Ferty syndrome

Introduction to Felty Syndrome Felty syndrome was first reported by Felty (1924), which is characterized by rheumatoid arthritis with splenomegaly and leukopenia, which is different from common rheumatoid arthritis. Hanrakar et al. (1932) referred to a disease with rheumatoid arthritis, splenomegaly, and leukopenia as the main cause of the disease. Many cases have been reported so far, and most of them believe that this syndrome is a subtype of rheumatoid arthritis. In recent years, it has been clarified that this syndrome has characteristic immune abnormalities and gradually becomes an independent disease. basic knowledge The proportion of illness: the incidence rate is about 0.003%-0.007% Susceptible people: no special people Mode of infection: non-infectious Complications: peripheral neuritis anemia

The cause of Felty syndrome

Hematology research (25%):

(1) Shortening of granulocyte life: Bishop (1971) found that granulocyte half-life was shortened in 4 patients, which is considered to be a major factor in neutropenia.

(2) Decreased ability of granulocyte production: Welch et al (1950) reported that leukopenia may be the result of inhibition of bone marrow function by the spleen. Greenbarg (1973) used granulocyte colony formation assay to find granulocyte precursors in patients with this syndrome. The proliferative capacity of the cells is decreased, and Gupta (1975) reports a decrease in colony stimulating factors as granulocyte proliferation promoting factors in serum and urine.

(3) granulosa cells are marginal hyperthyroidism: Wincenta et al. (1974) pointed out that the marginal hyperthyroidism and its accompanying granulocytes are transferred from the circulating pool in the peripheral blood to the marginal pool including the spleen, which can cause peripheral blood granulocytopenia. .

Immunology research (35%):

(1) There is a serum substance that causes a decrease in granulocytes: Calabersi (1959) intravenously injects the serum of a patient with this syndrome into a normal person, and as a result, a transient granulocyte decline occurs, so that a certain serum substance may be present in the patient's serum. Causes granulocyte decline.

(2) granulocyte-specific antibodies: the positive rate of serum antinuclear antibodies in patients with this syndrome is as high as 75% or more. Bnannei (1970) confirmed that the serum antinuclear antibody has no complement-binding ability in patients with this syndrome, and is called non-organ-specific antinuclear. Antibodies, and later proved that in addition to antinuclear antibodies in the serum of the disease, 80% to 90% of patients still have granulocyte-specific antibodies, which have complement-binding ability, may cause neutropenia, the granulocyte-specific antibody It can be found in rheumatoid arthritis joint fluid, which is generally considered to be a component of moderate-sized circulating immune complexes. It has also been reported that after splenectomy, peripheral granulocyte-specific antinuclear cells appear in the serum of patients with advanced disease. The antibody disappears during the remission period, and the IgG-IgG-RF intermediate complex may be formed depending on the law that the IgG-RF and the specific granulocyte anti-nuclear antibody almost simultaneously appear and disappear.

(3) Anti-granulocyte antibody: The serum of the patient was determined by an indirect anti-human globulin consumption test, and an anti-granulocyte antibody was found, and the antibody belongs to an IgG-type immunoglobulin.

(4) IgG and serum granulocyte-bound IgG closely related to granulocytes: Logue (1976) reported granulocyte surface or serum of patients with this disease by quantitative anti-human globulin consumption test and I staphylococcal protein A binding test The IgG in the IgG was significantly increased compared with the control group, and the value was decreased after splenectomy, which not only reflected the presence of anti-granulocyte antibodies, but also proved that such IgG is a constituent of the soluble immune complex.

(5) The ability of granulocytes to phagocytose immune complexes: Ziff et al (1976) used ultracentrifugation, C1q binding assay, monoclonal antibody, rheumatoid factor to detect the serum of patients with this disease, and found a high positive rate, complement binding ability Strong immune complexes exist, such immune complexes can be engulfed by normal human granulocytes, while the patient's granulocytes contain inclusion bodies composed of IgG and complement, indicating a decline in the phagocytic capacity of white blood cells.

(6) Inhibitory T cell-mediated mechanism: Abdou (1978) pointed out that normal human bone marrow cells were cultured with peripheral blood, bone marrow cells or spleen cells of patients with this disease, and the number of colony forming units was less than that of the control group. Inhibition may indicate that inhibition of inhibitory T cell formation is associated with decreased leukocytes in this disease.

Pathogenesis

1. Pathogenesis The etiology and pathogenesis of neutropenia have not yet been elucidated. Although several theories have been proposed, none of them can explain the whole picture of Felty syndrome. Hypersplenism is often thought to cause granulocytes. The reason for the decrease, but the spleen is not swollen, there may be neutropenia. The white blood cells are initially raised after splenectomy. The longer the follow-up period, the higher the frequency of granulocyte recurrence, so the spleen is most indicated. Certain effects, some studies show that humoral factors play a role in the pathogenesis of Felty syndrome, such as the blood loss of patients with Fertil syndrome to normal people can cause white blood cell decline, under normal circumstances, reducing urinary testosterone (etiocholanolone) Granulocytes can be mobilized from the bone marrow, and patients with Fertil syndrome have no response to reducing urinary testosterone. The plasma input into the patient can also block the granulocyte mobilization of this preparation. In addition, neutropenia and circulating immune complexes are also found. Related.

Recent studies have found that in a quarter of patients with Felty syndrome, there is an abnormal lymphocyte population, this abnormal lymphocyte and cells seen in large granular T-cell leukemia. Similar, but no T cell receptor beta and gamma chain rearrangement, this cell is similar to natural killer cells, but does not express all the markers of this series of cells, in these cases, neutropenia may be antibody dependent Sexual cytotoxic or large granular lymphocytes inhibit bone marrow.

Finally, don't forget the abnormal distribution of white blood cells. About half of the vascular granulocytes adhere to the vascular endothelium. This marginal pool increases in almost all of the Felty syndrome, and in some cases may be the main cause of neutropenia. Increased white blood cells in joint exudation and lower extremity ulcers suggest that marginal pools do exist even in the circulation of granulocytes.

In summary, a variety of factors (including antibodies, immune complexes, cellular immunity) alone or in combination are responsible for neutropenia.

2. Pathological joints are typical manifestations of different stages of rheumatoid arthritis, splenomegaly (240 ~ 2400g), non-specific changes to have a large germinal center, excessive proliferation of spleen lymphoid follicles, reticular cells and pulp Cells, liver lesions are interstitial lymphocyte infiltration and fibrosis.

Ferti syndrome prevention

1. Eliminate and reduce or avoid the disease factors, improve the living environment, improve the development of good habits, prevent infection, pay attention to food hygiene, and rational diet.

2. Pay attention to exercise, increase the body's ability to resist disease, do not fatigue, excessive consumption, quit smoking and alcohol.

3. Early detection and early diagnosis and early treatment, establish confidence in the fight against disease, adhere to treatment.

Fertil syndrome complications Complications peripheral neuritis anemia

Complications include scleral laminitis, peripheral neuritis and pericarditis. 60% of patients have more than one secondary infection, histologically, liver involvement, most patients have mild to moderate anemia, can also occur Glen syndrome, pleurisy, peripheral neuropathy, mild liver enlargement, swollen lymph nodes, weight loss, etc.

Fertil syndrome symptoms Common symptoms Spotted lymph nodes swollen shoulder joint pain Ascites edema

In a small number of patients with this disease, splenomegaly and neutropenia may be earlier than rheumatoid arthritis. Joint lesions are often more serious than general rheumatoid arthritis. There are many bone erosions and deformities, but there are also light ones. About 1/3 of cases have Inactive synovitis, the size of the spleen varies greatly, patients may have the characteristics of typical Fertil syndrome such as neutropenia and rheumatoid arthritis, but no splenomegaly, intrinsic leukopenia is neutral The relative and absolute decrease of cells, the degree of neutropenia can be reduced from mild to complete disappearance. Most patients do not often check blood routine, so the rate of neutropenia in the early stage of the disease is rarely known, but in some patients for several weeks. The granulocytes can be reduced from normal to very low levels. The white blood cell count often fluctuates to a considerable extent. The mild reduction can return to normal, but the severe reduction rarely returns to normal. Under infection or other stress conditions, white blood cells can recover. Normal, but rarely more than normal, in addition to the above-mentioned triad, Fertil syndrome has other characteristics, intrinsic more than the general rheumatoid arthritis; Weight loss and general malaise are common and can occur several months before diagnosis. Brown pigmentation is a common manifestation of this disease. It is more common in exposed parts of the extremities. It is not a specific phenomenon of Felty syndrome, and may be caused by small vessel disease. Red blood cell deposition and extravasation; the incidence of calf ulcers is higher, may be caused by vasculitis, local biopsy shows arteritis with fibrin-like necrosis and mild inflammation, vascular occlusion, other vasculitis manifests scleral outer layer Inflammation, peripheral neuritis and pericarditis.

About 60% of patients with this disease have more than one secondary infection. The infection site is more common in the skin and respiratory tract. The pathogenic bacteria are mostly common staphylococcus, streptococcus and gram-negative bacilli. The infection may be related to neutropenia. However, the degree of neutropenia is not significantly related to the severity of infection, so it is speculated that neutrophil function is more important than number in combating infection. Recent studies have shown that neutrophils of Fertil syndrome phagocytose foreign bodies. The ability to release active oxygen is reduced. After the normal human polymorphonuclear leukocytes are incubated with the plasma of the Fertil patient, the function of killing the cell bacteria is also reduced, and the degree of reduction is combined with the content of the immune complex in the circulation. The amount of IgG on polymorphonuclear leukocytes is related.

Fertil syndrome can be associated with hepatic nodular regenerative proliferation, a characteristic liver lesion rarely seen in lupus erythematosus and other connective tissue diseases, histologically, liver involvement, seen in 60% Fertil patients, the same number of patients may have abnormal liver function, other patients with abnormal histology, but normal liver function, tissue abnormalities including nodular regeneration, portal fibrosis, hepatic sinusoid lymphocytes Infiltration, Kupffer cell proliferation, 25% of patients with Fertil syndrome have nodular regenerative proliferation, liver biopsy does not necessarily achieve nodular tissue and the surrounding tissue is normal, so its diagnostic value is limited, portal vein High pressure, esophageal varices and gastrointestinal bleeding are common in the Fertil syndrome with regenerative proliferation of hepatic nodules, but these patients usually do not have ascites and liver function deterioration, which is different from cirrhosis, except for neutral granules. In addition to cell reduction, lymphocytes in the circulation can be reduced, but not obvious, monocytes and eosinophils are not reduced, occasionally eosinophilia, platelets normal or reduced, but rarely caused Erythema, while active rheumatoid arthritis, platelets tend to be increased, most patients with mild to moderate anemia, may be related to shorten the life span of red blood cells, anemia splenectomy may improve this.

The joint performance is not significantly different from typical rheumatoid arthritis. It usually occurs after several months to several years after the joint symptoms appear. Therefore, the patient is more than 40 to 50 years old. Because of the decrease of granulocytes, infection often occurs repeatedly. In particular, oral mucosa, sinusitis, bronchitis, carbuncles, etc., other manifestations of visible skin lesions, especially skin pigmentation at the exposed site, skin-mucosa-calf ulcer, purple spot, Sjogren's syndrome, pericarditis, pleurisy may also occur , peripheral neuropathy, mild liver enlargement, swollen lymph nodes, weight loss, etc.

Check of Ferdi syndrome

There are changes in various cell lines of the blood system. In addition to the mild anemia caused by the decrease of serum iron binding, which is common in rheumatoid arthritis, the shortening of red blood cell life is also characterized by a slight decrease in platelets and a prominent neutropenia. In severe cases, it can be as low as 0.1×109/L or less.

1. Bone marrow images show granulocytic maturation disorders.

2. Immunological examination of rheumatoid factor and anti-nuclear antibodies are often positive.

Ferti syndrome diagnosis and identification

Diagnostic criteria

What diseases are easily confused with Felty syndrome? With rheumatoid arthritis, splenomegaly and neutropenia three major signs, combined with other immunological examination to determine the diagnosis, but should also be combined with clinical manifestations, medical history and physical examination to further establish the diagnosis.

Important clinical manifestations, medical history and physical examination:

1. Patients can have a variety of symptoms, many of which are needed for diagnosis: patients often have a history of peak fever, usually > 39 ° C, fever is a peak every day or every 2 days, usually not hot during the interval, Fever often occurs in the afternoon or evening, forming a regular cycle.

2. Usually accompanied by fever is salmon rash or maculopapular rash: Still rash usually appears on the trunk and limbs, but also can be quickly produced by friction (Koebner phenomenon), which is difficult due to its transient characteristics. Found by routine inspection.

3. Arthritis and joint pain can affect the knee, wrist, ankle, elbow: PIP and shoulder pain are common symptoms of AOSD. Single joint joint invasion is slow and slight at the beginning, but can lead to clinical more For severe arthritis, inflammatory fluids can be found in joint punctures.

4. Other clinical symptoms include pharyngitis, lymphadenopathy, splenomegaly, hepatomegaly and cardiopulmonary invasion (pericarditis, pleural effusion or pulmonary infarction). However, attention should be paid to the identification of spotted syndrome, which has no joint symptoms and no positive findings from special immunological tests.

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