http://informahealthcare.com/mor ISSN 1439-7595 (print), 1439-7609 (online) Mod Rheumatol, 2015; 25(1): 138–142 © 2014 Japan College of Rheumatology DOI 10.3109/14397595.2013.874748

Case Report

Tocilizumab improves systemic rheumatoid vasculitis with necrotizing crescentic glomerulonephritis

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Takashi Iijima1, Tatsuya Suwabe1, Keiichi Sumida1, Noriko Hayami1, Rikako Hiramatsu1, Eiko Hasegawa1, Masayuki Yamanouchi1, Junichi Hoshino1, Naoki Sawa1, Kenmei Takaichi3, Kenichi Oohashi2, Takeshi Fujii2, and Yoshifumi Ubara3 1Nephrology Center, Toranomon Hospital Kajigaya, 1-3-1 Kajigaya, Takatsu, 213-8587, Kawasaki, Kanagawa, Japan 2Department of Pathology, Toranomon Hospital Kajigaya, Kanagawa, Japan, and 3Okinaka Memorial Institute for Medical Research, Toranomon Hospital Kajigaya, Kanagawa, Japan

Abstract

Keywords

We report a Japanese woman with systemic rheumatoid vasculitis (SRV) complicated by necrotizing crescentic glomerulonephritis (NCGN). Rheumatoid arthritis first occurred at the age of 19 years, followed by interstitial pneumonia, hepatitis, rheumatoid nodules, mononeuritis multiplex, and hypocomplementemia in chronological order. At the age of 51 years, rapidly progressive renal failure occurred with nephrotic proteinuria, and NCGN with subepithelial deposits was revealed by renal biopsy. Severe destructive changes of multiple joints and scleritis were detected, but anti-neutrophil cytoplasmic antibody was negative on enzyme-linked immunosorbent assays and indirect immunofluorescence. SRV was diagnosed due to involvement of multiple extra-articular organs. An anti-interleukin (IL)-6 receptor antibody (tocilizumab) was started at dosage of 280 mg (8 mg/kg) monthly. After 18 months, her serum creatinine decreased from 1.7 to 1.3 mg/dL, and urinary protein excretion declined from 5.2 to 1.2 g daily. Tocilizumab may be a therapeutic option for SRV associated with NCGN.

Systemic rheumatoid vasculitis, Necrotizing crescentic glomerulonephritis, Humanized anti-interleukin-6 receptor antibody, Tocilizumab

Introduction Rheumatoid arthritis (RA) is a systemic inflammatory disorder that primarily affects the synovial joints, but can be complicated by extra-articular manifestations. Such manifestations are more common in males than in females, and are considered to be a consequence of longstanding active disease that can lead to increased mortality [1, 2]. Systemic rheumatoid vasculitis (SRV) is the term for RA associated with vasculitis of extra-articular organs such as the skin, eyes, and lungs [1, 2]. Genta et al. performed a literature review (using the PubMed and MEDLINE databases) and concluded that SRV has the following clinical manifestations: skin lesions (ischemic focal digital lesions, petechiae, purpura, and ulcers), peripheral nerve involvement (mononeuritis multiplex and sensory peripheral neuropathy), ocular lesions (peripheral ulcerative keratitis and scleritis), and pulmonary involvement (diffuse alveolar hemorrhage, diffuse interstitial pulmonary fibrosis, and pleuritis). However, there has been little mention of glomerular involvement by vasculitis, except in Harper’s report [3]. We encountered a Japanese woman with onset of RA at the age of 19 years, who had thereafter suffered from interstitial pneumonia, hepatitis, rheumatoid nodules, mononeuritis multiplex, and hypocomplementemia in chronological order, with the occurrence of necrotizing crescentic glomerulonephritis (NCGN) at the age of 49 years. SRV was diagnosed, but anti-neutrophil cytoplasmic antibody (ANCA) was negative. Treatment with Correspondence to: Y. Ubara, Nephrology Center, Toranomon Hospital Kajigaya, 1-3-1 Kajigaya, Takatsu, Kawasaki, Kanagawa 213-8587, Japan. Tel: +81-44-8775111; Fax: +81-44-8775333. E-mail: ubara@toranomon. gr.jp

History Received 10 October 2012 Accepted 11 February 2013 Published online 15 March 2013

anti-interleukin-6 receptor antibody (tocilizumab) was effective in this patient with renal involvement and may be a new option for management of SRV. Case presentation A 51-year-old woman with RA was admitted to our hospital with nephrotic-level proteinuria and rapidly progressive renal failure. RA had been diagnosed due to arthralgia of multiple bilateral joints such as those of the fingers and toes at the age of 19 years. She was treated with nonsteroidal anti-inflammatory drugs (NSAIDs) only. Interstitial pneumonia occurred at the age of 27 years, followed by autoimmune hepatitis at the age of 30 years. Then, oral prednisolone was started at dosage of 50 mg daily, which was tapered to 10 mg. Rheumatoid nodules developed on her left elbow and wrist at the age of 40 years. Hypocomplementemia and right median nerve paralysis (consistent with mononeuritis multiplex) were noted at the age of 48 years. Methotrexate was started at 10 mg weekly. At the age of 49 years, plate fixation of the cervical spine was done for atlantoaxial subluxation. Proteinuria was detected, and C-reactive protein (CRP) was 3.8 mg/dL at that time, but her serum creatinine (Cre) level was 0.5 mg/dL. An angiotensin II receptor blocker (candesartan at 8 mg daily) was administered, and the dose of prednisolone was increased from 10 to 15 mg daily. Her CRP reduced to 0.0 mg/dL, but serum Cre level rose to 1.7 mg/dL, so she was admitted to our hospital. On admission, the patient was 147.0 cm tall and weighed 35.0 kg. Her blood pressure was 116/75 mmHg and temperature was 35.4 °C. Scleritis was obvious bilaterally. Her hands, feet, and ankles were deformed and swollen bilaterally, while the lower extremities were edematous.

Case report  139

DOI 10.3109/14397595.2013.874748

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Table 1. Laboratory data of the present case on admission to our hospital Urinalysis Protein (g/day) Sugar Erythrocytes (/HPF) Beta-2 microglobulin (μg/day) N-Acetyl-β-d-glucosaminidase    (NAG) (IU/day) Blood count White blood cells (/μL) Red blood cells (/μL) Hemoglobin (g/dL) Hematocrit (%) Platelets (/μL) Serum chemistry UN (mg/dL) Cr (mg/dL) UA (mg/dL) Na (mEq/L) K (mEq/L) Cl (mEq/L) TP (g/dL) Alb (g/dL) T-cho (mg/dL) Plasma glucose (mg/dL) HbA1c (%) MMP-3 (μg/L) Immunological findings CRP (mg/dL) IgG (mg/dL) IgA (mg/dL) IgM (mg/dL) CH50 (U/mL) C3 (mg/dL) C4 (mg/dL) Immune complex (μg/mL) ANA Anti-Ro/SS-A Ab (EU) Anti-dsDNA Ab (IU/mL) Anti-Sm Ab (U/mL) Anti-cardiolipin Ab (U/mL) RF (IU/mL) CCP (U/mL) MPO–ANCA (EU) (ELISA) p-ANCA (IIF) PR3-ANCA (EU) (ELISA) c-ANCA (IIF) Anti-GBM Ab (EU) Cryoglobulin

Values

Normal ranges

5.2 – 11–30 5,216 54

0.0 –

Tocilizumab improves systemic rheumatoid vasculitis with necrotizing crescentic glomerulonephritis.

We report a Japanese woman with systemic rheumatoid vasculitis (SRV) complicated by necrotizing crescentic glomerulonephritis (NCGN). Rheumatoid arthr...
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