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Felty's syndrome (FS) is characterized by the three conditions of rheumatoid arthritis (RA), neutropenia and splenomegaly, and occurs in few cases of longstanding erosive RA. Discriminating between rare occurrences of autoimmune diseases and malignancies is crucial. The present study describes the case of a 17-year-old female with a two-year histor...
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... antibodies were negative. Levels of complement C3 and C4 were normal. Rheumatoid factor, antibodies to cyclic citrullinated peptides, anti-keratin antibodies and anti-perinuclear factor autoantibodies were negative. X-rays of the joints, including the wrists and hands, showed soft-tissue swelling, bone erosion and narrowing of the joint cavity (Fig. 1). Splenomegaly (54x124 mm) with uniform density and multiple enlarged lymph nodes distributed along the retroperitoneal space, omental bursa, mesentery root and surrounding hepatic hilar was shown by abdominal computed tomography (CT) (Fig. 2). A lymph node biopsy from the right cervical node revealed chronic inflammation, with posi- ...
Citations
... Some studies have suggested that the initial treatment for FS should involve low-dose MTX [5,26,27]. Other DMARDs, including leflunomide, HCQ, CsA, sulfasalazine, azathioprine, and cyclophosphamide, have also been used to treat FS [4,[28][29][30]. ...
A triad of symptoms characterises Felty's syndrome: seropositive rheumatoid arthritis (RA), splenomegaly and neutropenia. The treatment of Felty's syndrome is based on using classic synthetic and biological disease-modifying drugs (DMARDs). In this article, we present a case of a patient with Felty's syndrome who was treated with biologic treatment. A systematic search of the literature on the electronic medical database was conducted. The drugs from the DMARD group, despite reducing the activity of the disease, may cause significant clinical complications. It is important to know about the diagnosis, differentiation and treatment of neutropenia and the prevention of febrile neutropenia. The article discusses the current therapeutic possibilities using both classical and biologic DMARDs.
... Beberapa faktor risiko TINJAUAN PUSTAKA dikaitkan dengan kejadian sindrom Felty, termasuk autoimunitas. 5 Faktor reumatoid (FR) dan anti-cyclic citrullinated peptide (anti-CCP) positif akan meningkatkan risiko perkembangan manifestasi ekstraartikular dari artritis reumatoid. 3 Sindrom Felty dapat saja ditemukan pada kasus artritis reumatoid ringan, tetapi sangat erat kaitannya dengan titer FR tinggi dan adanya sinovitis erosif yang agresif. ...
... Selain itu, pemeriksaan ultrasonografi dan CT scan juga dapat dilakukan untuk mendeteksi splenomegali. 3,5 Modalitas yang sama dapat digunakan untuk menilai respons pasien terhadap terapi. ...
... Namun, laporan klinis terkait penggunaan agen-agen tersebut dalam penatalaksanaan sindrom Felty masih sangat terbatas. 2,3,[5][6][7] Cyclophosphamide dosis tinggi dilaporkan memberikan respons pada beberapa kasus sindrom Felty refrakter. Namun, para klinisi menemukan cyclophosphamide lebih merespons baik saat digunakan pada vaskulitis reumatoid dan manifestasi ekstraartikular artritis reumatoid serius lainnya daripada untuk sindrom Felty. ...
Sindrom Felty merupakan kondisi medis dengan karakteristik trias yaitu artritis reumatoid, neutropenia, dan splenomegali; terjadi pada beberapa kasus artritis reumatoid erosif yang sudah berlangsung lama. Hanya 1 – 3% pasien artritis reumatoid akan berkembang menjadi Sindrom Felty. Genetik (HLA-DR4) dan faktor lingkungan berperan dalam terjadinya kondisi ini. Neutropenia persisten dengan hitung neutrofil absolut umumnya kurang dari 1500/mm3 merupakan ciri khas diagnosis Sindrom Felty. Kondisi medis ini biasanya asimtomatik, infeksi lokal serius atau sistemik bisa menjadi petunjuk awal. Terapi farmakologi menggunakan disease-modifying anti-rheumatic drugs (DMARDs); methotrexate oral dosis rendah menjadi modalitas terapi lini pertama. Splenektomi merupakan upaya terakhir dalam algoritma penatalaksanaan Sindrom Felty. Felty Syndrome is a medical condition characterized by triad of rheumatoid arthritis, neutropenia, and splenomegaly; occurs in few cases of longstanding erosive rheumatoid arthritis. Only 1 – 3% rheumatoid arthritis patient developed Felty Syndrome. Genetic (HLA-DR4) and environmental factors are involved in its pathophysiology. Persistent neutropenia with absolute neutrophil count less than 1500/mm3 is a diagnosis hallmark. Felty syndrome may be asymptomatic, but local serious or systemic infections may be the first clue to the diagnosis. Pharmacological therapy as the first-line therapy use disease-modifying anti-rheumatic drugs (DMARDs) such as oral low dose methotrexate. Surgical approach (splenectomy) is the last resort in Felty Syndrome management.
... Described in 1924 by American physician Augustus Felty, "Chauffard-Still-Felty syndrome, " or simply Felty syndrome (FS), refers to a severe form of rheumatoid arthritis (RA) that often presents clinically with splenomegaly and neutropenia [1,2]. This triad of features do not have to be complete in all patients, but neutropenia, defined as absolute neutrophil count of less than 1500/mm 3 , is a hallmark feature and must be present for diagnosis of Felty [1,3]. ...
... The exact cause is unknown, but it is found to have higher incidence in Caucasian populations as compared to African American [4]. Felty is also often seen in patients having chronic active RA for 10 or more years, positive for rheumatoid factor (RF), and positive for HLA-DR4 [1,2,[5][6][7]. In this report, we present an atypical case of early-onset Felty Open Access *Correspondence: agupta2@student.touro.edu 1 Touro College of Osteopathic Medicine, 60 Prospect Avenue, Middletown, NY 10940, USA Full list of author information is available at the end of the article syndrome with underlying RA. Informed consent was obtained from the patient's mother, her health proxy. ...
... Rheumatoid arthritis is a systemic autoimmune inflammatory disease characterized by synovitis with pannus formation and joint destruction [2]. RA often presents with extraarticular features such as interstitial lung disease, sjogren's syndrome, pericarditis, osteopenia, vasculitis, episcleritis, and rheumatoid nodules [2]. ...
Background
Felty syndrome is a rare manifestation of chronic rheumatoid arthritis in which patients develop extraarticular features of hepatosplenomegaly and neutropenia. The typical presentation of Felty syndrome is in Caucasians, females, and patients with long-standing rheumatoid arthritis of 10 or more years. This case report presents a patient with an early-onset and atypical demographic for Felty syndrome.
Case presentation
Our patient is a 28-year-old African American woman with past medical history of rheumatoid arthritis diagnosed in 2017, asthma, pneumonia, anemia, and mild intellectual disability who was admitted to inpatient care with fever, chills, and right ear pain for 7 days. The patient’s mother, also her caregiver, brought the patient to the hospital after symptoms of fever and ear pain failed to improve. Our patient was diagnosed with sepsis secondary to pneumonia and urinary tract infection. She had been admitted twice in the past year, both times with a diagnosis of pneumonia. During this visit in September 2019, it was discovered that the patient had leukopenia and neutropenia. Bone marrow biopsy revealed increased immature mononuclear cells with left shift and rare mature neutrophils. During the hospital course, the patient was provisionally diagnosed with Felty syndrome and treated with adalimumab and hydroxychloroquine for her rheumatoid arthritis. Her sepsis secondary to pneumonia and urinary tract infection was treated with ceftriaxone and doxycycline, which was later switched to cefepime because of positive blood and urine cultures for Pseudomonas aeruginosa. She was discharged with stable vital signs and is continuing to control her rheumatoid arthritis with adalimumab.
Conclusion
This case report details the clinical course of sepsis secondary to pneumonia and urinary tract infection in the setting of Felty syndrome. Our patient does not fit the conventional profile for presentation given her race, age, and the length of time following diagnosis of rheumatoid arthritis.
... Some studies suggested that the initial treatment for FS should be low dose Methotrexate [1,4]. Other disease-improving antirheumatic drugs including leflunomide, hydroxychloroquine, cyclosporin A, sulfasalazine, azathioprine, and cyclophosphamide have also been used to treat FS [5][6][7][8]. In terms of biological agents like rituximab, although a few reports are ineffective for FS [9], most of reports are effective, especially for improving the blood system. ...
... The value of splenectomy for FS patients is still under debate. In fact, it may have to be reserved for the most serious cases, which have been proved to be drug resistant [5,6]. Treatment with TNF-α antagonists has been found to increase the levels of antinuclear antibodies and anti-dsDNA antibodies in RA patients while most FS patients already have a high titer of anti-nuclear antibodies, which limits their application [18]. ...
Felty’s syndrome (FS) is a deforming disease, characterized by the triad of rheumatoid arthritis (RA), neutropenia, and splenomegaly. Currently, FS patients are treated mainly with immunosuppressants, such as methotrexate and glucocorticoids, which however are not suitable to some patients and may cause severe side effects. Here we report a clinical FS case that was treated with Tocilizumab (TCZ) successfully. The patient had symmetrical swelling and pain of multiple joints, deformity of elbow joints with obvious morning stiffness. Joint color Doppler ultrasound showed synovial hyperplasia and bone erosion of wrist and proximal interphalangeal joints and CT scan suggested splenomegaly. Further examination showed neutropenia and anemia, a high titer of anti-cyclic citrullinated peptide antibody, rheumatoid factor and anti-nuclear antibodies, positive p-ANCA, and elevated IgA and IgG. After treating with TCZ, the patient has been relieved of clinical symptoms. His spleen has recovered to normal size. The absolute neutrophil count (ANC) tended to be stable, and joint erosion did not deteriorate. We have reviewed the literatures on FS treatment with biological agents and found only a few reports using TNF-α antagonist and rituximab treating FS, but none with TCZ. So, it is the first time to report a successful FS case treated with TCZ. This case suggests that the TCZ may be a new choice for FS treatment, under the condition of closely monitoring the ANC.
... The complete triad is not an absolute requirement, but persistent neutropenia with an absolute neutrophil count (ANC) less than 1500-2000/mm 3 is generally regarded as necessary for establishing the diagnosis in adult [3]. FS affects 1-3% of patients with RA, but it is rarely seen in patients with juvenile idiopathic arthritis (JIA), with only a few cases having been reported throughout the world [4][5][6][7][8]. The active extra-joint clinical features can be misleading in FS, and certain pediatrician focus on severe extra-articular disease and neutropenia, which suggests infectious diseases. ...
... A few case studies for children with FS have been published in English, but no Chinese studies were found. We performed a literature review and identified 5 children with FS [4][5][6][7][8]. Table 2 shows the major features of these cases. ...
... Current data show that 1-3% of RA patients are complicated with FS, with an estimated prevalence of 10 per 100,000 populations [12]. FS is rarely seen in patients with JIA, with only five cases having been reported throughout the world [4][5][6][7][8]. Table 2 provides a comparison of these five patients with our patient (patient 6). ...
Background:
Felty's syndrome (FS) is characterized by the triad of rheumatoid arthritis (RA), splenomegaly and neutropenia. The arthritis is typically severe and virtually always associated with high-titer rheumatoid factor. The presence of persistent neutropenia is generally required to make the diagnosis. Most patients diagnosed with FS are aged 50-70 years and have had RA for more than 10 years. It is rarely seen in patients with juvenile idiopathic arthritis (JIA), with only five cases having been reported throughout the world.
Case presentation:
The present study describes the case of a 14-year-old female with a seven-year history of polyarticular JIA, presenting with splenomegaly, hepatomegaly, cholestasis and thrombocytopenia. However, she occasionally developed neutropenia. Titers of rheumatoid factor and anti-CCP were persistently high, and the antinuclear antibody titer was 1:320, while the antibody results for anti-dsDNA and anti-Sm were negative. Serum levels of IgA, IgG, IgM and IgE were all persistently elevated, and the ratio of CD19+ lymphocytes in the subgroups of lymphocytes was persistently high. The level of complements was normal. No STAT3 and STAT5B mutations were found by next-generation sequencing. The patient did not respond to methotrexate, prednisolone, hydroxychloroquine (HCQ), sulfasalazine and etanercept but was responsive to rituximab.
Conclusions:
JIA, thrombocytopenia and splenomegaly are the most common and important features in six children with FS, while persistent neutropenia is not seen in all these patients. No complement deficiency has been found in children with FS so far. Manifestations of FS without neutropenia may be extremely rare. There are differences between adults and children in the clinical and laboratory features of FS.
... In conclusion, this case suggests that ABT might be suitable for combination use together with MTX and RTX in the treatment of FS, particularly in cases with severe neutropenia. Furthermore, there is a link between large granular T cell lymphoma and FS, and an expansion of large granular lymphocytes are revealed in 3040% of FS patients [8]. Therefore, ABT might be a novel second-line therapy for FS. ...
... Felty's syndrome (FS), characterized by the triad of seropositive rheumatoid arthritis (RA), neutropenia and splenomegaly was first described in 1924 [1,2]. Less than 1% of RA patients develop FS and usually after more than 10 years of disease progression [3], prognosis is poor, increased mortality is related to the higher incidence of severe infection, with up to 36% 5-year mortality [4]. ...
... , with no history of recurrent infections. One month prior to her admission a lower neutrophil count (<500/mm 3 ) is detected in a routine check-up, she remained asymptomatic and the physical examination was normal. She was referred to the hematologist who performed a myelogram that showed neutrophils maturation arrest, polymorphonuclear decrease, no evidence of significant myelodysplasia and no cytological evidence of pathological infiltrates ( Figure 1). ...
... The patient then presented to the Emergency Department with a 3 day history of fever (38.5ºC), with no other symptoms and no alterations in the physical examination suggestive of an infectious focus. The initial laboratory evaluation revealed leucopenia (2610/mm3), neutropenia (440/mm 3 ) and thrombocytopenia (40000/mm 3 ), a slight increase in C-reactive protein (CRP) 2,81mg/dL. Urinalysis was normal, as where chest and abdominal X-rays. ...
Felty's syndrome (rheumatoid arthritis with neutropenia and splenomegaly) is a rare condition with poor long-term prognosis, mainly as a result of severe infection risk. An effective treatment strategy has not been developed so far and current treatment options are based upon case reports, small series and clinical experience since no randomized clinical trials are available. The authors describe the case of a 53-year-old female patient with a 14-year history of rheumatoid arthritis presenting with fever, neutropenia and splenomegaly. Broad-spectrum antibiotics and granulocyte colony-stimulating factor were administered with good clinical outcome and low dose methotrexate for disease control was successfully initiated after discharge. We would like to highlight the importance of being aware of this syndrome in the differential diagnosis of long term rheumatoid arthritis patients presenting with febrile neutropenia.
Background
Felty syndrome is defined by three conditions: neutropenia, rheumatoid arthritis, and splenomegaly. Neutropenia associated with pancytopenia may further affect the dental condition of a patient. Periodontal treatment and surgery in patients with Felty syndrome necessitates cooperation with a hematologist. Here we present a case of a patient with Felty syndrome who was initially referred to the oral surgery hospital attached to the School of Dentistry for extensive periodontitis. She was effectively treated in collaboration with the hematology department.
Case presentation
A 55-year-old Asian woman visited our department with concerns of worsening tooth mobility, discomfort, and spontaneous gingival bleeding. Initial periodontal examination revealed generalized severe periodontitis (Stage IV Grade C) resulting from leukopenia/neutropenia and poor oral hygiene. A thorough treatment strategy involving comprehensive dental procedures, such as multiple extractions and extensive prosthetic treatment, was implemented. Following the diagnosis of Felty syndrome, the patient was started on treatment with oral prednisolone 40 mg/day, which effectively controlled the disease. Furthermore, there was no recurrence of severe periodontitis after the periodontal treatment.
Conclusions
Dentists and physicians should be aware that immunocompromised individuals with pancytopenia and poor oral hygiene are at risk of developing extensive periodontitis. If their susceptibility to infection and pancytopenia-related bleeding can be managed, such patients can still receive comprehensive dental treatment, including teeth extractions and periodontal therapy. Cooperation among the dentist, hematologist, and patient is necessary to improve treatment outcomes and the patient’s quality of life.
Primary cardiac B cell lymphoma is rare. To date, fewer than 90 cases have been described in the literature. We report a 67-year-old woman with a 30-year history of rheumatoid arthritis, who had received treatment with leflunomide for 10 years and infliximab for 2 years. Secondary Felty's syndrome appeared. She was admitted to the hospital for abdominal pain. Investigations disclosed a 5 cm cardiac mass in the right atrium. Histopathologic examination of tissue specimens obtained at surgical myocardial biopsy demonstrated primary cardiac B cell lymphoma. The other iatrogenic lymphoproliferative disorders are reviewed. This lesion might be a manifestation of long term TNFα antagonists treatment.
Aim:
The aim of this study was to investigate the relationship between hematological markers and disease activity in patients with rheumatoid arthritis (RA).
Method:
The study was designed and performed in the Department of Rheumatology of the Sakarya University Faculty of Medicine. In total, 102 patients with RA were retrospectively enrolled. We used the Disease Activity Score of 28 joints (DAS28) instrument to evaluate disease activity. Laboratory assessments included complete blood cell counts, measurement of erythrocyte sedimentation rate (ESR) and assessment of C-reactive protein (CRP) level. Exclusion criteria included active infection and/or the presence of any hematological, cardiovascular or metabolic disorder.
Results:
We found that the neutrophil lymphocyte ratio (NLR) and mean platelet volume (MPV) varied by disease activity status. NLR values correlated positively with the DAS28 scores of RA patients. Especially, higher NLR values (3.92 ± 0.31) were evident in the group exhibiting high-level disease activity, whereas the MPV values were lowest (7.11 ± 0.91 fL) in this group. Additionally, no significant difference was evident between DAS28 scores and platelet distribution width (PDW) values in patients with RA (r = -0.055, P = 0.124).
Conclusions:
We found that the MPV value may serve as a marker of the absence of acute-phase disease, and the NLR level as a marker of the presence of such disease, in patients with RA. More detailed analysis of disease activity is required to further explain the associations of the markers described above with disease activity.
