Results (Outcome and Follow up):
After the treatment started, her neutrophil count improved. On her follow-up appointments, her CBC and WBC remained normal, and she reported improvement in her fatigue symptoms. She has not had any hospital admissions or life threatening infections, and nonspecific symptoms improved considerably.
Discussion :
Felty syndrome is an extracellular articular manifestation of rheumatoid arthritis. The lifetime risk of developing Felty syndrome (FS) for a patient with rheumatoid arthritis (RA) has been estimated to be approximately 1-3 percent (1). It is primarily diagnosed through clinical evaluation based on the presence of persistent neutropenia and enlarged spleen in chronic RA. It is crucial to emphasize this criterion because diagnosing the condition can be challenging when arthritis symptoms are absent (4,8). Arthritis usually occurs for almost ten or more years before neutropenia is diagnosed (6). Beyond this triad, clinical presentations of Felty’s syndrome can involve anemia, low platelet counts (thrombocytopenia), recurrent bacterial infections, skin ulcers, unexplained portal hypertension, and an elevated risk for the development of blood-related cancers like non-Hodgkin’s and Hodgkin’s lymphoma (3). Though uncommon, documented cases in the literature highlight instances of FS occurring without concurrent articular RA (Table 2). We have presented such a case and briefly reviewed the existing literature.
Table 2 shows a few cases, including ours, in which Felty syndrome was diagnosed before or simultaneously with the onset of arthritis symptoms. (Separate File)
Felty syndrome was found to be more common in females. All the patients presented with neutropenia and splenomegaly, and some of them had arthritis initially as well, as shown in the table.
Pathophysiology of neutropenia in Felty syndrome involves both cellular and humoral immunity. It includes the development of autoantibodies against granulocyte colony-stimulating factor (G-CSF) and polymorphonuclear neutrophils (PMN), resulting in apoptosis of neutrophils and neutropenia (13).
There are no specific criteria for diagnosing felty syndrome. It is a clinical diagnosis of rheumatoid arthritis with splenomegaly and neutropenia. In patients who present without arthritis, positive RF and anti-CCP antibodies can point towards the possibility of rheumatoid arthritis, like in our patient. High-titer RF and anti-CCP have a specificity of 99.5% for RA (15). When the patient tested positive for both factors, she was referred to rheumatology and diagnosed with RA.
Felty syndrome does not have a specific curative treatment. Literature needs guidelines in the management of Felty syndrome, although a few articles have highlighted the different strategies. Treatment primarily focuses on improving neutropenia with the treatment of rheumatoid arthritis, which is the underlying cause (8). Methotrexate and rituximab are the preferred DMARDs in patients with FS (11). Numerous case reports have demonstrated that rituximab can induce a sustained and complete response in autoimmune cytopenias, particularly in cases of Felty’s Syndrome (7, 10, 14). In our case, the patient responded well to the Rituximab treatment. Glucocorticoids can temporarily increase neutrophils as they cause the release of non-segmented neutrophils into the circulation (12). The role of splenectomy is small, although it has been used in refractory cases.
Our patient did not have arthritis; instead, she presented with remarkable fatigue, severe neutropenia in labs, and mild splenomegaly on imaging. Further workup showed high-titer rheumatoid factor and positive anti-CCP antibody. The neutropenia normalized with the use of a high dose of steroids but later relapsed. She was then started on rituximab therapy, which improved her neutrophils. However, the patient needs to be closely monitored for neutrophils and the development of arthritis.
While our case report and brief literature review provide valuable insights into Felty syndrome’s atypical presentation and management, it is essential to acknowledge that these findings may only sometimes apply. This limits our report, as it may not apply to all the cases due to individual differences in presentation and response to treatment.
Felty syndrome is a rare presentation of RA, but persistent neutropenia can lead to life-threatening infections. Therefore, it is necessary to check for RA and early refer to rheumatology even in the absence of arthritis symptoms. Early detection of Felty syndrome is crucial for further management and prevention of life-threatening infections.
Conclusions :
In conclusion, this case highlights the present atypical manifestation of Felty Syndrome, underscoring the challenges in managing this rare condition. The case findings emphasize the importance of considering diverse clinical manifestations of FS. We also emphasize the importance of ruling out other causes of neutropenia and splenomegaly. If left untreated, neutropenia can lead to the development of life-threatening infections. Awareness among healthcare professionals, particularly hematologists, is crucial for timely recognition and appropriate intervention, ensuring optimal care for patients with this complex autoimmune condition.