PMID- 11063295
OWN - NLM
STAT- MEDLINE
DCOM- 20010215
LR  - 20191025
IS  - 0172-8172 (Print)
IS  - 0172-8172 (Linking)
VI  - 19
IP  - 6
DP  - 2000
TI  - An unusual case of systemic lupus erythematosus, lupus nephritis, and transient 
      monoclonal gammopathy.
PG  - 235-41
AB  - A 23-year-old female patient suffering from active systemic lupus erythematosus 
      (SLE) was treated with azathioprine (2 mg/kg per day) and prednisone. Lupus 
      nephritis class III with increasing proteinuria developed 28 months after disease 
      onset. Treatment was switched to monthly pulse cyclophosphamide administered 
      intravenously for 6 months (total dose 6.3 g), followed by oral azathioprine and 
      low-dose prednisone to maintain partial remission. Eight months later, the 
      patient developed an acute exacerbation of SLE with fever, proteinuria of 9.1 
      g/day, pancytopenia, and cerebral involvement with cephalgias and a grand mal 
      seizure. She responded well to high-dose corticosteroids (500 mg prednisolone 
      pulses over 3 days, i.v.) and was azathioprine switched from to methotrexate 
      (12.5-15 mg per week). Under this treatment, lupus activity gradually decreased 
      and the patient felt well again. Five years after the initial diagnosis of SLE, a 
      rapidly increasing immunoglobulin G-kappa type (IgG-kappa) monoclonal gammopathy 
      developed, reaching a maximal serum paraprotein concentration of 73.5 g/l. Bone 
      marrow biopsy revealed 15% of moderately abnormal, highly differentiated plasma 
      cells arranged in small clusters and expressing IgG-kappa. No bony lesions were 
      detectable on skeletal radiographs. Pulses of dexamethasone (40 mg) were 
      administered and led to a transient decrease of paraproteinemia to a minimum of 
      31.9 g/l, followed by an increase to 62 g/l. At that point, high-dose 
      chemotherapy supported by autologous stem cell transplantation was considered. 
      Due to an intermittent pneumococcal septicemia, methotrexate was discontinued and 
      dexamethasone was replaced by 5-10 mg cloprednol. At this point, totally 
      unexpectedly, the paraprotein decreased spontaneously without any further 
      cytostatic treatment and was no longer detectable 1 year later. Concomitantly, 
      plasma cell counts in bone marrow biopsies fell to below 5%. As SLE remained 
      inactive, the patient became pregnant and gave birth to a healthy child. During 
      late pregnancy, SLE activity flared up with rising proteinuria and blood 
      pressure. Therefore, after delivery, cyclophosphamide (100 mg/day, orally) was 
      readministered for 4 months, resulting in an improvement of kidney function with 
      stable proteinuria of 1-2 g/l to date. Paraproteins are no longer detectable. In 
      conclusion, this case report documents the rare event of transient 
      paraproteinemia in a patient with SLE. A self-limiting regulatory defect in the 
      control of a terminally differentiated B-cell clone may be the origin of this 
      phenomenon.
FAU - Strobel, E S
AU  - Strobel ES
AD  - Department of Medicine, Klinikum der Albert-Ludwigs-Universitat Freiburg, 
      Germany.
FAU - Fritschka, E
AU  - Fritschka E
FAU - Schmitt-Graff, A
AU  - Schmitt-Graff A
FAU - Peter, H H
AU  - Peter HH
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - Germany
TA  - Rheumatol Int
JT  - Rheumatology international
JID - 8206885
RN  - 0 (Antirheumatic Agents)
RN  - 0 (Immunosuppressive Agents)
RN  - 8N3DW7272P (Cyclophosphamide)
RN  - MRK240IY2L (Azathioprine)
SB  - IM
MH  - Adult
MH  - Antirheumatic Agents/therapeutic use
MH  - Azathioprine/therapeutic use
MH  - Cyclophosphamide/therapeutic use
MH  - Female
MH  - Humans
MH  - Immunosuppressive Agents/therapeutic use
MH  - Lupus Erythematosus, Systemic/*complications/drug therapy/physiopathology
MH  - Lupus Nephritis/drug therapy/*etiology/physiopathology
MH  - Paraproteinemias/*etiology/physiopathology
MH  - Pregnancy
EDAT- 2000/11/04 11:00
MHDA- 2001/03/03 10:01
CRDT- 2000/11/04 11:00
PHST- 2000/11/04 11:00 [pubmed]
PHST- 2001/03/03 10:01 [medline]
PHST- 2000/11/04 11:00 [entrez]
AID - 10.1007/pl00006855 [doi]
PST - ppublish
SO  - Rheumatol Int. 2000;19(6):235-41. doi: 10.1007/pl00006855.