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Background/Aims@#This study aimed to identify the clinical characteristics of patients with concurrent rheumatoid arthritis (RA) and suspected non-tuberculous mycobacterial (NTM) infections as well as determine their prognostic factors. @*Methods@#We retrospectively reviewed the medical records of 91 patients with RA whose computed tomography (CT) findings suggested NTM infection. Subsequently, we compared the clinical characteristics between patients with and without clinical or radiological exacerbation of NTM-pulmonary disease (PD) and investigated the risk factors for the exacerbation and associated mortality. @*Results@#The mean age of patients with RA and suspected NTM-PD was 65.0 ± 10.2 years. The nodular/bronchiectatic (NB) form of NTM-PD was the predominant radiographic feature (78.0%). During follow-up, 36 patients (41.9%) experienced a radiological or clinical exacerbation of NTM-PD, whereas 12 patients (13.2%) died. Combined interstitial lung disease (ILD), microbiologically confirmed NTM-PD, and NB with the fibrocavitary (FC) form on chest CT were identified as risk factors for the clinical or radiological exacerbation of NTM-PD. Hydroxychloroquine use was identified as a good prognostic factor. Conversely, history of tuberculosis, ILD, smoking, microbiologically confirmed NTM-PD, and NB with the FC form on chest CT were identified as poor prognostic factors for mortality in suspected NTM-PD. @*Conclusions@#ILD and NB with the FC form on chest CT were associated with NTM-PD exacerbation and mortality. Hydroxychloroquine use may lower the risk of NTM-PD exacerbation. Therefore, radiographic features and presence of ILD should be considered when predicting the prognosis of patients with RA and suspected NTM-PD.
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Background@#This study aimed to compare the occurrence of adverse events (AEs) and disease flares after vaccination against coronavirus disease 2019 (COVID-19) and influenza in patients with autoimmune rheumatic diseases (ARDs). @*Methods@#Between November 2021 and March 2022, a survey was conducted among patients with ARD who received COVID-19 and influenza vaccinations. The questionnaire included 11 mandatory and closed-ended questions, and the following items were collected: medical history, immunization history, type of vaccine, patient-reported AEs, flare-up of the underlying disease after vaccination, and a confirmed diagnosis of COVID-19 or influenza. We compared the occurrence of vaccine-related adverse reactions to the COVID-19 and influenza vaccines based on the survey results. Multivariate logistic regression analysis was used to identify the factors affecting AEs or disease flares and to compare the post-vaccine response to mixed and matched vaccines. @*Results@#We analyzed 601 adults with ARD who received the COVID-19 vaccine, with a mean age of 49.6 years (80.5% female). A total of 255 participants (42.4%) received a complete course of primary vaccination, 342 (56.9%) completed the booster dose, and 132 (38.6%) received a mixed vaccine. The frequencies of AEs (188 [52.2%] vs. 21 [5.8%]; P< 0.001) and disease flares (58 [16.2%] vs. 5 [1.4%]; P < 0.001) after COVID-19 vaccination were significantly higher than those after influenza vaccination. In the risk factor analysis, previous allergic reaction to other vaccines (odds ratio, 1.95; confidence interval, 1.07–3.70; P = 0.034) was the only factor associated with the occurrence of AEs. There was no difference in the post-vaccine responses between the mixed and matched vaccines. @*Results@#We analyzed 601 adults with ARD who received the COVID-19 vaccine, with a mean age of 49.6 years (80.5% female). A total of 255 participants (42.4%) received a complete course of primary vaccination, 342 (56.9%) completed the booster dose, and 132 (38.6%) received a mixed vaccine. The frequencies of AEs (188 [52.2%] vs. 21 [5.8%]; P < 0.001) and disease flares (58 [16.2%] vs. 5 [1.4%]; P < 0.001) after COVID-19 vaccination were significantly higher than those after influenza vaccination. In the risk factor analysis, previous allergic reaction to other vaccines (odds ratio, 1.95; confidence interval, 1.07–3.70; P = 0.034) was the only factor associated with the occurrence of AEs. There was no difference in the post-vaccine responses between the mixed and matched vaccines. @*Conclusion@#The results of the survey of patients with ARD revealed that patient-reported AEs and underlying disease flares after receiving the COVID-19 vaccine were significantly higher than those after the influenza vaccine.
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Background@#We purposed to evaluate the seasonality and associated factors of the incidence of gout attacks in Korea. @*Methods@#We prospectively enrolled patients with gout attacks who were treated at nine rheumatology clinics between January 2015 and July 2018 and followed them for 1-year. Demographic data, clinical and laboratory features, and meteorological data including seasonality were collected. @*Results@#Two hundred-five patients (men, 94.1%) were enrolled. The proportion of patients with initial gout attacks was 46.8% (n = 96). The median age, body mass index, attack duration, and serum uric acid level at enrollment were 50.0 years, 25.4, 5.0 days, and 7.4 mg/dL, respectively. Gout attacks were most common during spring (43.4%, P < 0.001) and in March (23.4%, P < 0.001). A similar pattern of seasonality was observed in the group with initial gout attacks. Alcohol was the most common provoking factor (39.0%), particularly during summer (50.0%). The median diurnal temperature change on the day of the attack was highest in the spring (9.8°C), followed by winter (9.3°C), fall (8.6°C), and summer (7.1°C) (P = 0.027). The median change in humidity between the 2 consecutive days (the day before and the day of the attack) was significantly different among the seasons (3.0%, spring; 0.3%, summer; −0.9%, fall; −1.2%, winter; P = 0.015). One hundred twenty-five (61%) patients completed 1-year follow-up (51% in the initial attack group). During the follow-up period, 64 gout flares developed (21 in the initial attack group). No significant seasonal variation in the follow-up flares was found. @*Conclusion@#In this prospective study, the most common season and month of gout attacks in Korea are spring and March, respectively. Alcohol is the most common provoking factor, particularly during summer. Diurnal temperature changes on the day of the attack and humidity changes from the day before the attack to the day of the attack are associated with gout attack in our cohort.
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Objectives@#Osteoporosis and fracture are known complications of systemic lupus erythematosus (SLE). We assessed the prevalence and risk factors for osteoporosis in patients with SLE. @*Methods@#A total of 155 female SLE patients were recruited retrospectively in 5 university hospitals. The bone mineral density (BMD) was measured by dual-energy X-ray absorptiometry, and the fracture risk assessment tool (FRAX) for high-risk osteoporotic fractures was calculated with and without BMD. @*Results@#The mean age was 53.7 ± 6.8 years, and osteoporotic fractures were detected in 19/127 (15.0%) patients. The proportion of patients having a high-risk for osteoporotic fractures in the FRAX with and without BMD, and osteoporosis by the World Health Organization (WHO) criteria were 25 (16.1%), 24 (15.5%), and 51 (32.9%), respectively, and 48.0–68.6% of them were receiving treatment. On multivariate logistic analysis, nephritis (odds ratio [OR] 11.35) and cumulative dose of glucocorticoid (OR 1.1) were associated with high-risk by the FRAX with BMD, and low complement levels (OR 4.38), erythrocyte sedimentation rate (ESR) (OR 1.04), and cumulative dose of glucocorticoid (OR 1.05) were associated with osteoporosis by the WHO criteria in patients with SLE. @*Conclusions@#Among Korean female patients with SLE, the proportion of patients having a high-risk of osteoporotic fractures by the FRAX tool was 15.5%–16.1% and the proportion of patients having osteoporosis by the WHO criteria was 32.9%. In SLE, nephritis, low level of complement, ESR, and cumulative dose of glucocorticoids may contribute to fracture risk.
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Objectives@#Osteoporosis and fracture are known complications of systemic lupus erythematosus (SLE). We assessed the prevalence and risk factors for osteoporosis in patients with SLE. @*Methods@#A total of 155 female SLE patients were recruited retrospectively in 5 university hospitals. The bone mineral density (BMD) was measured by dual-energy X-ray absorptiometry, and the fracture risk assessment tool (FRAX) for high-risk osteoporotic fractures was calculated with and without BMD. @*Results@#The mean age was 53.7 ± 6.8 years, and osteoporotic fractures were detected in 19/127 (15.0%) patients. The proportion of patients having a high-risk for osteoporotic fractures in the FRAX with and without BMD, and osteoporosis by the World Health Organization (WHO) criteria were 25 (16.1%), 24 (15.5%), and 51 (32.9%), respectively, and 48.0–68.6% of them were receiving treatment. On multivariate logistic analysis, nephritis (odds ratio [OR] 11.35) and cumulative dose of glucocorticoid (OR 1.1) were associated with high-risk by the FRAX with BMD, and low complement levels (OR 4.38), erythrocyte sedimentation rate (ESR) (OR 1.04), and cumulative dose of glucocorticoid (OR 1.05) were associated with osteoporosis by the WHO criteria in patients with SLE. @*Conclusions@#Among Korean female patients with SLE, the proportion of patients having a high-risk of osteoporotic fractures by the FRAX tool was 15.5%–16.1% and the proportion of patients having osteoporosis by the WHO criteria was 32.9%. In SLE, nephritis, low level of complement, ESR, and cumulative dose of glucocorticoids may contribute to fracture risk.
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OBJECTIVE: Pulmonary hypertension (PH) develops frequently in connective tissue diseases (CTD) and is an important prognostic factor. The aim of this study was to assess the prevalence of PH in patients with CTD by non-invasive echocardiography and analyze the potential biomarkers for helping to detect PH. METHODS: All Korean patients with CTD who had dyspnea on exertion or interstitial lung disease (ILD) were screened for PH with echocardiography and clinical data were collected from four hospitals. RESULTS: Among 196 patients with CTD, 108 (55.1%) had ILD and 21 had PH defined as >40 mmHg. Of the 21 patients with PH, 10, 4, and 3 patients had systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease, respectively. There was no difference in the incidence of PH according to the presence of ILD; 12 patients (11.1%) with ILD had PH and 9 patients (10.2%) without ILD had PH. The results of the pulmonary function test, total cholesterol, red cell volume distribution width, alkaline phosphatase, and the New York Heart Association (NYHA) functional class III or IV differed significantly according to the presence of PH. In multiple regression analysis, NYHA functional class III or IV (odd ratio [OR]=7.3, p=0.009) and forced vital capacity (OR=0.97, p=0.043) were independent predictive factors of PH. CONCLUSION: PH is not associated with the presence of ILD in Korean patients with CTD. On the other hand, NYHA functional class III or IV and decreased forced vital capacity indicate the presence of PH in connective tissue disease.
Subject(s)
Humans , Alkaline Phosphatase , Biomarkers , Cell Size , Cholesterol , Connective Tissue Diseases , Connective Tissue , Dyspnea , Echocardiography , Hand , Heart , Hydrogen-Ion Concentration , Hypertension, Pulmonary , Incidence , Lung Diseases, Interstitial , Lupus Erythematosus, Systemic , Mixed Connective Tissue Disease , Prevalence , Respiratory Function Tests , Risk Factors , Scleroderma, Systemic , Vital CapacityABSTRACT
Systemic lupus erythematosus (SLE) is a systemic autoimmune inflammatory disease that frequently involves the joints at some stage during the disease course. Although lupus arthritis is usually non-erosive, approximately 5% of patients develop erosions. This paper reports a patient with SLE combined with erosive arthritis, who was treated successfully with tocilizumab. A 20-year-old female, who was first diagnosed with SLE at the age of 13 years, had been admitted frequently to hospital with disease flare-ups during the previous 3 years. Despite aggressive treatment, her arthritis became aggravated, particularly in both wrists and the right knee. Radiologically, erosive arthritis was noted in the wrists.The serum interleukin (IL)-6 level was elevated significantly along with the other inflammatory markers. After the tocilizumab treatment, the arthritis subsided with a normalization of the IL-6 level, suggesting that tocilizumab may be a suitable treatment for lupus erosive arthritis if the IL-6 level is high.
Subject(s)
Female , Humans , Young Adult , Arthritis , Interleukin-6 , Interleukins , Joints , Knee , Lupus Erythematosus, Systemic , WristABSTRACT
BACKGROUND/AIMS: ¹⁸F-fluorodeoxyglucose positron emission tomography/computed tomography (¹⁸F-FDG PET/CT) has been suggested as a reliable imaging technique for monitoring of disease activity in patients with adult-onset Still’s disease (AOSD). Therefore, we investigated the clinical significance of ¹⁸F-FDG PET/CT in Korean AOSD patients. METHODS: Thirteen AOSD patients were included in the study. The PET/CT images were evaluated with visual and semiquantitative method using standardized uptake values (SUVs). RESULTS: The presence of increased ¹⁸F-FDG uptake was noted in 90% of clinically active AOSD patients. ¹⁸F-FDG uptake was located in the lymph node, spleen, and bone marrow. Visual grade and SUV intensity of lymph node was significantly correlated with the systemic score of AOSD. Visual grade of spleen was significantly correlated with the systemic score, erythrocyte sedimentation rate (ESR), and ferritin. Additionally, visual grade and SUV intensity of bone marrow was significantly correlated with the systemic score, ESR, leukocyte, and neutrophil. CONCLUSIONS: Visual grade and SUV intensity of lymph node, spleen, and bone marrow on ¹⁸F-FDG PET/CT scan showed significant correlations with known disease activity markers. The data suggest that ¹⁸F-FDG PET/CT scan may be a useful imaging technique for evaluation of disease activity in AOSD patients.
Subject(s)
Humans , Blood Sedimentation , Bone Marrow , Electrons , Ferritins , Leukocytes , Lymph Nodes , Methods , Neutrophils , Positron Emission Tomography Computed Tomography , Positron-Emission Tomography , SpleenABSTRACT
Diffuse alveolar hemorrhage (DAH) is a life-threatening condition associated with many disorders. Here, we report a case of 59-year-old female who had diffuse alveolar hemorrhage associated with methimazole. She had been treated with methimazole for two weeks due to the recurrence of Grave's disease, before visiting the emergency room. She had to be intubated on the 3rd day of hospitalization because of unabated massive hemoptysis and rapid progression of diffuse alveolar infiltration on chest radiographs. Since her clinical condition improved substantially after cessation of methimazole and steroid pulse therapy, she was extubated on the 9th day of hospitalization and then discharged. After discharge, DAH did not recur with cessation of steroid and she had radioactive iodine therapy for her Grave's disease. This was a rare and interesting case of life-threatening DAH associated with cytoplasmic-antineutrophil cytoplasmic antibody and methimazole.
Subject(s)
Female , Humans , Middle Aged , Antibodies, Antineutrophil Cytoplasmic , Cytoplasm , Emergency Service, Hospital , Hemoptysis , Hemorrhage , Hospitalization , Iodine , Methimazole , Radiography, Thoracic , RecurrenceABSTRACT
Osteoporosis is a common adverse event among patients on glucocorticoid therapy. Glucocorticoids reduce bone formation and increase cortical porosity in proportion to the dose and duration of glucocorticoid use. While the epidemiology of glucocorticoid-induced osteoporosis has been well characterized, its pathophysiology and effective management remain unclear. Several recommendations for glucocorticoid-induced osteoporosis are used to determine which patients on long-term glucocorticoid treatment to treat and when. The fracture risk can be assessed using dual-energy X-ray absorptiometry and the Fracture Risk Assessment Tool algorithm, along with other clinical factors. The management of glucocorticoid-induced osteoporosis includes anti-osteoporotic therapy and measures to prevent bone loss. Bisphosphonates are currently the first choice treatment, with teriparatide and denosumab being alternatives.
Subject(s)
Humans , Absorptiometry, Photon , Bone Density , Denosumab , Diphosphonates , Epidemiology , Glucocorticoids , Osteogenesis , Osteoporosis , Porosity , Risk Assessment , TeriparatideABSTRACT
Pulmonary arterial hypertension is a critical manifestation of systemic sclerosis (SSc) and is a main cause of death. Several treatment modalities for SSc have been identified, with effects that improve quality of life and mortality rates. However, whether these drugs can also normalize pulmonary arterial pressure, remains unclear. Here, we report the case of a woman with diffuse SSc with pulmonary arterial hypertension, who had a functional status equivalent to the New York Heart Association class III. The patient was treated with inhaled iloprost. After six years of inhaled iloprost therapy, echocardiography showed that pulmonary arterial pressure normalized, accompanied by improvement in functional capacity. Inhaled iloprost might not only normalize pulmonary arterial pressure, but also improve the functional status of patients with SSc with pulmonary arterial hypertension.
Subject(s)
Female , Humans , Arterial Pressure , Cause of Death , Echocardiography , Heart , Hypertension , Hypertension, Pulmonary , Iloprost , Mortality , Quality of Life , Scleroderma, SystemicABSTRACT
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with diverse manifestations, and its pathogenesis is unclear and complicated. Infection and SLE are similar in that they both cause inf lammatory reactions in the immune system; however, one functions to protect the body, whereas the other is activated to damage the body. Infection is known as one of the common trigger factors for SLE; there are a number of reports on infectious agents that provoke autoimmune response. Several viruses, bacteria, and protozoa were revealed to cause immune dysfunction by molecular mimicry, epitope spreading, and bystander activation. In contrast, certain pathogens were revealed to protect from immune dysregulation. Infection can be threatening to patients with SLE who have a compromised immune system, and it is regarded as one of the common causes of mortality in SLE. A clinical distinction between infection and lupus f lare up is required when patients with SLE present fevers. With a close-up assessment of symptoms and physical examination, C-reactive protein and disease activity markers play a major role in differentiating the different disease conditions. Vaccination is necessary because protection against infection is important in patients with SLE.
Subject(s)
Humans , Autoimmune Diseases , Autoimmunity , Bacteria , C-Reactive Protein , Fever , Immune System , Lupus Erythematosus, Systemic , Molecular Mimicry , Mortality , Physical Examination , VaccinationABSTRACT
Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder characterized by fever, pancytopenia, hyperferritinemia, and phagocytosis of hematopoietic cells in bone marrow, liver, or lymph nodes. HLH can occur during the course of systemic lupus erythematosus (SLE), but can also be a presenting manifestation. Because development of pancytopenia occurs in less than 10 percent of SLE cases, investigation for HLH is necessary when otherwise unexplained pancytopenia persists despite adequate treatment. We experienced three cases of secondary HLH associated with SLE. Among the three patients, two patients developed HLH during the clinical course of SLE. The other patient who presented with pancytopenia was first diagnosed with HLH, and later with SLE. In her case, HLH turned out to be a presenting manifestation of SLE. We report on three successfully treated cases, and discuss the prevalence, characteristics, treatments, and prognosis of secondary HLH associated with SLE.
Subject(s)
Humans , Bone Marrow , Fever , Liver , Lupus Erythematosus, Systemic , Lymph Nodes , Lymphohistiocytosis, Hemophagocytic , Pancytopenia , Phagocytosis , Prevalence , PrognosisABSTRACT
We reviewed the recent findings in diagnosis, pathogenesis and management of Sjogren's syndrome. New diagnostic classification criteria for Sjogren's syndrome were published by the American College of Rheumatology in 2012, and validation was completed. Salivary gland ultrasonography has been examined as a diagnostic and prognostic marker for Sjogren's syndrome, and the results have revealed that it has high specificity and low sensitivity. Disease activity assessment tools for Sjogren's syndrome have been developed and validated for upcoming clinical trials. Several associated genes were identified by genome-wide association studies, with large cohorts in Europe and China. Several single nucleotide polymorphisms of Sjogren's syndrome are related to germinal center formation and lymphoma development. New treatment modalities, including interleukin-1 receptor antagonist, anti-CD20, anti-BAFF/Blys, and CTLA4-immunoglobulin were investigated, but the optimal therapeutic compounds have yet to be found.
Subject(s)
China , Classification , Cohort Studies , Diagnosis , Europe , Genome-Wide Association Study , Germinal Center , Interleukin-1 , Lymphoma , Polymorphism, Single Nucleotide , Rheumatology , Salivary Glands , Sensitivity and Specificity , Sjogren's Syndrome , UltrasonographyABSTRACT
Inflammatory myopathy is characterized by symmetrical proximal muscle weakness, elevated muscle enzyme levels and favorable response to glucocorticoids therapy. Although periorbital edema is a common manifestation of inflammatory myopathy, generalized subcutaneous edema is very rare. We report here a case of a 47-year-old female patient with acute polymyositis/systemic lupus erythematosus overlap syndrome with generalized subcutaneous edema and interstitial lung disease. We aggressively treated the disease with high-dose glucocorticoids, intravenous immunoglobulin, and immunosuppressive agents.
Subject(s)
Female , Humans , Middle Aged , Edema , Glucocorticoids , Immunoglobulins , Immunosuppressive Agents , Lung Diseases, Interstitial , Muscle Weakness , Muscles , MyositisABSTRACT
It is known that rheumatoid arthritis (RA) patients show increased incidence of multiple myeloma (MM), despite its rarity. Only one case of MM with seronegative RA was reported in Korea, thus far. We report a case of MM with seropositive RA. The patient was a 66 year old female who had been diagnosed with seropositive RA 4 years ago. Over the last 1 month, the patient experienced general weakness and weight loss of 10 kg. It was found that her serum creatinine had increased and her urine analysis showed proteinuria. To evaluate renal failure and proteinuria, renal biopsy, bone marrow biopsy and electrophoresis were carried out. A diagnosis of myeloma cast nephropathy was made. We report this rare case of MM represented as acute renal failure during the treatment for RA, and include a review of the literature.
Subject(s)
Female , Humans , Acute Kidney Injury , Arthritis, Rheumatoid , Biopsy , Bone Marrow , Creatinine , Diagnosis , Electrophoresis , Incidence , Korea , Multiple Myeloma , Proteinuria , Renal Insufficiency , Weight LossABSTRACT
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder that predominantly affects women of reproductive age. Risk of SLE flare during pregnancy is dependent on disease activity of SLE and proteinuria at the time of conception, which affect pregnancy outcome. We report on three patients who developed renal flares during pregnancy after remission of lupus nephritis before pregnancy. Two patients were treated successfully, with pregnancy outcomes of live births however, another patient's pregnancy was terminated with induced abortion. For SLE patients, family planningis needed until disease activity of SLE has been stable for at least six months prior to the pregnancy. Nevertheless, flares of lupus could develop and influence maternal and fetal outcome. Therefore, renal flares during pregnancy should be recognized and treated immediately.
Subject(s)
Female , Humans , Pregnancy , Abortion, Induced , Fertilization , Live Birth , Lupus Erythematosus, Systemic , Lupus Nephritis , Nephritis , Pregnancy Outcome , Pregnant Women , ProteinuriaABSTRACT
There are numerous studies about the transformation of renal pathology during lupus nephritis progression. A number of researchers suggest that patients with previous proliferative glomerulonephritis may not need to repeat renal biopsy in relation to treatment strategies. However, the pathology of renal biopsy could offer important information to clinicians about the progression of disease. Here, we report a rare case of the convertion of ISN/RPS classification from a proliferative lesion to a wholly non-proliferative lesion. A 40-year-old female was admitted complaining of generalized edema for 1 month. At the age of 33 she had been diagnosed as SLE with proliferative lupus nephritis. The renal remission was induced with corticosteroid pulse therapy and 12 cycles of intravenous cyclophosphamide treatment. The repeated renal biopsy revealed class V lupus nephritis compared with referential biopsy of class IV-G. A better prognosis is expected with lower activity and a lower chronicity index. Repeat renal biopsy may give useful information relating to the prognosis of nephritis.
Subject(s)
Adult , Female , Humans , Biopsy , Cyclophosphamide , Edema , Glomerulonephritis , Lupus Nephritis , Nephritis , PrognosisABSTRACT
Systemic lupus erythematosus (SLE) is an autoimmune disease with various manifestations, while its autoantibodies and immune reactions involve multiple organs. Neuropsychiatric involvement in SLE is known to be common, however, peripheral neuropathy is relatively rare. Guillain-Barre syndrome is clinically defined as an acute demyelinating peripheral neuropathy causing weakness and numbness in the legs and arms. We describe a case of Guillain-Barre syndrome with antiphospholipid syndrome and lupus nephritis. The patient was admitted with fever and diarrhea. He developed progressive weakness of the upper and lower extremities and dysarthria with characteristic nerve conduction patterns compatible with Guillain-Barre syndrome. He also had proteinuria and gangrene of the hand and toe with antiphospholipid antibody. He received intravenous immunoglobulin and plasmapheresis for progressive neuropathy, intravenous high dose steroid to control activity of SLE, and anticoagulation for antiphospholipid syndrome. Neuropsychiatric manifestation of SLE is related to lupus activity closely, so it is important to control lupus activity.
Subject(s)
Humans , Antibodies, Antiphospholipid , Antiphospholipid Syndrome , Arm , Autoantibodies , Autoimmune Diseases , Diarrhea , Dysarthria , Fever , Gangrene , Guillain-Barre Syndrome , Hand , Hypesthesia , Immunoglobulins , Leg , Lower Extremity , Lupus Erythematosus, Systemic , Lupus Nephritis , Neural Conduction , Peripheral Nervous System Diseases , Plasmapheresis , Proteinuria , ToesABSTRACT
OBJECTIVE: Henoch-Schonlein purpura (HSP) is a systemic vasculitis, characterized by small-vessel leukocytoclastic vasculitis with the deposition of immune complexes containing IgA. It is the most common acute vasculitic disorder affecting children but is relatively uncommon in adults. We investigated the clinical features and factors affecting the prognosis of adult HSP in Korea. METHODS: From 1996 to 2011 seventy patients over 15 years of age with HSP were retrospectively analyzed. RESULTS: Thirty eight patients (54.3%) were female and the age at disease onset ranged from 15 to 75 years (35.0+/-15.8 years). Purpuric skin rash was observed in all patients and arthralgia was present in 34 patients (48.6%). GI symptoms and kidney involvements were observed in 28 patients (40.0%) and 34 patients (48.6%), respectively. Complete remission was achieved in 46 patients (65.7%). The remission group showed a lower incidence of hematochezia (p=0.044), hematuria (p=0.008), and proteinuria (p=0.011) at diagnosis than the no remission group. About 10% of adult HSP patient progressed to chronic kidney disease (CKD), which showed higher a incidence of nephrotic range proteinuria. Only nephrotic range proteinuria at diagnosis was a significant risk factor for CKD (OR=16.7, p=0.008, 95% CI=2.1~133.1). CONCLUSION: Hematochezia, hematuria and proteinuria at the diagnosis of HSP are important prognostic factors in predicting remission. In addition, HSP patients with nephrotic range proteinuria at diagnosis have an increased risk of renal failure.