Medicine
®
Clinical Case Report
OPEN
Concurrent Kimura disease and lupus nephritis A case report Haitao Wang, MDa, Fang Fang, MDb, Ying Sun, MDa, Songlan Wang, MDa, Yonghui Mao, MDa,
∗
Abstract Background: Kimura disease is a rare chronic inflammatory disorder with peripheral eosinophilia and elevated serum IgE and is
also frequently complicated by nephropathy. Methods: We report a rare case of Kimura disease concomitant with lupus nephritis in a 72-year old male patient with recurrent unexplained lymphadenopathy, renal lesions, and immunologic abnormalities. Results: The patient was successfully managed with gamma immunoglobulin, intravenous pulse methylprednisolone therapy,
hydroxychloroquine, and prednisone. Conclusion: This is the first report of a case of Kimura disease concomitant with lupus nephritis and highlights the importance of
considering lupus nephritis as a possible concurrent disease in patients with Kimura disease that have immunologic abnormalities. Abbreviation: SLE = systemic lupus erythematosus. Keywords: case report, Kimura disease, lupus nephritis
and fever for 5 days. The patient underwent left submandibular lymphadenectomy in 1965 because of enlarged lymph nodes after a mosquito bite. His eosinophil count was 4.7 109/L (35%). He further received right submandibular lymphadenectomy in 1968 and supraclavicular lymphadenectomy in 1969 because of recurrent generalized lymph node enlargement. Pathological examination revealed eosinophilic granuloma. The patient received no treatment except Chinese herbal remedies, but his symptoms were gradually lessened. His eosinophil count fluctuated between 0.53 and 1.38 109/L (6.9%–17.9%). Routine urinalysis in March 2011 showed 20 to 30 deformed red blood cells per high power field and no proteinuria, and laboratory studies revealed a titer of 1:1280 for ANA and low C3 (29 mg/dL). The patient was otherwise asymptomatic and received no treatment. Two years later, proteinuria worsened with a 24-hour urinary protein of 1.7 g, and the patient was diagnosed with nephrotic syndrome and admitted. Physical examination revealed a blood pressure of 160/80 mm Hg. He had generalized lymph node enlargement. His eosinophil count was 1.13 109/L and serum IgE was markedly elevated (149,000 IU/mL). The 24-hour urinary protein was 2.42 g and the patient was positive for ANA (1:320), anti-RNP antibody, and antiribosomal P antibody, but negative for antidouble stranded (ds) DNA antibody and anti-Sm antibody. He had a low C3 (48 x0200A;mg/dL) and C4 (5 mg/dL). Serological study revealed HBsAg ( ), HBeAg ( ), HBsAg, and HBcAg(+) and HBV DNA was undetectable (1:1280 1:160 ( ) (+) (+) ( )
1:320 ( ) ( ) (+) (+) ( )
1:80 ( ) ( ) ( ) ( ) ( )
NT NT NT
29 5 NT
49 4 NT
53 5 NT
44 11 (+)
54 13 NT
63 18 NT
( ( ( ( ( (
) ) ) ) ) )
79–152 16–38 Negative
ANA = antinuclear antibody, Ig = immunoglobulin, NT = not tested, SLE = systemic lupus erythematosus, WBC = white blood cell.
3
Wang et al. Medicine (2016) 95:41
Medicine
Table 2 Changes over time in lymphocyte subsets. Lymphocyte populations Total T lymphocytes, % Helper/inducer T lymphocytes (CD4+), % Cytotoxic/suppressive T lymphocytes (CD8+), % NK cells, % B lymphocytes, % CD4+/CD8+ SLE score SLE activity SLEDAI
Normal references
10/2014
01/2015
09/2015
58.6–83.1 27.1–49.8 19.4–41.1 6.9–37.9 0–20 0.7–2
60.64 19.41 43.23 15.67 20.37 0.45 19 Severe activity 28
68.73 22.17 47.61 15.31 23.35 0.74
®
Clinical Case Report
OPEN
Concurrent Kimura disease and lupus nephritis A case report Haitao Wang, MDa, Fang Fang, MDb, Ying Sun, MDa, Songlan Wang, MDa, Yonghui Mao, MDa,
∗
Abstract Background: Kimura disease is a rare chronic inflammatory disorder with peripheral eosinophilia and elevated serum IgE and is
also frequently complicated by nephropathy. Methods: We report a rare case of Kimura disease concomitant with lupus nephritis in a 72-year old male patient with recurrent unexplained lymphadenopathy, renal lesions, and immunologic abnormalities. Results: The patient was successfully managed with gamma immunoglobulin, intravenous pulse methylprednisolone therapy,
hydroxychloroquine, and prednisone. Conclusion: This is the first report of a case of Kimura disease concomitant with lupus nephritis and highlights the importance of
considering lupus nephritis as a possible concurrent disease in patients with Kimura disease that have immunologic abnormalities. Abbreviation: SLE = systemic lupus erythematosus. Keywords: case report, Kimura disease, lupus nephritis
and fever for 5 days. The patient underwent left submandibular lymphadenectomy in 1965 because of enlarged lymph nodes after a mosquito bite. His eosinophil count was 4.7 109/L (35%). He further received right submandibular lymphadenectomy in 1968 and supraclavicular lymphadenectomy in 1969 because of recurrent generalized lymph node enlargement. Pathological examination revealed eosinophilic granuloma. The patient received no treatment except Chinese herbal remedies, but his symptoms were gradually lessened. His eosinophil count fluctuated between 0.53 and 1.38 109/L (6.9%–17.9%). Routine urinalysis in March 2011 showed 20 to 30 deformed red blood cells per high power field and no proteinuria, and laboratory studies revealed a titer of 1:1280 for ANA and low C3 (29 mg/dL). The patient was otherwise asymptomatic and received no treatment. Two years later, proteinuria worsened with a 24-hour urinary protein of 1.7 g, and the patient was diagnosed with nephrotic syndrome and admitted. Physical examination revealed a blood pressure of 160/80 mm Hg. He had generalized lymph node enlargement. His eosinophil count was 1.13 109/L and serum IgE was markedly elevated (149,000 IU/mL). The 24-hour urinary protein was 2.42 g and the patient was positive for ANA (1:320), anti-RNP antibody, and antiribosomal P antibody, but negative for antidouble stranded (ds) DNA antibody and anti-Sm antibody. He had a low C3 (48 x0200A;mg/dL) and C4 (5 mg/dL). Serological study revealed HBsAg ( ), HBeAg ( ), HBsAg, and HBcAg(+) and HBV DNA was undetectable (1:1280 1:160 ( ) (+) (+) ( )
1:320 ( ) ( ) (+) (+) ( )
1:80 ( ) ( ) ( ) ( ) ( )
NT NT NT
29 5 NT
49 4 NT
53 5 NT
44 11 (+)
54 13 NT
63 18 NT
( ( ( ( ( (
) ) ) ) ) )
79–152 16–38 Negative
ANA = antinuclear antibody, Ig = immunoglobulin, NT = not tested, SLE = systemic lupus erythematosus, WBC = white blood cell.
3
Wang et al. Medicine (2016) 95:41
Medicine
Table 2 Changes over time in lymphocyte subsets. Lymphocyte populations Total T lymphocytes, % Helper/inducer T lymphocytes (CD4+), % Cytotoxic/suppressive T lymphocytes (CD8+), % NK cells, % B lymphocytes, % CD4+/CD8+ SLE score SLE activity SLEDAI
Normal references
10/2014
01/2015
09/2015
58.6–83.1 27.1–49.8 19.4–41.1 6.9–37.9 0–20 0.7–2
60.64 19.41 43.23 15.67 20.37 0.45 19 Severe activity 28
68.73 22.17 47.61 15.31 23.35 0.74
