunosorba; Fresenius Medical Care, Bad Hom-burg, Germany), while immunosuppression therapywas switched to mycophenolate mofetil. We havepreviously described the procedure of protein A IA(4). In the present case, the patient received fourtreatment cycles per week, with 5–6 L of processedplasma during each cycle.

With increasing cANCA titers, our patient experi-enced the following symptoms and signs: increasingweakness, weight loss of 3 kg, intermittently elevatedtemperatures of up to 38°C (100.4°F), a mild, non-focal increase in C-reactive protein, no signs of leu-

kocytosis, no symptoms associated with the joints(such as pain, swelling, or redness), nasal speech, non-bacterial conjunctivitis, severe malaise, an increase inblood pressure, hoarse voice, and a productive coughwith bloody imbibition.

During the initial treatment the patient’s weaknesshad further progressed, and she reported thatbetween the first and second treatment cycles she hadbeen suffering from severe back pain despite takingparacetamol. As of the third treatment, the patientwas slowly feeling clinically better, although herhemoglobin (Hb) level had dropped to as low as7.8 g/dL. Due to persisting low Hb levels despiteadministration of high doses of erythropoietin, thepatient was subjected to a blood transfusion duringthe seventh protein A IA treatment, which causedthe Hb levels to increase to 10.0 g/dL. The leukocytecount remained between 5500 and 7800/mL duringthe entire course of treatment.

The final, tenth protein A IA treatment took placeon 12 June 2007 and the patient felt much improved;however, she did complain of some newly developedrespiratory-related symptoms in the left paraverte-bral region that finally disappeared, and prednisonedose could then be reduced to 10 mg per day. Tenmonths after treatment, the patient is currently incomplete disease remission without any clinicalsymptoms of WG.

Concentrations of cANCA decreased markedly(see Fig. 1) from the beginning of protein A IA treat-ment, and since the beginning of October 2007cANCA has either not been detectable or is presentin very low concentrations (20 RU/mL in April 2008).Also, since all other subclasses of immunoglobulin G(IgG) are also eliminated by the protein A column,the serum IgG titer reached a value as low as44 mg/dL at the conclusion of the ninth protein A IAtreatment (reference range 800–1800 mg/dL).

DISCUSSION

We have described the case of a young woman withESRD due to WG, who had been undergoing long-term hemodialytic and immunosuppressive treat-ment in order to successfully control her clinical andserological WG symptoms and signs. However, afterbeing switched to the standard maintenance therapyazathioprine, the patient developed a disease relapsewith slowly increasing anti-PR3/cANCA levels(Table 1 and Fig. 1), but initially without concomitantclinical symptoms. After reaching the peak cANCAlevel (573 RU/mL), her clinical symptoms becamefulminant and her overall health markedly deterio-rated. A single treatment of intravenous pulse cyclo-

02/0

703

/07

04/0

705

/07

06/0

707

/07

08/0

709

/07

10/0

7

0

100

200

300

400

500

600

Time (month/year)

protein A IA mycophenolate mofetilazathioprine

c-A

NC

A (

RU

/mL

)

FIG. 1. Blood serum concentrations (relative units, RU) ofthe antiproteinase-3 (PR3)/cytoplasmic-staining antineutrophil-cytoplasmic-antibody (cANCA) complex between February andOctober 2007, measured by ELISA immunoassay. All values arethe highest measured in a specific month. Starting August 2005, thepatient had been under azathioprine immunosuppression therapy75 mg/day. Starting in May 2007, the patient was switched toprotein A immunoadsorption therapy (IA) with additional immu-nosuppressive cyclophosphamide (700 mg) on 31 May 2007, whichwas replaced by mycophenolate mofetil (2 ¥ 500 mg/day) starting12 June 2007 (see Table 1).

M Koch et al.154


phosphamide and ten courses of protein A IAtherapy combined with mycophenolate mofetilmaintenance therapy, however, resulted in completeremission of WG. While still under hemodialysis andmycophenolate mofetil immunosuppressive therapy,the patient is currently completely rehabilitated andclinically and serologically symptom-free half a yearafter protein A IA.

Our WG patient on long-term hemodialysis hadbeen treated with standard therapy to successfullycontrol clinical and serological symptoms of thedisease. Standard management of WG can be dividedin two stages: induction or remission by cyclophos-phamide and prednisone, and maintenance therapyby, for instance, azathioprine to reduce toxicity due tocumulative cyclophosphamide (5,6). Several authorshave found that hemodialysis itself, either withoutor with minimal immunosuppressive therapy, mayprotect, although not always, against disease activity(7,8) or relapse (9), whereas others could not confirma different relapse rate between dialyzed and non-dialyzed WG patients (10). We assume that in ourpatient the relapse of WG, reflected by increases ofPR3/cANCA levels and concomitant fulminant clini-cal symptoms, may be due to azathioprine failure. Wetherefore switched azathioprine therapy to plasma-pheresis therapy in the form of protein A IA, whichhad previously proved successful in similar cases(11,12), in combination with mycophenolate mofetilas long-term maintenance therapy.

The rationale behind the therapy used was toreduce or eliminate cANCA titers and all other sub-classes of IgG by protein A IA (and consequently toimprove the described clinical symptoms), whileinhibiting new cANCA formation by adding myco-phenolate mofetil to the treatment regimen. Myco-phenolate mofetil has shown favorable results in thetreatment of WG (13). A high-maintenance therapywith cyclophosphamide was not considered becausethe patient had previously been treated with cyclo-phosphamide and had already received a high totalcumulative dose during the course of her illness.

Alternative treatments for WG have beendescribed in the literature, for example Iwatani et al.(14) have successfully used double filtration plasma-pheresis (DFPP) in combination with steroids andimmunosuppressants in a patient in whom WG wasassociated with severe pulmonary bleeding. Nonethe-less, DFPP has been mainly described in retrospec-tive case reports from Japanese authors, mainly inconjunction with Guillain–Barré syndrome (15).Other authors describe the use of intravenousimmunoglobulin (IVIg) in patients with cANCA-associated systemic vasculitis who suffered from per-

sistent disease or who were poor respondersto conventional therapy (16,17). Richter et al. (16),however, observed a benefit in only 40% of hispatients, whereas complete remission of diseaseactivity did not occur in any of them. Jayne et al. (17)described mild adverse effects in all of his 17 IVIg-treated patients.

CONCLUSION

We understand that WG patients may respond dif-ferently toward a certain therapy and that generaliza-tion about the most beneficial therapy in thesepatients may not be possible. However, in our specificcase of a WG patient with failed standard mainte-nance therapy, protein A IA therapy—which does nothave the risk of transmitting any microorganisms orviruses and during which a high volume of plasma canbe processed, removing up to 87% of the initial levelof IgG in one session without a clinically significantloss of fibrinogen—combined with the immunosup-pressant mycophenolate mofetil proved very success-ful clinically, meaning that ten months after protein AIA, and while still under hemodialysis and mycophe-nolate mofetil therapy, our patient is completely reha-bilitated. We consider that achieving long-termremission under the described therapy that does notinclude any long-term, high-dose cyclophosphamidetherapy is rather unique and has, to our knowledge,not yet been described in the literature. The clinicalimprovement was associated serologically with a sig-nificant decrease of cANCA and IgG levels, support-ing the notion that the cANCA titer level is a goodindicator of disease activity and correlates very wellwith disease severity, at least for the higher titer levelssuch as those observed in our patient.

Acknowledgments: We thank Silke Haidekker, PhD,ELS, for her excellent assistance in the preparation of themanuscript.

REFERENCES

1. Seo P, Stone JH. The antineutrophil cytoplasmic antibody-associated vasculitides. Am J Med 2004;117:39–50.

2. Wegener F. Über generalisierte, septische Gefässerkrankun-gen. Report of 3 cases. Verh Dtsch Ges Pathol 1936; 29:202.

3. Belak M, Borberg H, Jimenez C, Oette K. Technical and clini-cal experience with protein A immunoadsorption columns.Transfus Sci 1994;15:419–22.

4. Koch M, Trapp R. Ethyl mercury poisoning during a proteinA immunoadsorption treatment. Am J Kidney Dis 2006;47:e31–4.

5. Riccieri V, Valesini G. [Treatment of Wegener’s granulomato-sis]. Reumatismo 2004;56:69–76.

6. Haubitz M. ANCA-associated vasculitis: diagnosis, clinicalcharacteristics and treatment. Vasa 2007;36:81–9.



7. Pedersen RS, Aunsholt NA. Hemodialysis does not alwaysprotect against recurrence of Wegener’s granulomatosis.Scand J Urol Nephrol 1990;24:223–5.

8. Kuross S, Davin T, Kjellstrand CM. Wegener’s granulomatosiswith severe renal failure: clinical course and results of dialysisand transplantation. Clin Nephrol 1981;16:172–80.

9. Weidanz F, Day CJ, Hewins P, Savage CO, Harper L. Recur-rences and infections during continuous immunosuppressivetherapy after beginning dialysis in ANCA-associated vasculi-tis. Am J Kidney Dis 2007;50:36–46.

10. Haubitz M, Koch KM, Brunkhorst R. Survival and vasculitisactivity in patients with end-stage renal disease due toWegener’s granulomatosis. Nephrol Dial Transplant 1998;13:1713–8.

11. Matic G, Michelsen A, Hofmann D et al. Three cases ofC-ANCA-positive vasculitis treated with immunoadsorption:possible benefit in early treatment. Ther Apher 2001;5:68–72.

12. Schneidewind J, Gliesche T, Sehland D et al. [Protein A immu-noadsorption al a new apheresis procedure for elimination of

HLA antibodies]. Beitr Infusionsther Transfusionsmed 1994;32:360–5.

13. Antoniu SA. Treatment options for refractory Wegener’sgranulomatosis: a role for rituximab? Curr Opin InvestigDrugs 2007;8:927–32.

14. Iwatani H, Uzu T, Kakihara M et al. A case of Wegener’sgranulomatosis with pulmonary bleeding successfully treatedwith double filtration plasmapheresis (DFPP). Clin ExpNephrol 2004;8:369–74.

15. Lyu RK, Chen WH, Hsieh ST. Plasma exchange versus doublefiltration plasmapheresis in the treatment of Guillain-Barresyndrome. Ther Apher 2002;6:163–6.

16. Richter C, Schnabel A, Csernok E, De Groot K, Reinhold-Keller E, Gross WL. Treatment of anti-neutrophil cytoplasmicantibody (ANCA)-associated systemic vasculitis with high-dose intravenous immunoglobulin. Clin Exp Immunol 1995;101:2–7.

17. Jayne DR, Chapel H, Adu D et al. Intravenous immunoglobu-lin for ANCA-associated systemic vasculitis with persistentdisease activity. QJM 2000;93:433–9.

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