Two Cases of Refractory Thrombocytopenia in Systemic Lupus Erythematosus that Responded to Intravenous Low-Dose Cyclophosphamide

Treatment of thrombocytopenia in systemic lupus erythematosus (SLE) is considered in cases of current bleeding, severe bruising, or a platelet count below 50,000/µL. Corticosteroid is the first choice of medication for inducing remission, and immunosuppressive agents can be added when thrombocytopenia is refractory to corticosteroid or recurs despite it. We presented two SLE patients with thrombocytopenia who successfully induced remission after intravenous administration of low-dose cyclophosphamide (CYC) (500 mg fixed dose, biweekly for 3 months), followed by azathioprine (AZA) or mycophenolate mofetil (MMF). Both patients developed severe thrombocytopenia in SLE that did not respond to pulsed methylprednisolone therapy, and started the intravenous low-dose CYC therapy. In case 1, the platelet count increased to 50,000/µL after the first CYC infusion, and remission was maintained with low dose prednisolone and AZA. The case 2 achieved remission after three cycles of CYC, and the remission continued with low dose prednisolone and MMF.ope

A Case of Atypical Giant Cell Arteritis Presenting as a Fever of Unknown Origin

Giant cell arteritis (GCA) is a systemic vasculitis predominantly found in individuals of Northern European ancestry over 50 years of age. Typically it presents with new-onset persistent headache, claudication of jaws, and existence of an abnormal temporal artery. However, the diagnosis of GCA and the assessment of its activity remain challenging, especially in patients presenting with a variety of non-specific symptoms and laboratory tests. In those cases, 18F-fluorodeoxy-glucose positron emission tomography (18F-FDG-PET) is useful for the diagnosis of GCA. Recently, the number of foreign patients who visit domestic hospitals for medical care is increasing in Korea. Here-in, we report a Russian patient who was admitted to our hospital with fever of unknown origin (FUO). FUO study was performed to search for infection or malignancy and GCA was suspected by 18F-FDG-PET. The patient was eventually diagnosed with GCA by random temporal artery biopsy.ope

Differences in Clinical Manifestations and Outcomes between Adult and Child Patients with Henoch-Schonlein Purpura

We aimed to investigate differences in clinical manifestations and outcomes between adult and child patients with Henoch-Schönlein purpura (HSP), and to analyze the factors associated with poor prognosis for HSP nephritis. This retrospective 10-yr study enrolled 160 patients with HSP who visited Severance Hospital. Purpura was mostly detected in lower extremities, but purpura in upper extremities was more frequently observed in adults than children (41.7% vs 19.3%). Children had a greater frequency of arthralgia (55.4% vs 27.1%), while adults had a greater frequency of diarrhea (20% vs 1.6%). Anemia, elevated C-reactive protein, and level of IgA were more frequently observed in adults (25% vs 7.1%, 65.6% vs 38.4%, 26.3% vs 3.5%). Renal involvement in adults was more severe than in children (79.2% vs 30.4%). Chronic renal failure showed a significant difference in outcomes of HSP between adults (10.4%) and children (1.8%) after a follow up period of an average of 27 months. Furthermore, renal insufficiency at diagnosis was significantly related to the progression to chronic renal failure. Our results showed several differences in the clinical features of HSP between adults and children. Adults with HSP had a higher frequency of renal insufficiency and worse renal outcomes than children. Renal insufficiency at diagnosis might be of predictive value for the progression to chronic renal failure in HSP patients.ope

Typical 18-FDG-PET/CT Findings of Polymyalgia Rheumatica: A Case Report

Polymyalgia rheumatica (PMR) is an inflammatory rheumatic condition characterized by generalized pain and morning stiffness in the shoulders, hip girdle, and neck. Since the pathogenesis of PMR is still uncertain, the diagnosis of PMR depends on clinical features. There have been several studies regarding radiological tools for the diagnosis of PMR. Recent studies using 18-FDG-PET showed bursitis, synovitis, uptake in the spinous process and asymptomatic large-vessel vasculitis in PMR patients. However, there was no report on the efficacy of 18-FDG-PET for diagnosis of PMR in Korea. Here, we are first reporting a case of a Korean patient with PMR, who had radiological findings including bursitis, synovitis, uptake in the spinous process and asymptomatic large-vessel vasculitis on 18-FDG-PET/CT.ope

Adipokines, inflammation, insulin resistance, and carotid atherosclerosis in patients with rheumatoid arthritis.

INTRODUCTION: Cardiovascular (CV) morbidity and mortality are increased in patients with rheumatoid arthritis (RA). Inflammation is thought to be an important factor in accelerated atherosclerosis in RA, whereas insulin resistance is a known risk factor for atherosclerosis in RA. We hypothesised that adipokines could be a link between inflammation, insulin resistance, and atherosclerosis in RA. METHODS: The common carotid artery (CCA) intima-media thickness (IMT), CCA resistive index (RI), and carotid plaques were measured by ultrasonography in 192 patients with RA. Insulin resistance was assessed by the homeostasis model assessment for insulin resistance (HOMA-IR). Serum adiponectin, leptin, resistin, tumor necrosis factor-α, and interleukin (IL)-6 concentrations were determined. RESULTS: The CCA RI was associated with CCA IMT and the estimated total plaque volume after adjustment for conventional CV risk factors. Among adipokines, resistin and IL-6 were correlated with inflammatory parameters. Leptin and leptin:adiponectin (L:A) ratio were correlated with metabolic risk factors, including HOMA-IR. And L:A ratio was related to the CCA RI after adjustment for conventional and nonconventional CV risk factors, including HOMA-IR, erythrocyte sedimentation rate and C-reactive protein. CONCLUSION: L:A ratio was associated with HOMA-IR and carotid RI. L:A ratio might be an independent factor for predicting cardiovascular risk in patients with RA.ope