Autologous Stem Cell Transplantation in the Treatment of Refractory Rheumatoid Arthritis

The concept of using high-dose immunosuppressive treatment (HDIT) with autologous stem cell transplantation (ASCT) to treat patients with refractory rheumatoid arthritis has been provided by animal studies and anecdotal case reports. Over the past five years, an increasing number of patients with refractory rheumatoid arthritis have received HDIT with ASCT as an adjunct to intense immunosuppression. Here, we present a case of refractory rheumatoid arthritis in a 54-yr-old woman using HDIT with ASCT. Peripheral blood stem cells were mobilized with cyclophosphamide (4 g/m(2)) followed by G-CSF (5microgram/kg/day). Leukapheresis continued daily until the number of harvested progenitor cells reached 2 x 10(6) CD34+ cells/kg after CliniMax(R) CD34+ positive selection. For HDIT, high-dose cyclophosphamide (total dose 200 mg/kg) and antithymocyte globulin (total dose 90 mg/kg) were administered and CD34+ cells were infused 24 hr after HDIT. The patient tolerated the treatment well but experienced an episode of neutropenic fever. She achieved an early dramatic improvement of joint symptoms during therapy. Fifty percent of improvement of rheumatoid arthritis by the American College of Rheumatology (ACR 50) preliminary definition was fulfilled during the 6 months following ASCT. Although further long-term follow-up is required, the patient's activity of arthritis has been stable since receiving HDIT with ASCT.

A case of Poncet's disease with tuberculosis of the intestine and lymph nodes / 대한내과학회지

In 1897, Poncet described polyarthritis in patients suffering from tuberculosis which was not caused by tuberculosis infection of the joints. Poncet's disease is characterized by a polyarthritis that occurs during the acute tuberculosis infection in which no mycobacterial involvement can be found or other known cause of polyarthritis detected. It is a different entity from tuberculous arthritis, which is usually monoarticular and is caused by direct tuberculous infection. It was little known for pathogenesis of Poncet's disease, but immunologic reaction was suggested. Poncet's disease is associated with an excellent prognosis with rapid resolution on commencing therapy and no sequelae. Therefore, recognition of Poncet's disease can be important. Poncet's disease remains a diagnosis of exclusion.Recently, we experienced one case of Poncet's disease which have characterized by the clinical manifestations, and report this case with a review of the literatures.

One case of Giardia lamblia-associated arthritis in a 23 year-old woman / 대한내과학회지

Giardia lamblia, a gastrointestinal protozoan, is one of the most common disease-causing parasites in the world. Giardiasis is primarily encountered in areas with poor sanitation, but it is also seen in more developed countries. A possible sequela of Giardia infections of the bowel is reactive arthritis or synovitis. Few reports of synovitis secondary to giardiasis exist in the literature. Arthropathy secondary to giardiasis is uncommon, but may be underdiagnosed. We present a 23 year-old woman who had polyarthritis after Giardia lamblia infestation. The synovitis subsided with treatment of the giardiasis with metronidazole. The diagnosis of Giardia synovitis should be suspected by the presence of Giardia cysts in the stool. Although uncommon, giardiasis can cause severe synovitis that may be confused with a septic joint.

A Case of Protein-Losing Enteropathy Associated with Systemic Lupus Erythematosus / 대한류마티스학회지

Systemic lupus erythematosus (SLE) is a multisystemic disease that can affect most organ system, although gastrointestinal (GI) manifestations are relatively uncommon. Protein losing enteropathy (PLE) is associated with several clinical disorders, but it is an unusual manifestation of SLE. Of over 20 reported cases of PLE associated with SLE, the pathogenesis of lupus associated PLE remains unclear. We describe a patient with edema, diarrhea, abdomianl pain, and hypoalbuminemia who had been diagnosed SLE. PLE was diagnosed by the method of alpha1-antitrypsin clearance in stool.

Activation of Stat Protein in Synovial Cell in Rheumatoid Arthritis / 대한류마티스학회지

OBJECTIVE: To characterize Stat activity in synovial tissue in rheumatoid arthritis (RA) in order to see if Stat molecule contributes to the pathogenesis of RA by regulatory expression of genes that play an important role in inflammation and tissue destruction. METHODS: Synovial tissue were obtained immediately after operative excision. Immuno-histochemistry was done with the antibodies for Stat 3 and Stat 5. Cells were stimulated with interleukin 6 (IL-6) and soluble interleukin 6 receptor (sIL-6R) or steroid using chambered slide. In supershift experiment, cell extracts were incubated with 0.5ng of 32P-labelled double-stranded oligonucleotide probe. Samples were resolved on 4.5% polyacrylamide gels, which was transferred to polyvinylidene fluoride membranes. Anti-phosphotyrosine Stat 3 antibody was used for Western blotting. RESULTS: Stat 3 was not shown on the synovial tissue section done by immuno-histochemistry. However, activated Stat 3 was expressed on cultured synovial cell stimulated with IL-6 and sIL-6R, and also with IL-6 and dexamethasone using chambered slide. In contrast to Stat 3, activated Stat 5 was expressed on the synovial tissue section, especially around blood vessel. CONCLUSION: Stat is activated in cultured synovial cells as shown in other immune associated cells, and IL-6 is the strong activator of Stat 3. Further analysis of the regulation of Stats in synovitis and the role of Stats in driving synovial inflammation will yield insight into the pathogenesis of RA and the development of novel therapeutic modality.

Secondary renal amyloidosis in adult onset Still's disease: case report and review of the literature

We report a 25-year-old Korean woman with Adult onset Still's disease (AOSD) presented with renal amyloidosis, which had developed four years after disease onset. We successfully treated her with prednisolone, colchicine and cyclophosphamide. A review of the literature uncovered about 10 cases, most of which were treated by various regimens that resulted in poor outcomes. Renal amyloidosis should be suspected in patients with AOSD who have unexplained proteinuria. Although the mechanism of renal amyloid deposition is not well known, earlier histopathologic diagnosis and choice of regimen may affect prognosis.

Injectable gold-induced hepatitis and neutropenia in rheumatoid arthritis

Gold salts have been used for many years in the treatment of rheumatoid arthritis. The common side effects are mucocutaneous reactions, but hepatotoxic reaction and isolated neutropenia are rare complications. We report a 62-year-old woman with rheumatoid arthritis who had developed hepatitis and neutropenia simultaneously after receiving 137.5 mg of sodium aurothiomalate.

A case of parasymphyseal and associated insufficiency fractures of pubic rami in a patient with mixed connective tissue disease

Parasymphyseal insufficiency fractures are uncommon. Furthermore, none have been reported in systemic rheumatic diseases other than rheumatoid arthritis. In this article we report on parasymphyseal insufficiency fractures in a patient with mixed connective tissue disease.

Two cases of pseudorheumatoid hand deformities in parkinson's disease / 대한내과학회지

Parkinson's disease is a chronic, progressive disorder characterized by progressive muscular rigidity, pill-rolling tremor, stooping posture, shuffling gait, and akinesia. Hand deformities associated with Parkinson's disease were first described in 1864 by Ordenstein. The hand deformity of Parkinsons disease consists of metacarpophalangeal joint flexion and interphalangeal joint extension. In clinical practice, the problem is often overlooked or misdiagnosed as rheumatoid arthritis. We experienced 2 cases of pseudorheumatoid hand deformities secondary to Parkinsons disease, and report these cases with a review of the literatures.

A Case of Successful Treatment with High Dose Intravenous Immunoglobulin in Systemic Lupus Erythematosus Patient with Salmonella Group D Bacteremia and Myelofibrosis / 대한류마티스학회지

A 22-year-old woman presented with fever and pancytopenia. One year ago, she was diagnosed as Salmonella group D bacteremia and myelofibrosis associated with SLE at another hospital. She was placed on high dose steroid, however, there was no improvement. Two months ago, she was diagnosed as recurrent Salmonella group D bacteremia. After admission to our hospital, she was placed on intravenous antibiotics and high dose intravenous immunoglobulin. A significant improvement in laboratory and clinical condition occurred and bone marrow biopsy showed complete resolution of fibrosis. We report a case of SLE with myelofibrosis and recurrent Salmonella group D bacteremia.

A Case of Microscopic Polyangiitis with Peripheral Neuropathy / 대한류마티스학회지

Microscopic polyangiitis (MPA) is a systemic necrotizing vasculitis of small-sized vessels (capillary, venule, or arterioles) whose clinical manifestations are very similar to those of PAN (polyarteritis nodosa), but it is characterized by the presence of rapidly progressive glomerulonephritis (RPGN), which is nearly constant, and pulmonary involvement usually absent in PAN. Peripheral neuropathy occurs in only 14~36% of the patients with MPA, distinctly less frequent than in those with classic PAN. While PAN may be considered a self-limiting disease which tend not to recur once remission is acquired, relapse occurs frequently in MPA. Prognosis has been transformed by corticosteroids and immunosuppressive drugs, especially cyclophosphamide. We reported a 72-year-old woman with subacute sensorimotor polyneuropathy involving peroneal and sural nerve accompanied by focal segmental glomerulonephritis which was proven by renal biopsy.

A Case of Myelopathy due to the Nodular Ossification of the Ligamentum Flavum of Thoracic Spine in Diffuse Idiopathic Skeletal Hyperostosis / 대한류마티스학회지

Diffuse idiopathic skeletal hyperostosis (DISH), also known as Forestier? disease, is an idiopathic rheumatological abnormality in which exuberant ossification occures along ligaments throughout the body, most notably the anterior longitudinal ligament of spine, frequently posterior longitudinal ligament or ligamentum flavum. Ossification of ligamentum flavum (OLF) causes narrowing of spinal canal and cord compression. It has been described almost exclusively in the Japanese, extremely rare in Caucasian and Korean population. The predominant location of symptomatic OLF is at the thoracolumbar junction, particularly at T10-11 followed by T11-12, and rarely in the cervical region. We experienced a case of progressive myelopathy due to the nodular ossification of ligamentum flavum in patient with DISH, who was treated by posterior decompressive laminectomy, removal of the ossified ligamentum flavum. The neurologic status of the patient was improved dramatically after operation.

Two Cases of Allergic Reactions to Mesna which Imitate Malar Rash / 대한류마티스학회지

Hemorrhagic cystitis is potentially life-threatening sequellae of chemotherapy using oxazaphosphorine alkylating agents (cyclophosphamide and ifosfamide). Mesna contains a sulfhydryl group that is believed to bind acrolein within the urinary collecting system and reduce the hemorrhagic cystitis without affecting the chemotherapeutic potential. To date, about thirty cases of hypersensitivity or allergic reactions of the delayed and urticarial type associated with mesna have been reported. We reported two patients with systemic lupus erythematosus who developed facial rash and flushing associated with mesna which imitate malar rash.

A Case of Inflammatory Arthritis in the Myelodysplastic Syndrome / 대한류마티스학회지

The myelodysplastic syndrome (MDS) is a heterogeneous group of refractory anemia resulting from a clonal abnormality in the pleuripotent stem cell. A variety of immunologic abnormalities have been reported in patients with MDS, and various rheumatic diseases, including rheumatoid arthritis, lupus, Sjogren? syndrome, vasculitis, have been reported in association with MDS. Inflammatory arthritis is a rare complication of MDS. Here we report a patient with refractory anemia with excess blasts with unusual manifestation of inflammatory arthritis, with a review of the literature.

A Case of Abdominal Aortic Aneurysm Treated with Immunosuppressants in Behcet's Disease / 대한류마티스학회지

Behcet's disease can elicit serious vascular complications such as thrombophlebitis, arterial occlusion, and arterial aneurysm. The most common cause of death in Behcet's disease is secondary to vascular complication. The treatment for abdominal aortic aneurysm which was frequently complicated by fatal rupture (60%) is mainly a surgical management. If surgical treatment is not possible due to various causes, alternative medical therapy such as immunosuppressives and steroid would be recommended. We report here the case of medically controlled abdominal aortic aneurysm in a 47-year-old patient with Behcet's disease who refused to undergo surgical treatment. Immunosuppressive therapy and steroid could be an alternative treatment of aneurysmal manifestations in Behcet's disease.

The Characteristics of Elderly Onset Rheumatoid Arthritis / 대한류마티스학회지

OBJECTIVES: Patients who develop rheumatoid arthritis over 60 years old(elderly-onset rheumatoid arthritis, EORA) are different from those of younger-onset disease(YORA). Several aspects are emphasized; more equal sex distribution, more often systemic complaints, more larger joints involvement, high ESR rates, and less rheumatoid factor positivity. We compared the clinical characteristics of EORA with those of YORA. METHODS: Five hundred and fifty-six patients who had met the ACR criteria for rheumatoid arthritis were studied. We compared the difference with clinical manifestation, disease activity markers, serologic markers including rheumatoid factor and anti-perinuclear factor, and radiologic changes between EORA and YORA. RESULTS: EORA : YORA ratio is 49 : 507, no difference between male and female ratio. The age at onset of EORA is 67.3 +/- .89 years old, YORA is 4.9 +/-10.5 years old, disease duration is not different in both groups. The ESR of EORA (47.65 +/-13.8) is higher than that of YORA ( 39.49+/-.1 ), the positivity of antiperinuclear factor is significantly increased in the EORA group as compared with YORA group. The comparisons of disease activity including Ritchie index; early RA in both groups showed significant difference only in rheumatoid factor titer. CONCLUSION: The EORA is similar to YORA except high ESR, high rheumatoid factor titer, and high positivity of antiperinuclear factor. However, we failed to demonstrate that the EORA runs different course with heterogeneous pathogenesis to the YORA.

Is Juvenile Onset Ankylosing Spondylitis Different from Adult Onset Ankylosing Spondylitis? / 대한류마티스학회지

OBJECTIVE: Ankylosing spondylitis is a well known form of spondyloarthropathy. It is recognized that age at onset is factor that may influence both the clinical presentation and course of disease. The pattern of symptoms in juvenile onset ankylosing spondylitis (JAS) differs significantly from that of adult onset (AS). METHODS: We investigated retrospectively the clinical characteristics of Korean JAS and AS. We reviewed two hundred and forty-two patients who had met the Modified New York criteria for AS. RESULTS: The JAS:AS ratio in the total AS patients was 88:154. The age of onset was 12.9+/-.67 (mean+/-D) in JAS and 22.6+/-.35 in AS. The sex ratio (M:F 81:7 in JAS, M:F 127:27 in AS) and disease duration (9.7+/-.7 in JAS, 801+/-.8 in AS) were similar in both groups. The most common site of initial symptom was knee in both groups, more frequent in JAS than in AS (JAS: 38%, AS: 25%, p<0.05). The peripheral arthritis was more frequent than axial symptom in initial symptom of both groups (JAS: 84%, AS:65%, p<0.01). There were significant differences in knee arthritis (JAS: 83%, AS:67%, P<0.001), ankle arthritis (JAS: 63%, AS: 39%, p<0.001), and tarsus involvement (JAS: 32%, AS: 19% P<0.05). The peripheral arthritis was found in 79%, more frequent in JAS than in AS (JAS: 90%, AS: 73%, p<0.001),. The arthritis in lower extremities was more frequent in JAS than in AS (JAS: 90%, AS: 72%, p<0.001). There were no differences in laboratory findings, spine involvement, and extraarticular symptoms including renal involvement, uveitis, and enthesitis (JAS: 86%, AS: 81%) between both groups. CONCLUSIONS: Contrary to foreign reports, the peripheral arthritis was more common, sex ratio of male versus female was higher among JAS in Korea. The peripheral arthritis in JAS is more frequent than in AS, and main symptoms in JAS was found more common in peripheral than in axial joints. There were significant differences on initial symptom site and peripheral arthritis of lower extremity, especially knee, ankle and tarsus in both group.

Two Cases of Antisynthetase Syndrome Combined with Mechanic's Hand / 대한류마티스학회지

The antisynthetase syndrome is characterized by inflammatory myositis associated with intersititial lung disease (ILD), polyarthritis, mechanic's hand, and Raynaud's phenomenon, and usually with fever. The patients with these conditions have autoantibodies to aminoacy1-tRNA synthetases (histidy1-, threony1-, alany1-, isoleucy1-, and glycy1-tRNA synthetase) ; the most common is anti-histidy1-tRNA sythetase (anti Jo-1), present in 20% of myositis patients in Western countries. However, the mechanisms of production of autoantibodies to aminoacy1-tRNA synthetases are not understood. Several hypotheses about the relationship of anti-aminoacy1-tRNA synthetases with inflammatory myositis have been reported. The poor prognostic outcome for the antisynthetase patients are related to ILD. We experienced two cases of antisynthetase syndrome which have characterized by the clinical manifestations, and report these cases with a review of the literatures.

Clinical and serologic characteristics of mixed connective tissue disease in korea / 대한내과학회지

The concept that mixed connective tissue disease (MCTD) is a clinically distinct disease entity has been the subject of debate. Some authors regard it as a transitional phase in the development of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), or polymyositis/ dermatomyositis (PM-DM). This study set out to analyze the clinical and serologic characteristics of MCTD in Korean patients and to determine whether the diagnosis of MCTD held up on follow-up. METHODS: Forty-five patients diagnosed as MCTD using the diagnostic criteria of Alarcon-Segovia between October 1989 and February 1998 at Hanyang University Hospital were retrospectively reviewed. RESULTS: All but two patients were female. The average age at diagnosis was 39.3 years and the average diaseae duration was 7.5 years. The most frequent clinical symptoms were Raynauds phenomenon (100%), followed by synovitis (73%), swollen hands (68%), and myositis (48%). Lung involvement was evident in 53% and renal involvement in 15%. After a follow-up period averaging 3.3 years, 3 patients showed evidence of progressing towards SLE (increasing anti-dsDNA, decreased complement level, or disappearance of anti-nRNP and appearance of anti-Sm). One of our patients converted to SSc, but none of our patients converted to PM/DM. CONCLUSION: Only 4 (9%) of our patients showed evidence of developing into a classic connective tissue disease. Although we believe that regular follow-up on our patients would be needed, our findings lead us to conclude that MCTD is a distinct entity in Korean patients.

Clinical and serologic characteristics of mixed connective tissue disease in korea / 대한내과학회지

The concept that mixed connective tissue disease (MCTD) is a clinically distinct disease entity has been the subject of debate. Some authors regard it as a transitional phase in the development of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), or polymyositis/ dermatomyositis (PM-DM). This study set out to analyze the clinical and serologic characteristics of MCTD in Korean patients and to determine whether the diagnosis of MCTD held up on follow-up. METHODS: Forty-five patients diagnosed as MCTD using the diagnostic criteria of Alarcon-Segovia between October 1989 and February 1998 at Hanyang University Hospital were retrospectively reviewed. RESULTS: All but two patients were female. The average age at diagnosis was 39.3 years and the average diaseae duration was 7.5 years. The most frequent clinical symptoms were Raynauds phenomenon (100%), followed by synovitis (73%), swollen hands (68%), and myositis (48%). Lung involvement was evident in 53% and renal involvement in 15%. After a follow-up period averaging 3.3 years, 3 patients showed evidence of progressing towards SLE (increasing anti-dsDNA, decreased complement level, or disappearance of anti-nRNP and appearance of anti-Sm). One of our patients converted to SSc, but none of our patients converted to PM/DM. CONCLUSION: Only 4 (9%) of our patients showed evidence of developing into a classic connective tissue disease. Although we believe that regular follow-up on our patients would be needed, our findings lead us to conclude that MCTD is a distinct entity in Korean patients.