A 67 years old Chinese male, complained headache accompanied by hearing loss, with obvious hearing loss in the right ear and obvious headache in the right temporal, and was admitted to the local hospital 7 years ago. Craniocerebral magnetic resonance (MRI)indicated thickening of the right dura which could be intensified. The patient was diagnosed as inflammatory pseudotumor and given treatment. Six years ago, the patient was treated with traditional Chinese medicine (the specific ingredient is not known), and the patient's hearing recovered slightly, and the headache symptoms were still worse. Three years ago, the patient had an epileptic seizure, and the enhanced computed tomography (CT) and MRI showed obvious uneven thickening and strengthening of the dura mater, and the right side was obvious. The patient is thought to have hypertrophic pachymeningitis and treated with glucocorticoids. Two years ago, the patient was admitted to a superior hospital and was finally diagnosed with hypertrophic pachymeningitis and treated with glucocorticoids and immunosuppressants. The patient was treated with levetiracetam for epilepsy control. Glucocorticoids and immunosuppressants were discontinued as prescribed by the doctor, and occasionally seizures occurred.
The patient was admitted to our hospital due to "fatigue with poor appetite for 20 days" this time. The patient showed fatigue, poor appetite, nausea, dizziness, and discomfort without obvious inducement 20 days ago, accompanied by lower limb pain after activity. He had no chest tightness, no suffocation, no palpitation, no discomfort in the centerless anterior area, no back pain, no facial edema and lower limb edema, and no significant decrease in urine volume, and he was admitted to our department for further examination and treatment. Since the onset of the disease, the patient has clear mind, general spirit, poor diet, poor sleep, basically normal urine and feces, weight loss. He has a history of stomach ulcers for more than 30 years. He suffered from coronary heart disease for 4 years and underwent coronary stent implantation due to acute myocardial infarction 4 years ago. He found his blood pressure elevated 20 days ago. He had no history of diabetes. He denied the history of infectious diseases such as hepatitis and tuberculosis. He had no history of trauma, blood transfusions, drug, or food allergies. He had no familial genetic disease and history of infectious diseases.
Physical examination at admission revealed that he had a temperature of 36.3°C, heart rate of 92 bpm, respiratory rate of 18 breaths per minute, blood pressure of 178/106 mmHg and oxygen saturation of 98% in room air. His physical examination showed no abnormality in cardiopulmonary abdominal examination and no edema in lower limbs.
Laboratory data revealed urine analysis: occult blood: 1+; urinary red blood cell: 24.70/ul, urine protein negative; Urinary renal tubule function display: urine α1 microglobulin:39.20mg/L; Blood routine: white blood cell count (WBC) :12.74×10^9/L; neutrophil cell count (NEU):9.38×10^9/L; red blood cell count (RBC):2.85×10^12/L; hemoglobin (HB) :80.00g/L; C-reactive protein (CRP):113.21mg/L; Blood biochemistry: total protein (TP) 59.6g/L; albumin (ALB) :21.6 g/L; urea nitrogen (BUN):17.80 mmol/L; Creatinine (Cr):365umol/L; kalium (K):3.39 mmol/L; sodium (Na):129 mmol/L; chlorine (Cl): 91mmol/L; calcium (Ca):1.92 mmol/L; Anti-neutrophil cytoplasmic antibody (ANCA): myeloperoxidase (MPO) 5.6 U/ml. Two days later, we reviewed the relevant blood test indicators, the results showed, ALB: 24.3g/L; BUN: 28.2mmol/L; Cr: 524µmol/L; Na: 130mmol/L; Cl: 93mmol/L; Ca: 1.98mmol/L; WBC:31.42×10^9/L; NEU:28.14×10^9 /L; RBC:2.78×10^12 /L; HB:79.00g /L; ESR:124. 00 mm/h; CRP:103.59mg/L; complement 3(C3):0.82g/L; rheumatism complete set was negative. MPO 7.4 U/ml; immunoglobulin G (IgG)4: 4.9g/L; Abdominal ultrasound: no obvious abnormality was found in the liver, bile, pancreas and spleen, no obvious abnormality was found in the portal vein system and hepatic vein system. Parotid ultrasonography: no obvious abnormality was found in bilateral parotid glands. Submandibular gland ultrasound: no obvious abnormality was found in bilateral submandibular glands, and no obvious abnormality was found in bilateral neck. Lower limb vascular ultrasound showed: arteriosclerosis and plaque formation in both lower limbs, no obvious abnormality in deep veins of both lower limbs, and no obvious abnormality in superficial veins of both lower limbs. Echocardiography showed the abnormal segmental motion of the left ventricular wall and abnormal left ventricular filling. CT showed double emphysema, double pulmonary fiber foci, thickening of the stomach wall, slight inflammatory changes around both kidneys. CT showed no evidence of retroperitoneal fibrosis. Urological ultrasonography showed the normal volume of both kidneys, slightly stronger echo of the parenchyma of both kidneys, poor perfusion of color doppler imaging of blood flow of both kidneys, abnormal spectrum. Brain MRI revealed thickening of the dura mater (Fig. 1). The patient initially diagnosed acute renal failure. Combined with positive IgG4 and ANCA, we considered acute renal failure according two aspects: ANCA-associated vasculitis renal damage, and IgG4-related diseases. To confirm the pathological type, the patient underwent an ultrasound-guided renal biopsy. Pathological results indicated that the glomerulus presented diffuse ischemic changes, accompanied by diffuse renal tubulointerstitial damage, and a fibrinoid necrotic structure suggested that small vasculitis could not be excluded. The specific description is as follows: Immunofluorescence examination: one glomerular was detected, but no obvious fluorescence distribution was observed. IgA-, IgG-, C3-, F-, IgM-, C1q-, Kappa-, Lambda-. HE and special staining showed a total of 11 glomeruli,2 of which were completely fibrotic, the capillary walls of the glomerulus were significantly shrunk, the segments were slightly thickened, the mesangial region was slightly proliferated, mesangial cells were 3–5/mesangial regions, mesangial matrix was slightly enlarged, endothelial cells were swollen, and one glomerulus was found to have focal segmentalized sclerosis with balloon adhesion. Masson staining showed no significant myoglobin deposition. About 60% of renal tubules were slightly atrophic, a few tubules were dilated and granulocyte degeneration was observed, a small amount of protein tubules were observed, and about 30% of renal interstitial fibrosis was observed, accompanied by more lymphocytes, plasma cells and scattered granulocyte infiltration. Immunohistochemistry: IgG4 were scattered (+), Congo red stain was negative (-) (Fig. 2). The fibrinoid necrosis of arteriole with inflammation was found in the interstitium (Fig. 3). A final diagnosis of acute renal failure, ANCA-associated microvasculitis was made. Subsequently, we treated the patient with methylprednisolone and mycophenolate, and the patient underwent regular outpatient review, with blood creatinine maintained at about 140umol/l.