Decreases in rates of hospitalizations for manifestations of severe rheumatoid arthritis, 1983-2001.

OBJECTIVE: To determine whether the rates of hospitalization for 4 manifestations of severe rheumatoid arthritis (RA), which are used as indicators of long-term health outcomes, have changed from 1983 to 2001. METHODS: Data on all patients with RA who were hospitalized with rheumatoid vasculitis or to undergo splenectomy for Felty's syndrome, cervical spine fusion for myelopathy, or total knee arthroplasty at hospitals in California were abstracted from a state hospitalization database. Changes in rates of hospitalization from 1983 to 2001 were examined in this serial cross-sectional study. RESULTS: Rates of hospitalization for rheumatoid vasculitis and splenectomy in Felty's syndrome decreased progressively over time. The risk of hospitalization for rheumatoid vasculitis was one-third lower in 1998-2001 than in 1983-1987. The risk of hospitalization for splenectomy in Felty's syndrome was 71% lower in 1998-2001 than in 1983-1987. There were no significant decreases in the rates of hospitalization for cervical spine surgery or total knee arthroplasty (primary and revision), although in 1998-2001 there was a reversal of the trend of increasing rates of total knee arthroplasty. The risk of hospitalization for primary total knee arthroplasty was significantly lower in 1998-2001 than in 1990-1993 (rate ratio 0.90, 95% confidence interval 0.86-0.95; P < 0.0001). CONCLUSION: Rates of hospitalization for rheumatoid vasculitis and splenectomy in Felty's syndrome have decreased over the past 19 years, and there has been a recent decrease in the rates of primary total knee arthroplasty in patients with RA. Although several factors may account for these decreases, these findings suggest that since the early 1980s, the long-term health outcomes of patients with RA have improved.

[Felty's syndrome]. / Feltys syndrom.

BACKGROUND: Felty's syndrome is a complication of rheumatoid arthritis whereby patients develop neutropenia of varying severity and splenomegaly. The major sources of morbidity and mortality are recurrent local and systemic infections, although some patients remain asymptomatic. MATERIAL AND METHODS: In this paper two patients with Felty's syndrome are presented. RESULTS: One patient had recurrent infections. Clinical manifestations, laboratory features and different modalities of treatment are reviewed. INTERPRETATION: Splenectomy has long been standard therapy, but disease modifying antirheumatic drugs (such as gold salts and methotrexate) and colony stimulating factors should also be considered in Felty's neutropenia complicated with infections.

Possible feedback control of white blood cells: effect of splenectomy in Felty's syndrome.

Feedback control of the white blood cell (WBC) count was analyzed in 20 cases reported by Coon, before and after splenectomy in Felty's syndrome. In only one case did the total white cell count fail to rise postsplenectomy, while in a second case the polymorphonuclear (poly) count did not exceed the preoperative value. Both of these patients had initial poly counts above 1000/mm3. If the preoperative poly count was 35% or greater of total WBC, the postoperative percentage was lower in five out of six cases. A comparison was made between pre- and postoperative poly counts, and three cases were noted to be underresponsive. However, two had a later rise in polys ('late responders'). A non-poly was defined as total WBC minus polys; a relationship was noted between pre- and post-operative non-polys, described by the difference between initial and postoperative counts. Thus, there is evidence for feedback control of the white cell count in Felty's syndrome after splenectomy.

GM-CSF and G-CSF in Felty's syndrome.

The risk of infection is increased in patients with Felty's syndrome, neutropenia being one of the main reasons for the susceptibility to infection. We report the case of a 56-year-old patient with Felty's syndrome in whom successive therapy with GM-CSF, splenectomy, and G-CSF was tried because of recurrent severe infections. Therapy with GM-CSF and G-CSF resulted in improvement of neutropenia and in successful treatment of cutaneous and pulmonary infections.

[Improvement of pancytopenia and articular and by splenectomy in a patient with Felty's syndrome].

A forty seven year-old woman with a 18 years history of rheumatoid arthritis presented with recurrent infection and pancytopenia. A diagnosis of Felty's syndrome was made hand on clinical and laboratory findings. In spite of drug therapy using nonsteroidal anti-inflammatory drugs, D-penicillamine and corticosteroid, the hematological abnormalities and active joint symptoms continued. Recurrences of severe bacterial infection and the rupture of esophageal varices necessitated splenectomy. Soon after the operation, dramatic improvement of hematological abnormalities were observed. Furthermore, after 3 months postoperatively, marked decrease in rheumatoid activities and disappearance of subcutaneous nodules were noticed. Titer of rheumatoid factor also showed a significant reduction and became negative 6 months after the operation. Although the efficacy of splenectomy on hematological abnormalities in Felty's syndrome has been well documented, its effect on rheumatic disease has not been clarified. The case presented here might suggested that suppression of reticuloendothelial system may have therapeutic effects in rheumatoid arthritis. The possible mechanisms involved in these findings have been discussed.

Splenectomy as treatment for nonhealing soft tissue defect after total knee arthroplasty in a patient with Felty's syndrome.

We describe the apparent remarkable effect of splenectomy on wound healing in a patient with rheumatoid arthritis and Felty's syndrome. The patient had previously undergone a knee replacement with 4 months of failure of the surgical wound site to heal or close. The wound, which had broken down and dehisced on 3 separate occasions after plastic surgical attempts at closure, healed cleanly and without recurrence within 3 weeks of splenectomy and plastic repair at the same operation.

Pneumococcal septic arthritis after splenectomy in Felty's syndrome.

A patient with Felty's syndrome who developed bilateral knee septic arthritis and septicaemia due to Streptococcus pneumoniae is described. She had had a previous splenectomy for symptomatic thrombocytopenia, having received pneumococcal vaccine before the operation. Measurement of antibody to the 23 vaccine serotypes showed protective concentrations before infection to just two. The infecting serotype was not represented in the vaccine, but a vigorous antibody response to this serotype occurred. The patient also developed glomerulonephritis due to immune complex deposition.

The spleen in Felty's syndrome: a histological, morphometrical, and immunohistochemical study.

A histological study employing morphometrical and immunohistochemical methods was performed in 3 spleens removed because of Felty's syndrome (FS). A comparison was made with control spleens and with spleens removed from patients with idiopathic thrombocytopenic purpura (ITP) or autoimmune hemolytic anemia (AIHA). In FS, the spleen is enlarged, mainly due to expansion of the red pulp. The sinuses are enlarged, too, and contain many macrophages. 2 of our specimens showed many hyperplastic germinal centers; the 3rd contained no germinal centers. Only in the former cases did splenectomy lead to an increase in the number of circulating granulocytes. Immunohistologically, FS spleens differed from the control, the ITP, and the AIHA spleens. The results have provided further evidence indicating that the pathogenesis of granulocytopenia in FS differs from that of autoimmune hematocytopenias, and have given rise to the hypothesis that, in FS, the spleen is the site of interaction between immune complexes and granulocytes.

Felty's syndrome in black Americans: a case report.

This is a case report of a black woman with Felty's syndrome, who had recurrent, severe leg ulcers refractive to the usual treatment. The leg ulcers healed promptly after splenectomy and there has not been a recurrence of these lesions after three years' follow-up. The rarity of Felty's syndrome in blacks is highlighted and the literature on this aspect is reviewed. Information on seven patients with Felty's syndrome seen at the University of Cincinnati Medical Center is provided and the relationship of skin ulcers to splenectomy reviewed.

Felty's syndrome: when is splenectomy indicated?

The spectrum of disease was assessed in 43 patients with Felty's syndrome. Twenty-three underwent splenectomy. Although complete remission of neutropenia occurred in only two nonoperated patients, most of the patients had no serious infections during the subsequent period of observation. Operative morbidity was minimal in the splenectomized patients. On the basis of natural history of this condition, splenectomy should probably be restricted to neutropenic patients with serious or recurrent infections, patients with severe anemia requiring transfusions, those with the rare circumstance of profound thrombocytopenia, and those with non-healing leg ulcers.

111Indium-labelled white blood cells in the diagnosis of Felty's syndrome.

The use of isotope scanning of the spleen in conjunction with 51Cr-labelled red blood cells has become an established technique in the evaluation of patients with hypersplenism. As far as we are aware the similar technique using labelled white blood cells to demonstrate splenic sequestration in a neutropenic patient has not been described. We report a case where this technique proved valuable in confirming the diagnosis and in predicting a favourable response to splenectomy.

Neutrophil kinetics in Felty's syndrome.

In 19 patients with Felty's syndrome, marrow production of neutrophils and neutrophil distribution were studied. Despite accelerated marrow release and disappearance of mature blood neutrophils, there was little or no increase in the marrow mitotic pool or in vitro progenitor cells. Only two patients had an increase in marrow neutrophils and precursors. Antineutrophil antibody was detected in seven of nine patients studied. Neither abnormal margination of blood neutrophils nor impaired marrow release of cells was detected. Skin exudate cellularity tended to correspond to prior history of infections, asymptomatic patients having more cellular exudates. Sustained neutrophil increments were observed in six of 10 patients following splenectomy, but in no patient did neutrophil kinetics return completely to normal. Three of four patients who failed to respond to splenectomy with sustained increments in blood neutrophils had a reduced mass of marrow neutrophils and neutrophil precursors when studied prior to splenectomy. No diminution in neutropenia was observed in any of five patients treated with lithium carbonate. This study indicates that multiple factors are involved in the pathogenesis of neutropenia in Felty's syndrome. In particular, neutropenia was associated with inadequate marrow granulocytopoiesis. The severity of the impairment, as determined by the mass of marrow neutrophils and precursor cells, may be useful in predicting response to splenectomy.

Felty's syndrome without splenomegaly.

A woman with chronic rheumatoid arthritis and severe granulocytopenia but without splenic enlargement by physical examination or radionuclide scanning was studied for granulocyte-bound immunoglobulin G (IgG) and serum antigranulocyte antibodies. Prior to splenectomy 73 to 110 X 10(-14) g/cell of IgG were detected on the patient's granulocytes, a value in the range (20 to 220 X 10(-14) g) found in 16 patients with classic Felty's syndrome. Granulocyte-bound IgG in 21 patients with rheumatoid arthritis without Felty's syndrome was less than 20 X 10(-14) g. Following splenectomy, the patient had a partial correction of her peripheral granulocyte count, and granulocyte bound IgG was repeated less than 20 X 10(-14) g/cell. When paraformaldehyde-fixed granulocytes, obtained either from normal donors or from the patient after splenectomy, were incubated in the patient's serum obtained before splenectomy, more IgG was bound than with control serums from patients with rheumatoid arthritis. Similar results were obtained when serums from patients with classic Felty's syndrome were incubated with paraformaldehyde-fixed granulocytes. Thus, patients with rheumatoid arthritis without overt splenic enlargement may have pathophysiologic Felty's syndrome, and in vitro studies such as these may be used to define this process.

Splenectomy for Felty's syndrome. Clinicopathological study of 27 patients.

The major clinical and pathological features and the long-term follow-up of 27 patients with Felty's syndrome who were treated with splenectomy for sever granulocytpenia and for acute, chronic, or recurrent infection were studied. Granulocyte counts rose within days in most patients, although slow responses and transient granulocytopenia did occur; only 12% of the patients had persistent or recurrent granulocytopenia. Infections resolved promptly in 77% of the patients, more slowly in the remainder, and only one patient had new problems of infection after aplenectomy. Splenic enlargement, present in all but one case, was attributable to expansion of the sinusoidal pulp. The most substantial pathological features of immune stimulation included germinal center hyperplasia and prominent clusters of plasma and preplasma cells within sinuses.

Felty's syndrome. Clinical and serological analysis of 34 cases.

Review of 34 cases of Felty's syndrome showed this to be a form of 'super' rheumatoid disease because of the severity of joint disease, the prominence of extra-articular features and the remarkable incidence of infection. The response to splenectomy in these 34 patients was shown by a return towards normal of peripheral blood abnormalities and a decrease in bone marrow granulopoiesis. Although some patients remained free of infection after splenectomy, others have continued to have infections despite the return of white blood cell counts to normal levels. Although splenectomy and subsequent increase in white blood cell levels may be beneficial, our experience suggests that other factors are important in the susceptibility to infection of Felty's syndrome patients. Moreover, we think that splenectomy may have been instrumental in the fatal infection of one of our patients.