International Journal of Rheumatic Diseases 2014; 17: 122–124


Felty’s syndrome with hyperthyroidism: a case report and literature review

Dear Editor, A 36-year-old non-smoking, married mother with a 20-year history of rheumatoid arthritis (RA) presented with neutropenia, splenomegaly, hyperthyroidism and scabies, which resulted in an admission to hospital. The patient’s RA symptoms began with pain in her bilateral wrists and proximal interphalangeal joints 20 years ago. With X-ray examination and serum test for C-reactive protein, erythrocyte sedimentation rate (ESR) and rheumatoid factor (RF), RA was diagnosed. Pharmacologic treatment was started with methotrexate 15 mg/week and prednisone 10 mg daily. The patient’s symptoms decreased and functional status improved over time with irregular oral intake of medications. At the time of admission, physical examination revealed pallor and deformities of the bilateral joinst of hands, wrists, elbows and feet. Swan-neck, boutonniere deformity and ulnar deviation of the digits were noted in both hands. Rheumatoid nodules were absent. Her thyroid gland was slightly enlarged without tenderness. The spleen was palpable about 5 cm below the left costal margins. Skin rash composed of small red bumps and blisters were found between the fingers, wrists, back of the elbows, waist and around the umbilicus. The itch was typically worse at night. X-rays of the hands displayed the evidence of cartilage and bone damage and osteoporosis around joints, joint deformity with permanent joint fixation and abnormalities of soft tissue around joints. The thyroid gland measured 50 mm 9 21 mm 9 20 mm in the left lobe and 50 mm 9 20 mm 9 16 mm in the right lobe by typeB ultrasound. Echo-enhancement and no lump in the parenchyma were found in the gland. The spleen was 4.5 cm below the left costal margins via type-B ultrasonic measurement. Microscope test and ink test were performed to identify a typical scabies case. Complete blood cell counting showed white blood cells (WBC) 3.73 9 109/L, neutrophil 1.49 9 109/L, red blood cell 2.81 9 1012/L, hemoglobin 69 g/L, hematocrit 17.5% and platelets 75 9 109/L. A doctor

performed bone marrow biopsy and this demonstrated hyperplasia anemia. A thyroid function test displayed the free thyroxine (free T4) to be 4.15 ng (normal range: 0.89–1.80 ng/dL), free triodothyronine (free T3) to be 3.48 pg/mL (normal range: 2.30–4.20 pg/mL), thyroid stimulating hormone (TSH) < 0.01 IU/mL (normal range: 0.35–5.50 IU/mL) and TSH receptor antibody (TRAb) at 36% (normal: 0–9%). ESR was 140 mm/h, and RF was positive with a titer of 1 : 320. Erythrocyte direct antiglobulin (Coombs) test for autoimmune hemolytic anemia, Ham test for paroxysmal nocturnal hemoglobinuria and purified protein derivative (PPD) skin test were negative. Anti-nuclear antibody (ANA), anti-ds-DNA antibody, and anti-elutable nuclear antigen antibodies were negative. A chest computed tomography (CT) scan was normal. The patient was diagnosed with Felty’s syndrome (FS), Graves disease and scabies. Treatment was started with sulfur ointment, leflunomide 20 mg, propylthiouracil 50 mg and celecoxib (Celebrex) 200 mg daily. After 7 days of therapy, the scabies symptom was significantly improved. The manifestations of RA and Graves disease were partly relieved. However, the enlarged spleen was still palpable about 5 cm below the left costal margins. White blood cells and neutrophils were reduced from 3.6 9 109/L and 1.45 9 109/L, to 2.7 9 109/L and 1.17 9 109/L, respectively 2 weeks after the admission, as showed in Figure 1. Two weeks after the admission, oral 10 mg prednisone daily was added to the patient’s regimen. There was a dramatic increase in the WBC and neutrophil counts, and normal counts were restored within 1 week after the treatment, as displayed in Figure 1. Dramatically, the enlarged spleen contracted to normal size. FS is characterized by the triad of RA, neutropenia and splenomegaly. We present here a rare case of a 36-year-old woman with FS, hyperthyroidism and scabies. In the present case, the patient presented with skin infection in neutropenic settings. Neutropenia and splenomegaly with elevated ESR, elevated levels of

© 2013 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd


Figure 1 White blood cell and absolute neutrophil counts are shown to progressively increase. (♦) neutrophils and (■) white blood cells.

serum C-reactive protein and RF, joint deformities, joint pain, X-ray manifestations in the hands and anemia of chronic disease pointed toward FS. Enlargement in size of the thyroid gland and serum test of thyroid function and specific antibodies indicated the diagnosis of hyperthyroidism.1 Treatment was commenced with disease-modifying anti-rheumatic drugs (DMARDs), including leflunomide and Celebrex for RA, sulfur ointment for scabies and propylthiouracil for hyperthyroidism. The scabies was cured, and the symptoms of RA and hyperthyroidism were largely relieved after 1 week of treatment. White blood cells and neutrophil counts and enlargement of spleen did not respond to the therapy prior to the oral intake of glucocorticoids. Researchers suppose that the first choice for treating both neutropenia and arthritis is methotrexate, which is safe, effective and well tolerated in these patients.2 Many studies suggest that application of rituximab is useful in the treatment of FS, while other researchers have found a different result.3 Controlled trials of different treatment modalities are not available because of the rarity of this syndrome. Splenectomy has produced a long-term hematologic response in 80% of patients but is usually reserved at the end of the treatment algorithm for treatment-resistant cases.4 In some patients with FS, the presence of antibodies against neutrophils has been described, which might be associated with increased neutrophil destruction. The underlying mechanism of developing neutropenia in FS is similar to that in other forms of

International Journal of Rheumatic Diseases 2014; 17: 122–124

immune-mediated neutropenia.5 However, there are no reports of the prevalence of association between hyperthyroidism and FS. Thus, autoimmune or immunologic processes were assumed in the pathogenesis of both autoimmune thyroid diseases (Graves’ disease) and FS. It had become recognized that Th1/Th2 balance controls the immune system. From the viewpoint of imbalance, autoimmune Graves’ disease was considered to be a Th2-type disease.6 Systemic involvement in RA is characterized by B cell overactivity, immune complex formation and complement consumption, suggesting that Th2 cells are involved in the pathogenesis of extraarticular manifestation of FS. Therefore, regarding Th1/ Th2 imbalance, it is not surprising that there is a prevalence of Graves’ disease in FS patients. Leflunomide may exert its effects by inhibiting the mitochondrial enzyme dihydroorotate dehydrogenase, which plays a pivotal role in the synthesis of the pyrimidine ribonucleotide uridine monophosphate (rUMP). Therefore, we propose that leflunomide prevents the expansion of activated and autoimmune lymphocytes by interfering with the cell cycle progression caused by inadequate production of rUMP.7

ACKNOWLEDGEMENT This study was supported by ‘The Incubative Program for Youth Scientists of Jiangxi Province, China’ no. 20112BCB23029.

CONFLICTS OF INTEREST There is no potential conflict of interest in this paper. Tianlun HUANG,1† Mengyuan LIU1,2† and Gaosi XU1 1

Department of Rheumatology, Second Affiliated Hospital, and 2Medical Center of the Graduate School, Nanchang University, Nanchang, China Correspondence: Professor Gaosi Xu, email: [email protected] †These two authors contributed equally to this work.

REFERENCES 1 Cooper DS (2005) Anti-thyroid drugs. N Engl J Med 352, 905–17. 2 Narvaez J, Domingo-Domenech E, G omez-Vaquero C, L opez-Vives L, Estrada P, Aparicio M (2012) Biological agents in the management of Felty’s syndrome: a systematic review. Semin Arthritis Rheum 41, 658–68.



3 Sordet C, Gottenberg JE, Hellmich B, Kieffer P, Mariette X, Sibilia J (2005) Lack of efficacy of rituximab in Felty’s syndrome. Ann Rheum Dis 64, 332–3. 4 Rashba EJ, Rowe JM, Packman CH (1996) Treatment of the neutropenia of Felty syndrome. Blood Rev 10, 177–84. 5 Pixley JS, Patchin GM (1997) Felty’s syndrome. BioDrugs 7, 356–65.


6 Snowden N, Kay RA (1995) Immunology of systemic rheumatoid disease. Br Med Bull 51, 437–8. 7 Jagoda JS, Rajapakse CN (2011) Leflunomide in clinical practice. A retrospective observational study on use of leflunomide in New Zealand. Int J Rheum Dis 14, 340–4.

International Journal of Rheumatic Diseases 2014; 17: 122–124

Copyright of International Journal of Rheumatic Diseases is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.

Copyright of International Journal of Rheumatic Diseases is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.

Felty's syndrome with hyperthyroidism: a case report and literature review.

Felty's syndrome with hyperthyroidism: a case report and literature review. - PDF Download Free
103KB Sizes 0 Downloads 0 Views

Recommend Documents

Takotsubo Myocardiopathy and Hyperthyroidism: A Case Report and Literature Review.
BACKGROUND Takotsubo cardiomyopathy (TM), also called stress myocardiopathy or transient left ventricular apical ballooning syndrome, is characterized by acute left ventricular dysfunction with reversible wall motion abnormalities. TM resembles acute

Mazabraud's syndrome: case report and literature review.
Mazabraud's syndrome is a rare disorder characterized by the association of single or multiple intramuscular myxomas with fibrous dysplasia. Here, we present the first case of Mazabraud's syndrome visualized on 18F-FDG PET/CT with histopathological c

Joubert syndrome in a neonate: case report with literature review.
Joubert syndrome is a rare autosomal recessive disorder. It is characterized by congenital ataxia, hypotonia, developmental delay and at least one of the following features: neonatal respiratory disturbances and abnormal eye movements; including nyst

Hyperthyroidism-associated hypercalcemic crisis: A case report and review of the literature.
Hyperthyroidism is one of the major clinical causes of hypercalcaemia, however, hyperthyroidism-related hypercalcemic crisis is rare, only 1 case have been reported. The potential mechanisms are still not too clear. It may be related that thyroid hor

Role of Cholestyramine in Refractory Hyperthyroidism: A Case Report and Literature Review.
Hyperthyroidism is a common disease that usually responds to the conventional therapy of anti-thyroidal medications (methimazole or PTU) and beta-blocker. Refractory hyperthyroidism is a rare condition in which hyperthyroidism fails to respond to the

Sweet's syndrome with pulmonary involvement: Case report and literature review.
A 74 year old female presented with fever, associated with papules and plaque in her upper and lower extremities. Exams revealed blood leukocytosis and a positive urine culture. Antibiotic therapy was initiated with no clinical response. After 1 week

Morbihan syndrome: a case report and literature review.
Morbihan syndrome is a rare entity that more commonly affects women in the third or fourth decade of life. It is considered a special form of rosacea and its pathogenesis is not fully known. It is clinically characterized by the slow appearance of er

Atypical hemolytic-uremic syndrome: a case report and literature review.
Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by hemolysis, thrombocytopenia, and renal dysfunction. It is a disease related to genetic mutations in the alternative complement pathway and has a distinct pathophysiology but

De Sanctis-Cacchione syndrome: A case report and literature review.
De Sanctis-Cacchione (DSC) syndrome is one of the rarest, most severe forms of xeroderma pigmentosum (XP). These patients with XP are of short stature, have mental disabilities, and develop progressive neurologic degeneration because of a severe inab

Fraser Syndrome - a Case Report and Review of Literature.
Fraser syndrome is a rare autosomal recessive genetic disorder characterized by major features such as cryptophthalmos, syndactyly, malformations of the larynx and genitourinary tract, craniofacial dysmorphism, orofacial clefting, mental retardation