​Physical examination found that the kidney was "bad", it was because of this rheumatism

*It is only for medical professionals to read for reference.
It seems that the annual physical examination is correct! Elderly men who found hydronephrosis due to physical examination, is it just a kidney problem? I'm afraid it's not that simple.
After a series of examinations, it was discovered that it was a "pot" of rheumatism.
The patient was introduced to the case, male, 72 years old
.
He was admitted to the hospital mainly because of "physical examination found hydronephrosis in April" .

The patient's physical examination before April revealed hydronephrosis, no hematuria, no frequent urination, no urgency, no nausea and vomiting, no joint pain, no rash, and no Raynaud's phenomenon
.

I was hospitalized at the Department of Urology 1 month ago.
The enhanced CT examination of the abdomen showed that there were multiple soft tissue density around the abdominal aorta and iliac arteries on both sides, the lower end of the left ureter was involved, the left hydronephrosis, renal parenchyma atrophy, small right renal cyst, retroperitoneum With multiple small lymph nodes, a double J tube was placed in the left ureter
.

Now for further diagnosis and treatment, go to the rheumatology and immunology department
.

Physical examination: the patient is clear and normal, the heart and lung auscultation is normal, the abdomen is soft, no tenderness, no muscle tension and rebound pain, no mass in the abdomen, no palpable liver and spleen under the ribs, no percussion pain of the liver and kidney, moving Sexual dullness is negative
.

Laboratory and imaging tests: no abnormalities in antinuclear antibody spectrum, complement, tumor markers and stool routine; urine routine: occult blood++++, red blood cells 361/μl, white blood cells 21.
6/μl; blood routine: red blood cells 3.
7x1012/ L, hemoglobin 115g/L; erythrocyte sedimentation rate 25mm/h; C-reactive protein 24mg/L; IgG4 4.
136g/L, IL-6 8.
7pg/ml; biochemical tips: total protein 61.
9g/L, white blood cell ratio 1.
4, glomerulus Filtration rate 57ml/min, creatinine 110.
6μmol/L; Thyroglobulin <0.
1ng/ml, Thyroglobulin antibody 157.
2IU/ml, Methperoxidase antibody 11.
1IU/ml
.

24-hour urine protein 0.
55g/24h
.

Seeing this, maybe still confused, but the careful friend found an abnormal IgG4 result, could it be.
.
.
Then, the PET-CT examination revealed that the hilum and mediastinum were frequently enlarged lymph nodes, accompanied by sugar Metabolism is increased, considering the possibility of inflammation, sarcoidosis is not excluded; soft tissue density around the abdominal aorta and iliac arteries on both sides, accompanied by increased glucose metabolism, combined with clinical hints that may be retroperitoneal fibrosis; elderly brain changes; chronic branch-like changes; double Lung scattered inflammation sequelae; aortic and coronary artery wall calcification foci; prostate calcification foci; vertebral body degenerative changes
.

Diagnosis: retroperitoneal fibrosis treatment: prednisone acetate 30mg qd po, ​​hydroxychloroquine sulfate 0.
2g qd po combined with cyclophosphamide 0.
8g q4w iv treatment
.

One month later, the patient's renal function, erythrocyte sedimentation rate and C-reactive protein levels returned to normal
.

What is retroperitoneal fibrosis? Retroperitoneal fibrosis (RPF) is rare.
Due to its non-specific manifestations and the early clinical symptoms are hidden, most patients start with obstructive symptoms caused by compression of the intra-abdominal cavity organs.
Clinically, due to lack of understanding of it, Easily ignored by doctors
.

The most susceptible to compression of the disease is the ureter, leading to urinary tract obstruction and hydronephrosis, and even renal failure
.

It seems that this patient "perfectly" fits the characteristics of the disease~What are the clinical characteristics of RPF? RPF is divided into idiopathic (iRPF) and secondary (sRPF)
.

iRPF is a rare connective tissue disease characterized by chronic non-specific inflammation and fibrosis in retroperitoneal tissues.
It is surrounded by an abnormally proliferating fibrous inflammatory mass surrounding the abdominal aorta and its adjacent structures, such as ureters, Inferior vena cava
.

iRPF accounts for about 70% of RPF and is a rare disease with an incidence of about 1/500000~1/200000
.

The prevalence of iRPF is between 40 and 60 years old, but children and adolescents also have the disease
.

The ratio of male to female is about 2~3:1
.

There are clear reasons for the occurrence of sRPF, such as tumors, drugs, trauma, inflammation, radioactive materials, endometriosis, etc.
, accounting for about 30% of RPF
.

RPF usually appears as an isolated disease, but with the deeper understanding of IgG4-related diseases (IgG4-RD), it is found that some iRPFs are closely related to IgG4-RD
.

IgG4-RD is a type of immune-mediated fibro-inflammatory disease that can invade multiple organs throughout the body.
The clinical manifestations are highly heterogeneous, including partial autoimmune pancreatitis, Mikulicz's disease, Riedel's thyroiditis, interstitial nephritis, RPF and so on
.

IgG4-RPF accounts for about 30%~60% of iRPF patients
.

What are the clinical manifestations of RPF? The early symptoms of RPF are insidious and non-specific
.

In the advanced stage, the clinical manifestations are often symptoms of compression or involvement of adjacent organs, blood vessels, and nerves, so it is easy to cause clinical misdiagnosis
.

Mainly include the following manifestations: (1) Pain: mostly dull pain, continuous, not related to body position, mostly located in the abdomen or lumbosacral region, can radiate to the lower abdomen or groin, and some may have dull chest pain
.

(2) systemic symptoms: fever, fatigue, weight loss, nausea, vomiting, high blood pressure and so on
.

(3) urinary symptoms: hematuria, back pain, urinary urgency, renal failure
.

(4) lower extremity edema (5) Other rare manifestations: abdominal pain, intestinal obstruction, change in bowel habits, jaundice, aortic obstruction, portal hypertension, scrotal swelling
.

How is RPF diagnosed? Because RPF has hidden symptoms and lack of specificity, and is a progressive disease, it is necessary to conduct comprehensive evaluation in combination with laboratory, imaging and pathological indicators to confirm the diagnosis and start treatment as soon as possible
.

Laboratory abnormal indicators include: increased erythrocyte sedimentation rate, increased C-reactive protein, anemia, increased gamma globulin, and renal insufficiency
.

Elevated serum IgG4 level (>135mg/dl) is one of the basis for the diagnosis of IgG4-RD, but it can be seen in only 51% of patients
.

Imaging examinations include: ultrasonography can find hydronephroureter, and sometimes hypoechoic masses surrounding the abdominal aorta, inferior vena cava, and ureter
.

IVP is typically characterized by smoothly thinning of the bilateral ureters at the obstructive plane (mostly at the level of the 4th to 5th lumbar vertebrae), the middle ureter is deviated to the inside, and the renal pelvis and ureter above the obstruction site are dilated
.

CT shows a soft tissue mass around the aorta.
The enhancement of the mass is related to the different stages of the disease.
The enhancement is significant in the early stage, but not obvious in the late stage.
Sometimes the lesion wraps the ureter and surrounding blood vessels, resulting in hydronephrosis
.

If the renal function is significantly reduced, retrograde ureteropyelography can be performed
.

MRI can show that the lesion has a low signal on T1WI, and on T2WI, there are different signal intensities due to different courses of disease, with high signal in the early stage and low signal in the late stage
.

PET/CT tracer FDG can be used to diagnose RPF due to its ability to accumulate in inflammatory cells such as neutrophils, lymphocytes, and macrophages other than tumor cells.
It is helpful for secondary RPF caused by malignant tumors.
Yu found the primary tumor of the malignant tumor
.

Therefore, PET/CT has obvious advantages in the diagnosis and differential diagnosis of RPF, and can be applied to patients with obvious renal insufficiency
.

The diagnosis of RPF requires pathological examination through mass biopsy, which can be obtained by percutaneous puncture, or during open or laparoscopic exploration or ureterolysis
.

The comprehensive diagnostic criteria of IgG4-RPF, in addition to clinical findings of local masses and elevated serum IgG4 levels (>135mg/dl), histopathology showed lymphocytes and plasma cells infiltrating inflammation, and the count of IgG4+ plasma cells in the tissue>10/ High power field and IgG4+ plasma cell/IgG+ plasma cell ratio>40%
.

How is RPF treated? Treatment is divided into two aspects: surgery and drugs
.

For patients who have developed severe obstructive nephropathy and cause renal failure, firstly, conservative treatments such as percutaneous nephrostomy or placement of ureteral stents are needed to urgently relieve the obstruction
.

Once iRPF is diagnosed, glucocorticoids are usually the first choice, which can be combined with immunosuppressive therapy such as cyclophosphamide, azathioprine, cyclosporine, and mycophenolate mofetil
.

There are also reports of the use of biological agents rituximab, infliximab and tocilizumab to treat refractory iRPF
.

References: [1] Wu Ruiyi, Wang Guomin.
Research progress in diagnosis and treatment of retroperitoneal fibrosis (RPF)[J].
Fudan Journal (Medical Edition), 2020,47(01):47-52.