Porto-Sinusoidal Vascular Disease as the Cause of Portal Hypertension in Felty's Syndrome: A Case Report and Literature Review.

Felty's syndrome (FS) is a disorder wherein patients with rheumatoid arthritis develop splenomegaly, neutropenia, and in some cases, portal hypertension without underlying cirrhosis. Esophageal variceal bleeding is a complication of FS in patients with portal hypertension. In contrast to splenectomy, few reports exist on the management of variceal bleeding with endoscopic therapy. Moreover, the long-term outcome has not been reported. We present a patient with esophageal variceal bleeding due to portal hypertension secondary to Felty's syndrome. The patient was followed up for two years postendoscopy intervention. Literature review was performed and the histological features of portal hypertension in FS are discussed. The patient presented with a typical triad of rheumatoid arthritis (RA), splenomegaly, and neutropenia and was diagnosed as Felty's syndrome in 2012. She was admitted to our hospital in September 2017 for esophageal variceal bleeding. At the time of admission, her liver function test was normal. Abdominal CT showed no signs of cirrhosis and portal vein obstruction. Liver biopsy further excluded diagnosis of cirrhosis and supported the diagnosis of porto-sinusoidal vascular disease (PSVD), which was previously named as noncirrhotic idiopathic portal hypertension (NCIPH). An upper abdominal endoscopy revealed gastric and esophageal varices. A series of endoscopies was performed to ligate the esophageal varices. The patient was followed up for two years and did not show rebleeding. In conclusion, comorbid PSVD might be a cause of portal hypertension in FS patients. The present case had excellent outcome in two years, which supported the use of endoscopic therapy for the management of variceal bleeding in FS patients. Further large prospective study is needed to confirm the findings.

Síndrome de Felty atípico / Atypical Felty's syndrome

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Síndrome de felty: reporte de caso / Felty syndrome: a case report

El síndrome de Felty (SF) es una manifestación extraarticular de la artritis reumatoide (AR), caracterizada por: AR, neutropenia y esplenomegalia es infrecuente aunque severo. Se desarrolla en personas de mediana edad, con una historia de AR. El diagnóstico es estrictamente clínico y el tratamiento está enfocado a disminuir el dolor articular, las infecciones y evitar las deformidades óseas. Presentamos el caso de una mujer de 46 años, que ingresa por el servicio de emergencias con dificultad respiratoria, fiebre y neutropenia siendo diagnosticada con neumonía y encontrándose en estudio. El trabajo de seguimiento mostró esplenomegalia, y fue diagnosticada con AR de varios años de evolución. Lo enunciado junto a otras evidencias ayudaron a que fuera diagnosticada con SF. El interés del caso es presentar una rara complicación de la AR, que en ocasiones puede ser sub-diagnosticada. Los pacientes con neutropenia continua, altos nivel de factor reumatoide y auto anticuerpos deben ser sospechosos de desarrollar el SF como una presentación inicial de AR o con una AR no diagnosticada como en este caso.

Felty's Sindrome (SF) is an extraarticular manifestation of rheumatoid arthritis (AR), characterized by: AR, neutropenia and splenomegaly which is infrequent but hursh. It is developed in people of middle age and with an history of AR. The diagnosis is strictly clinical and the treatment is focused on subside the joint pain, the infections and relieve osseus deformities. We report the case of a woman of 46 years old who entered by the service of emergency with respiratory difficult, fever and neutropenia who was diagnosed with pneumonia in study. The following work shows splenomegaly and was diagnosed with AR of many years of evolution. It stated along with another evidences supported the diagnosis of SF. The particular interest ofthis case is to presentan unusual complication of AR, which occasionally could be sub-diagnosticated. The patients with continuous neutropenia, high levels of rheumatoid factor and autoantibodies have to be suspects of develop SF as an initial presentation of AR or with an AR without diagnosis like on this case.

Cystic rheumatoid arthritis with Felty's syndrome and ankylosing spondylitis.

A 63-year-old man with strictly axial ankylosing spondylitis since the age of 28 years had a seven-year history of cystic seronegative rheumatoid arthritis with Felty's syndrome. Cysts were present in the hands, feet, wrists, shoulders, hips, one elbow, and one knee. There was no evidence of juxtaarticular demineralization, joint space loss, erosions, or joint destruction. Rheumatoid pannus was demonstrated within the cysts, particularly at the hip, ruling out cystic hip disease due to ankylosing spondylitis. HLA typing demonstrated the B27 and DR4 haplotypes. HLA B27 may be associated with a worse prognosis of rheumatoid hip involvement.

Articular disease severity in rheumatoid subjects with and without Felty's syndrome.

Articular disease severity as measured by a radiographic score of hands and feet was compared in rheumatoid subjects with (21) and without (170) Felty's syndrome. Disease duration was a major determinant of joint destruction. When this was taken into consideration radiographic scores were no higher in the Felty's group than in rheumatoid subjects without Felty's syndrome. Immunogenetic associations previously described in Felty's syndrome are unlikely therefore to represent non-specific markers of disease severity and more likely to represent markers of extra-articular disease.

Felty's syndrome without concomitant arthritis: a variant.

A young female patient who had documented seropositive rheumatoid arthritis and was treated consecutively with aspirin, diclofenac, and gold salts was admitted years later for severe neutropenia. On examination she had, in addition, fever, positive rheumatoid factor, reversible swan-neck deformity of the fingers but otherwise normal joint findings. The patient responded to prednisone therapy. This case would appear to be a most unusual variant of Felty's syndrome.

Some extra-articular manifestations of arthritis and complications of therapy. A pictorial essay.

More than 100 years ago Lasegue wrote, "Rheumatic fever licks at the joint but bites at the heart." This truth may be expanded to include nearly every joint ailment on one hand, and nearly every organ system on the other hand. In connective tissue disorders, bleeding diasthesias and metabolic and endocrine disorders, the joint manifestations are only one part of the total picture. This article shows a few examples of cardiopulmonary, gastrointestinal, and other manifestations. A few examples of iatrogenic complications are also described. The list of the examples described in this article is far from complete.

[Incomplete Felty syndrome with manifestation of arthritis following a long-standing course of the disease]. / Inkomplettes Felty-Syndrom mit Manifestation der Arthritis nach jahrelangem Krankheitsverlauf.

A 62-year-old female patient with splenomegaly, severe granulocytopenia, recurrent infections and negative rheumatoid factor test initially had no clinical evidence of rheumatoid arthritis. Leucopenia responded to splenectomy and did not recur during 7 years follow-up. Symmetrical metacarpophalangeal joint-swelling developed 9 months after 7 years of radiographic destruction. This case emphasizes that arthritis may occasionally be a late and minor manifestation of Felty's syndrome.

Radiocolloid scintigraphy in Felty's syndrome.

The Tc-99m sulfur colloid liver-spleen scintigrams of nine patients with Felty's syndrome (a triad of rheumatoid arthritis, splenomegaly, and neutropenia) were reviewed. The characteristic scintigraphic findings include (1) moderate or severe splenomegaly, (2) the reversal of liver-to-spleen uptake ratio despite normal liver function tests, and (3) virtually no visualization of the bone marrow uptake and no pulmonary radiocolloid sequestration. In a late stage of cirrhosis of the liver, moderate-to-severe splenomegaly with the reversal of liver-to-spleen uptake ratio almost always accompanies abnormal liver function, and sometimes there may be associated pulmonary radiocolloid uptake. It was therefore concluded that in a proper clinical setting, a radiocolloid scintigraph may differentiate between Felty's syndrome and cirrhosis of the liver in a late stage.

Labeled cells in the investigation of hematologic disorders.

Radiolabeling techniques for white cells, platelets, and erythrocytes are reviewed. The early studies using diisopropylfluoro-32P contributed to an understanding of the production and circulation of the blood elements, and 51Cr proved useful in localizing sites of cell migration or destruction. 111In-oxine has further improved the understanding of blood cell organ sequestration, and permitted combined kinetic and organ imaging studies. Radionuclide labels have been essential for the elucidation of various hematologic disorders, such as the neutropenias, thrombocytopenias, anemias, and polycythemia. Many new treatments, including monoclonal antibodies, have been evaluated with radionuclides.

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.