On August 19, 2013, the Department of Nephrology of Nanshan Hospital admitted a rare case of Kimura disease in the world. The case is reported as follows: a male, 23 years old, was admitted to the hospital with “a swelling on the right upper arm for nearly 3 years and swelling of both lower extremities for 11 days”. The patient was found to have a swelling on his right upper arm in October 2010, but it was not taken seriously and not treated; in June 2013, he felt that the swelling on his right upper arm was significantly enlarged and the local skin temperature was elevated, but it was still not treated; on August 8, 2013, he developed swelling of both lower extremities, abdominal distension, and urination, but it was not treated; on August 10, 2013, he had an ultrasound examination of his right upper arm at a hospital in Shenzhen: multiple lymph nodes were seen on the inner side of his right upper arm (considered inflammatory). On August 17, 2013, the patient felt that the swelling of the eyelids, face and both lower limbs had increased, accompanied by nausea and epigastric distension, without abdominal pain and diarrhea. The patient came to our hospital on August 17, 2013, and checked the blood routine: WBC 7.7×10^9/L, NEUT 23.6%, NE# 1.8×10^9/L, EO# 48.5%, EO# 3.71×10^9/L, HGB 169g/L, PLT 172×10^9/L; liver function: TP 50.3g/L, ALB 16g /L, GLB 34.3g/L, A/G 0.5, ALT 20U/L, AST 31U/L, ALP 98U/L, GGT 34U/L, TBIL 6.5umol/l, DBIL 1.6umol/l, IBIL 4.9umol/l; renal function: BUN 4.9mmol/l, UA 379μmol/l, Cr Urine routine: PRO 4+, BLD2+, URO+, BIL+; AFP 9.81ng/ml. ultrasound of liver, gallbladder, spleen and pancreas: edema and thickening of gallbladder wall. The patient was admitted to our department for “investigation of the cause of swelling: nephrotic syndrome”. The patient had a history of hepatitis B “small triple-positive” for many years. He denied any history of hypertension and diabetes, food and drug allergy, and smuggling. On admission: T 36.6℃ P 84 times/min R 20 times/min BP 117/81mmHg. clear consciousness, mild puffiness of eyelids and face. The pharynx was not congested and the tonsils were not enlarged. The heart, lungs and abdomen have no obvious positive signs. There was no percussion pain in both kidney areas. The scrotum was moderately edematous. There was obvious sunken edema in both lower extremities. Multiple enlarged lymph nodes were palpable in the slide of the right upper arm, with a maximum of about 30*20 mm. Auxiliary examination: WBC 7.7×10^9/L, NEUT 50%, EO# 28.4%, EO# 2.18×10^9/L, HGB 183g/L, PLT 198×10^9/L; urine routine: SG 1.042, PRO4+, BLD2+, BIL +The rest was normal; 24h urine total microprotein 9373mg/d. Stool routine was normal. Coagulation function: PT 14.5 S, PTA %83, APTT 46s, TT 24.4S, FIB 10.36 g/L, AT-IIIA 42%. Renal function: BUN 6.0 mmol/l, UA 348.6 μmol/l, Cr 66.5 μmol/L, cystatin C1.31 mg/L. Urinary NAG enzyme 77 U/L. Electrolytes: K 3.6 mmol/l, Na 143 mmol/l, Cl 107.3 mmol/l, Ca 1.82 mmol/L, P 0.99 mmol/l, CO2 27.2mmol/l, Glu 4.22mmol/l; liver function on August 20: TP 40.8g/L, ALB 13.1g/L, GLB 27.7g/L, A/G 0.5, ALT 17U/L, AST 46U/L, ALP 81U/L, GGT 29U/L, TBIL 7.6umol /l, DBIL 2.2umol/l, IBIL 5.4umol/l; liver function on August 29: TP 37.1g/L, ALB 11.2g/L, GLB 25.9g/L, A/G 0.4, ALT 23U/L, AST 35U/L, ALP 64U/L, GGT 33U/L, TBIL 2.2umol/l, Cardiac enzymes: CK 888U/L, CKMB 24U/L, CKMB/CK 0.03, LDH 217U/L, LDH1 44U/L; CRP <0.5mg/L; Lipids IV: CH 13.36mmol/L, TG 1.85mmol/L, HDL 2.32mmol/l, LDL 9.37mmol/l; amylase and lipase were normal; A-factor five: TOTT3 0.32nmol/l, TT4 68.44nmol/l, TSH 6.65mIU/L, FT3 2.69pmol/l, FT4 9.04pmol/l; immune six: C3 1.54g/L, C4 0.37g/L, IgG 5.82g/L, IgA 3.69g/L, IgM 1.44g/L, IgE 1510 IU/ml; Hepatitis B two-to-one quantification: HbsAg>299.91ng/ml (0-0.25), HbsAb 1mIU/ml (0-10), HbeAg 0.002 NCV/ml (0-0.03 HbeAb 29.91 NCV/ml (0-2), HbcAb 14.91 NCV/ml (0-0.6); HBV-DNA quantification: 1.84×10^3 IU/ml. negative for hepatitis A, C and E antibodies. negative for syphilis. Syphilis two negative. HIV negative. Rheumatology II (ASO, RF), Rheumatology VI (including anti-RNP, anti-Sm, anti-SS-A, anti-SS-B, anti-Scl-70, anti-JO-1), anti-nuclear antibody II (ANA, anti-ds-DNA) were negative. Vasculitis antibody profile (MPO, GBM, PR3), ACL, cANCA and pANCA were negative. Blood and urine immunofixation electrophoresis were negative. Chest X-ray: right middle lung fibrosis foci were possible; urological ultrasound showed: left kidney size about 111*53*51mm, right kidney size about 106*43*54mm, both kidney cortex thickness about 9-10m, parenchymal echogenicity, pelvic effusion, left kidney urine salt crystals, right kidney and prostate currently did not show obvious abnormal sonograms. Ultrasound of both renal arteries and abdominal aorta showed no significant abnormalities in both kidneys, bilateral adrenal area, both renal arteries and abdominal aorta. MRI of the right upper arm: multiple lymph nodes in the right upper arm were considered to be enlarged, and enhanced scanning was recommended. Lymph node biopsy of the right upper arm: consistent with eosinophilic proliferative lymphogranuloma. Bone marrow cytology: active proliferation of nucleated cells in the bone marrow, with 47% granulocytes, an increased eosinophil ratio (14% total, including 2.0% late juvenile granulocytes, 2% rod nuclei, and 10% lobulated nuclei), 12% juvenile erythrocytes, 36% lymphocytes, and 1.0% anisocytic lymphocytes. 28 megakaryocytes were seen in the whole film, and small scattered clusters of platelets were easily seen. Bone marrow biopsy: microscopically, the ratio of bone marrow hematopoietic tissue to adipose tissue was about 5:5, the ratio of granulocytes to red lineage was approximately normal, granulocytes were mainly eosinophils, all stages of red blood cells were seen, and the ratio was not abnormal; megakaryocytes were seen, and the morphology was not abnormal, reticulocyte staining was grade 1. Blood smear: eosinophils accounted for 6% (all were lobulated nuclei), heterogeneous lymphocytes accounted for 2%, mature red blood cells were not significantly abnormal, and platelets were easily seen scattered. Genetic testing: karyotype analysis: no abnormal chromosomal clones were seen, normal male karyotype. Bone marrow fusion gene FIP1L1-PDGFRα: negative. Bone marrow fusion gene ETV6-PDGFRβ: negative. Bone marrow FISH for 8p12/8p11.1-q11.1 locus: no abnormal signal was seen. Qualitative testing for JAK2 V617F mutation in peripheral blood: negative. Renal pathology: focal segmental glomerulosclerosis (cellular type). Diagnosis: 1, Kimura disease 2, secondary nephrotic syndrome (focal segmental glomerulosclerosis, cellular type) 3, hepatitis B virus carrier. Treatment: Methylprednisolone 80mg/d×3d was given, later changed to methylprednisolone tablets 36mg/d, while in lamivudine to inhibit hepatitis B virus replication; and symptomatic treatment such as stomach protection, calcium supplementation, kidney protection, and improvement of coagulation function was given. 6 days later the patient’s generalized swelling subsided, urine protein turned negative, blood albumin returned to normal, and the swelling on the right upper arm basically subsided. The patient has been followed up for 1.5 years without recurrence, and we will follow up this patient for a long time. (