The latest research: The Chinese population of Behcet's disease is more likely to be of these 5 types!

*For medical professionals to read and refer to the clinical characteristics and phenotypic distribution of Chinese Behcet's disease patients, must-see ~ Behcet's disease can be found in all kinds of people in my country, from teenagers to the elderly, but young people between 16 and 40 years old More often, it can be affected by both men and women.

Previously, foreign researchers proposed that Behcet's disease can be divided into six phenotypes: skin and mucosal lesions, arthropathy, vascular lesions, ocular lesions, neuropathy and gastrointestinal lesions.

However, this type of classification may not be suitable for the Chinese patient population, and because there are few relevant studies on Behcet's disease patients in China, we still cannot fully understand its clinical phenotype.

On January 30, 2021, scholars from East China Hospital of Fudan University launched a cohort study based on Chinese Behcet's disease patients, aiming to investigate the clinical characteristics and phenotype distribution of Chinese Behcet's disease patients based on their clinical manifestations and organ involvement.
The research results were published in the journal Arthritis Research & Therapy.

What are the symptoms of 014 main manifestations of Behcet's disease? Behcet’s disease, also known as Behcet’s syndrome, or eye-mouth-genital syndrome, or Behcet’s syndrome, was first reported by Turkish dermatologist Behcet in 1937.
The global incidence is only 10.
3 per 100,000 people.

Interestingly, Behcet’s disease has obvious regional differences in prevalence.
It is more common in East Asia, the Middle East and the Mediterranean basin, such as Iran, Arabia, Turkey, etc.
, distributed along the ancient Silk Road, so it is also called "Silk Road Disease" .

The basic pathological feature of Behcet's disease is non-specific vasculitis.

Behcet's disease not only affects the eyes, mouth and genitals, but also affects multiple systems throughout the body.
In severe cases, it can cause complete blindness, brain atrophy or even death.

Four basic symptoms of Behcet's disease: 1.
Oral ulcers Recurrent small ulcers, only a few are deep ulcers. Ulcers can occur on the lips, tongue, cheeks, palate, and gums, and they can heal in about 10 days.

2.
Eye lesions can be manifested as conjunctivitis, keratitis, choroiditis and retinitis, and more serious are iridocyclitis and anterior chamber empyema.

Optic neuritis and optic nerve atrophy can cause vision loss and even blindness.

3.
Genital lesions are more common in the scrotum and penis in men, and can also cause epididymitis.

Women often develop ulcers on the labia.

The size of the ulcer is similar to or deeper than that of the mouth ulcer, and the pain is obvious.

Generally, the interval between attacks is much longer than that of oral ulcers, ranging from several months or one to several years.

4.
Skin lesions Erythema nodosa, folliculitis, boils, ulcers, etc.
appear on the skin, as well as erythema multiforme.

In addition, Behcet’s disease also manifests as some special symptoms, such as joint pain, redness and swelling, cardiovascular system damage (pericarditis, aneurysm, vascular infarction), digestive tract disease (peptic ulcer, hemorrhage), nervous system damage (meninges brain Inflammatory syndrome, organic insanity, etc.
).

These symptoms are an important basis for the diagnosis and classification of Behcet's disease.

02There are still these differences between men and women with Behcet's disease! This study included 860 patients with Behcet’s disease from the Department of Rheumatology and Immunology, East China Hospital, Fudan University, from September 2012 to January 2020.
It complies with the revised standards of the International Research Group (ISG), the revised standards of Japan, the standards of China’s Cheng and Zhang, and Behcet’s Any one of the International Standards for Diseases and Diseases (ICBD), verified and diagnosed by at least two rheumatologists, records detailed clinical and laboratory data, including demographic data, laboratory evaluations, imaging studies, and pathological results.

At the same time, the study assesses organ involvement by examining the patient’s symptoms, past medical history, physical examination, laboratory examination, imaging examination and endoscopic examination results.

All clinical manifestations include ocular (such as uveitis) symptoms, intestinal Behcet (attention should be paid to distinguish from Crohn’s disease), vascular involvement (such as deep vein thrombosis), heart disease (such as valvular regurgitation, etc.
), nerves System (CNS) disease. The severity of the disease was evaluated by the Krause score; at the same time, SPSS was used to analyze the relative risk (RR) of different clinical variables of different genders, and it was statistically significant when P<0.
05.

Table 1: Clinical manifestations and gender differences in 860 patients.
Research data shows that oral ulcers are the most common symptom of Behcet's disease patients (99.
5%), the intestine is the most common main organ involved (20.
7%), followed by uveitis , Accounting for 18.
0%.

There are gender differences in some clinical manifestations, which are mainly reflected in the involvement of the eyes (RR=2.
32, P＜0.
0001), uveitis (RR=2.
72, P＜0.
0001), vascular involvement (RR=2.
00, P= 0.
004), heart disease (RR=5.
46, P<0.
0001), central nervous system disease (RR=2.
95, P=0.
007), skin lesions (RR=1.
15, P=0.
021) and folliculitis (RR=1.
70, P ＜0.
0001) has a higher correlation with male patients with Behcet’s disease; female patients with Behcet’s disease are more likely to develop genital ulcers than male patients (RR=0.
840, P<0.
0001).

 There was no gender difference in the incidence of anterior uveitis, intestinal lesions (including intestinal erosions, ulcers and perforations) and esophageal ulcers, and the others were not statistically significant.

The 035 phenotypes are more suitable for Chinese Behçet's disease classification! After clustering these 860 patients with Behcet’s disease, the study observed that these patients can be divided into 5 subgroups with different characteristics (C1-C5), and each subgroup represents a different clinical phenotype (Figure 1 ): Figure 1 Clinical characteristics of patients with 5 types of Behcet's disease Mucocutaneous Behcet's disease (C1, n=307, 35.
7%) This category is the largest group of patients with Behcet's disease, mainly women, all with genital ulcers.
The prevalence of erythema nodosa was 40.
7%, and the prevalence of papule-like lesions was 20.
8%. Except for 1 case of myelodysplastic syndrome (MDS), the other patients had no symptoms of major organ involvement, and the overall condition was mild; articular Behcet's disease (C2, n=124, 14.
4%) all patients had joint involvement, male to female ratio It was 0.
97:1; Except for 18 cases of small bowel disease and 2 cases of esophageal disease, all patients with Behcet's disease had no symptoms of organ involvement.
The median value of Krause score was 4; Gastrointestinal Behcet's disease (C3, n= 156, 18.
1%) The male to female ratio was 1.
08:1; the patients mostly showed intestinal involvement (92.
3%), including intestinal erosion (5 cases), ulcers (117 cases) and perforation (22 cases), without eye or heart Vascular disease.

The median value of Krause score is 4; all subjects with uveitis-type Behcet's disease (C4, n=142, 16.
5%) have ocular lesions, mainly young men, with a male to female ratio of 2.
94:1; Intestinal and cardiovascular diseases are rare.

The median score of Krause is 6; cardiovascular Behcet’s disease (C5, n=131, 15.
2%) has a male-to-female ratio of 2.
54:1; patients are mainly male, and 44.
3% of Behcet’s disease patients have vascular disease, of which deep There were 29 cases of venous thrombosis, 7 cases of large vein (venous, liver) thrombosis, and 46 cases of arterial thrombosis or aneurysm.

And central nervous system diseases are more common (19.
8%), and the median value of Krause score is 6.

The researchers stated that the five phenotypes of Chinese Behcet's disease patients can be summarized into these five phenotypes based on the clinical manifestations of different phenotypes, which will help rheumatologists to identify the clinical characteristics of patients in clinical work and understand the onset of patients with specific phenotypes.
The similarity of the mechanism.

Based on these 5 phenotypes, it may be possible to speculate on the patient's disease development.

For example, young male patients with Behcet's disease who suffer from uveitis are less likely to have intestinal lesions; female patients with Behcet's disease are more likely to develop genital ulcers.

In addition, the clarification of the clinical phenotype will help the clinical development of more precise treatment plans.

However, more studies are still needed to confirm whether these five phenotypes can explain the differences in the pathogenesis of Behcet's disease, so as to guide clinical treatment.

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