FELTI SYNDROME

From Big Medical Encyclopedia

FELTI SINDR (A. R. Felt at, an amer. doctor, sort. in 1895) — the form of a pseudorheumatism which is characterized in addition to damage of joints, a neutropenia and a splenomegaly. For the first time the syndrome is described by Felti in

1924.

According to Short (1. A, Short, 1957) with sotr., T. the page is observed less than at 1% of patients with a pseudorheumatism (see). Women at the age of 50 years are ill more often and are more senior.

Etiology and pathogen e z. The origins are difficult and up to the end are not studied. The origin of a neutropenia (see the Leukopenia) is connected with several reasons. On the one hand, the circulating cell-bound immune complexes in a large number found in patients with F. pages, can break functions of neutrophils, promoting their accelerated phagocytosis first of all in a spleen. It is confirmed by works as V. A. Nasonova and sotr. (1980), the Hard (E. R. Hurd, 1977), to-rye found in cytoplasm of neutrophils of inclusion, consisting of immunoglobulins and a complement. It is not excluded that the main role in this process is played by specific antibodies to leukocytes, including and neutrophylic, found in patients with F. page. Assume also that the humoral and cellular factors oppressing maturing of leukocytes in marrow take part in development of a neutropenia.

Clinical carat of t and - N and. Felti's syndrome develops at patients with a pseudorheumatism, as a rule, in the presence in blood serum of a rhematoid factor (see) on average in 10 years from the beginning of a disease. Damage of joints is characterized by polyarthritis of preferential small joints of brushes and feet, typical for a classical pseudorheumatism, degree of manifestation to-rogo can be various.

At patients with F. pages often are found various system manifestations of a pseudorheumatism: hypodermic small knots, a polyneuropathy (see the Neuropathy in neurology), the expressed muscular atrophy (see the Atrophy muscular), a bystry lose of weight, fever, an episcleritis (see the Sclerite), a generalized lymphadenopathy, a hepatomegalia, a polyserositis (see), myocarditis (see). More than at a half of patients signs of a syndrome of Shegre-na are noted (see Shegren a syndrome). Decrease in the main functions of leukocytes — phagocytosis, a chemotaxis, bactericidal activity — explains the increased frequency of developing of infectious diseases at this syndrome.

The sizes of a spleen are usually increased considerably (see the Splenomegaly), its weight is on average 4 times more than norm. F is possible. page and without splenomegaly. At early stages of development of a syndrome the leukopenia can be non-constant.

The diagnosis does not cause difficulties. It is put on the basis of emergence in patients with a pseudorheumatism of a neutropenia and a splenomegaly. In blood find a leukopenia (less than 2000 leukocytes in 1 mkl blood) and a neutropenia. Degree of a leukopenia and a neutropenia can fluctuate. At a research of red marrow in most cases reveal a myeloid hyperplasia with relative increase in quantity of unripe forms. For patients with F. the page is characteristic disturbance of a humoral link of immunity. As a rule, in blood serum find rhematoid factors in high credits, a hypergammaglobulinemia, especially increase in IgG and IgM (see Immunoglobulins). Often reveal antinuclear antibodies, especially antigra-nulotsitarny, circulating cell-bound immune complexes of a transition type. In neutrophils of blood by method of an immunofluorescence (see) it is possible to find the large inclusions consisting of IgG, IgM and a complement. Similar inclusions at incubation appear also in donor leukocytes if previously to process them blood serum of patients with F. page.

Differential diagnosis is carried out with myeloproliferative diseases (see), at the expressed lymphadenopathy — with lymphoma (see the Lymphoma), a sarcoidosis (see), tuberculosis (see), and at a hepatomegalia — with cirrhosis (see).

The l e e in N and e developed h insufficiently. At development F. pages usually apply corticosteroids in average doses (20 — 30 mg of Prednisolonum a day). However at a dose decline, as a rule, the leukopenia and a neutropenia develops again. Cases of successful use of salts of lithium are described, to-rye are capable to stimulate leykotsitopoez. Results of prolonged use of lithium at F. pages are unknown, use of drug is limited because of side reactions.

The splenectomy is shown to patients with the expressed neutropenia, a splenomegaly and frequent infections (see). At most of patients it leads to normalization of level of leukocytes and neutrophils in blood, however at a part of patients [apprx. 24% according to survey data of R. Ri-nols] effect of operation unstable.

The issue of expediency of use at F is not resolved. page of such most effective in treatment of a pseudorheumatism of means as salts of gold, D-penitsilamin and cytotoxic immunodepressants since they are capable to cause a leukopenia. Nevertheless, in separate observations it is established that, suppressing activity of a pseudorheumatism, these drugs are capable to reduce also expressiveness of manifestations F. page.

The forecast is serious, especially in case of accession of infectious diseases.

Prevention is not developed.

Bibliography: Nasonova V. A., At me-t about in and M. D. and Speransky A. I. Kliniko-immunologichesky features of a syndrome of Felti, Rubbed. arkh., t. 52, No. 4, page 105, 1980, bibliogr.; Decker J. L. and. P 1 about t z P. H. Extra-articular rheumatoid disease, in book: Arthritis and allied conditions, ed. by D. J. Me Carty, p. 470, Philadelphia, 1979; F e 1 t at A. R. Chronic arthritis in the adult, associated with splenomegaly and leucopenia, Bull. Johns Hopk. Hosp., v. 35, p. 16, 1924; To e 1-1 e at W. N. a. o. Textbook of rheumatology, p. 964, Philadelphia, 1981.

V. A. Nasonova.

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