We reported the diagnostic and therapeutic process of a young male patient with systemic lupus erythematosus (SLE) who presented with severe hyponatremia as the main manifestation upon admission, and analyzed and discussed the case. The patient was a 19-year-old young male with a subacute course of disease, fever ≥38.3 ℃ that could not be explained by other causes, acute and subacute cutaneous lupus erythematosus, oral ulcers, arthritis, leukopenia (< 4×10⁹/L), low C3+low C4, and positive anti-double-stranded DNA (anti-dsDNA). According to the 2019 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria, the score was 27 points. The patient was admitted to the hospital with SLE. After admission, further diagnosis of lupus was confirmed, excluding infection, tumor, endocrine disease, etc. Hyponatremia was the main complication of this lupus patient. Hyponatremia was a rare complication of lupus, only a few cases have been reported. In this study, the paient ' s blood osmotic pressure was significantly reduced, which was considered to be hypotonic hyponatretic, urine osmotic pressure increased, maximum urine dilution caused by excessive water intake such as primary polydipsia, hypoosmotic fluid intake, and beer drinking were excluded, and 24 h urine volume and sodium were improved. The urinary sodium concentration was close to 20 mmol/L although with severe hyponatremia, considering the possibility of isovolemic hypotonic hyponatremia, the syndrome of improper secretion of antidiuretic hormone or adrenal cortical insufficiency. The patient had no manifestations, such as hypotension, typical site pigmentation, and high potassium, and there was little possibility of adrenal cortical insufficiency, and syndrome of inappropriate antidiuretic hormone secretion (SIADH) was considered for hyponatremia in the patient. The etiological mechanism of hyponatremia in lupus patients is not clear, but it is related to acute kidney injury, drugs and systemic inflammation. In this case, we reported for the first time that SLE was associated with abnormal hypothalamic signals, suggesting a possible mechanism of lupus hyponatremia. The patient underwent water restriction, intravenous and oral sodium supplementation, and the blood sodium quickly returned to normal after pulse therapy. The abnormal signal of the head magnetic resonance imaging (MRI) fornix column was improved after 1 month of treatment, further confirming our diagnosis. SLE complicated with hyponatremia is rare, but severe hyponatremia can be life-threatening, and attention should be paid to it. The possibility of neuropsychiatric lupus should be vigilant in patients with lupus combined with hyponatremia.
Humans ; Hyponatremia/etiology* ; Lupus Erythematosus, Systemic/diagnosis* ; Male ; Young Adult

Humans ; Lupus Erythematosus, Systemic/diagnosis* ; Hemorrhage/etiology* ; Lung Diseases/etiology* ; Pulmonary Alveoli

Chorea/etiology* ; Humans ; Lupus Erythematosus, Systemic/diagnosis* ; Phototherapy

Adult ; Cerebral Angiography ; Female ; Humans ; Intracranial Aneurysm ; complications ; diagnostic imaging ; Lupus Erythematosus, Systemic ; complications ; diagnostic imaging ; Subarachnoid Hemorrhage ; diagnosis ; diagnostic imaging ; etiology ; Young Adult

BACKGROUNDMyocarditis is an uncommon but serious manifestation of systemic lupus erythematosus (SLE). This study aimed to investigate clinical characteristics and outcomes of lupus myocarditis (LM) and to determine risk factors of LM in hospitalized Chinese patients with SLE.

METHODSWe conducted a retrospective case-control study. A total of 25 patients with LM from 2001 to 2012 were enrolled as the study group, and 100 patients with SLE but without LM were randomly pooled as the control group. Univariable analysis was performed using Chi-square tests for categorical variables, and the Student's t-test or Mann-Whitney U-test was performed for continuous variables according to the normality.

RESULTSLM presented as the initial manifestation of SLE in 7 patients (28%) and occurred mostly at earlier stages compared to the controls (20.88 ± 35.73 vs. 44.08 ± 61.56 months, P = 0.008). Twenty-one patients (84%) experienced episodes of symptomatic heart failure. Echocardiography showed that 23 patients (92%) had decreased left ventricular ejection fraction (<50%) and all patients had wall motion abnormalities. A high SLE Disease Activity Index was the independent risk factor in the development of LM (odds ratio = 1.322, P < 0.001). With aggressive immunosuppressive therapies, most patients achieved satisfactory outcome. The in-hospital mortality was not significantly higher in the LM group than in the controls (4% vs. 2%,P = 0.491).

CONCLUSIONSLM could result in cardiac dysfunction and even sudden death. High SLE disease activity might potentially predict the occurrence of LM at the early stage of SLE. Characteristic echocardiographic findings could confirm the diagnosis of LM. Early aggressive immunosuppressive therapy could improve the cardiac outcome of LM.

Adult ; Case-Control Studies ; China ; Echocardiography ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; Male ; Multivariate Analysis ; Myocarditis ; diagnosis ; etiology ; Retrospective Studies ; Risk Factors

BACKGROUND/AIMS: Protein-losing enteropathy (PLE), characterized by severe hypoalbuminemia and peripheral edema, is a rare manifestation of systemic lupus erythematosus. This present study aimed to identify the distinctive features of lupus-related PLE and evaluate the factors related to the treatment response. METHODS: From March 1998 to March 2014, the clinical data of 14 patients with lupus PLE and seven patients with idiopathic PLE from a tertiary center were reviewed. PLE was defined as a demonstration of protein leakage from the gastrointestinal tract by either technetium 99m-labelled human albumin scanning or fecal alpha1-antitrypsin clearance. A positive steroid response was defined as a return of serum albumin to > or = 3.0 g/dL within 4 weeks after initial steroid monotherapy, and remission as maintenance of serum albumin > or = 3.0 g/dL for at least 3 months. A high serum total cholesterol level was defined as a level of > or = 240 mg/dL. RESULTS: The mean age of the lupus-related PLE patients was 37.0 years, and the mean follow-up duration was 55.8 months. Significantly higher erythrocyte sedimentation rate and serum total cholesterol levels were found for lupus PLE than for idiopathic PLE. Among the 14 patients with lupus PLE, eight experienced a positive steroid response, and the serum total cholesterol level was significantly higher in the positive steroid response group. A positive steroid response was associated with an initial high serum total cholesterol level and achievement of remission within 6 months. CONCLUSIONS: In lupus-related PLE, a high serum total cholesterol level could be a predictive factor for the initial steroid response, indicating a good response to steroid therapy alone.
Adult ; Aged ; Biomarkers/blood ; Cholesterol/blood ; Drug Therapy, Combination ; Edema/diagnosis/drug therapy/*etiology ; Female ; Glucocorticoids/therapeutic use ; Humans ; Hypoalbuminemia/diagnosis/drug therapy/*etiology ; Immunosuppressive Agents/therapeutic use ; Lupus Erythematosus, Systemic/*complications/diagnosis/drug therapy ; Male ; Middle Aged ; Protein-Losing Enteropathies/diagnosis/drug therapy/*etiology ; Remission Induction ; Risk Factors ; Serum Albumin/metabolism ; Tertiary Care Centers ; Time Factors ; Treatment Outcome

We describe an unusual case of systemic lupus erythematosus with pulmonary manifestations presenting as hypoglycemia due to anti-insulin receptor antibodies. A 38-year-old female suffered an episode of unconsciousness and was admitted to hospital where her blood glucose was found to be 18 mg/dL. During the hypoglycemic episode, her serum insulin level was inappropriately high (2,207.1 pmol/L; normal range, 18 to 173) and C-peptide level was elevated (1.7 nmol/L; normal range, 0.37 to 1.47). Further blood tests revealed the presence of antinuclear antibodies, anti-double-stranded DNA antibodies, and anti-Ro/SSA, anti-La/SSB, anti-ribonucleoprotein, and anti-insulin receptor antibodies. A computed tomography scan of the abdomen, aimed at tumor localization, such as an insulinoma, instead revealed ground-glass opacities in both lower lungs, and no abnormal finding in the abdomen. For a definitive diagnosis of the lung lesion, video-associated thoracoscopic surgery was performed and histopathological findings showed a pattern of fibrotic non-specific interstitial pneumonia.
Adult ; Autoantibodies/*blood ; *Autoimmunity ; Biological Markers/blood ; Blood Glucose/metabolism ; Female ; Humans ; Hypoglycemia/blood/*complications/immunology ; Insulin/blood ; *Insulin Resistance ; Lung Diseases, Interstitial/diagnosis/*etiology/immunology/surgery ; Lupus Erythematosus, Systemic/*complications/diagnosis/immunology ; Receptor, Insulin/*immunology ; Thoracic Surgery, Video-Assisted ; Tomography, X-Ray Computed ; Treatment Outcome

Treatment of thrombocytopenia in systemic lupus erythematosus (SLE) is considered in cases of current bleeding, severe bruising, or a platelet count below 50,000/microliter. 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/microliter 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.
Azathioprine/therapeutic use ; Bone Marrow/pathology ; Cyclophosphamide/*therapeutic use ; Drug Therapy, Combination ; Female ; Humans ; Immunosuppressive Agents/*therapeutic use ; Infusions, Intravenous ; Lupus Erythematosus, Systemic/complications/*diagnosis ; Middle Aged ; Mycophenolic Acid/analogs & derivatives/therapeutic use ; Platelet Count ; Thrombocytopenia/*diagnosis/*drug therapy/etiology ; Young Adult

Stroke mimickers are common, and they represent a diagnostic dilemma for clinicians. Many, like posterior reversible encephalopathy syndrome (PRES), are easily reversible. The manifestation of PRES is characterised by headaches, convulsions, altered mental functioning and blindness. In most cases, computed tomography of the brain will show hypodense lesions in the parieto-occpitial lobe, which only further confounds the physician. Although this syndrome is uncommon, prompt and accurate recognition allows early treatment, which has been shown to produce favourable outcomes. Herein, we report the case of a 54-year-old woman, who presented with PRES, as an acute manifestation of systemic lupus erythematous (SLE) and lupus nephritis. The patient was initially thought to be experiencing an ischaemic stroke, but the diagnosis was later changed. On management of her underlying condition, her symptoms resolved. PRES should be recognised as an acute emergency manifestation of SLE. It should not be mistaken for an ischaemic stroke as inappropriate treatment could have adverse outcomes.
Acute Disease ; Biopsy ; Diagnosis, Differential ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; diagnosis ; Middle Aged ; Posterior Leukoencephalopathy Syndrome ; diagnosis ; etiology ; Tomography, X-Ray Computed

Child ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; diagnosis ; Myxoma ; etiology