A case of spontaneous hypoglycemia

We describe a case of systemic lupus erythematosus with POEMS syndrome presenting as spontaneous hypoglycemia. A 58-year-old female suffered repeated episodes of hypoglycemia. During thesehypoglycemic episodes, her postprandial insulin level was inappropriately high. Further blood tests revealed the presence of antinuclear antibodies, anti-double-stranded DNA antibodies,low C4level.Altered albumin-globulin ratio,monoclonal gammopathy (IgG LAMBDA), polyneuropathy and organomegaly lead to suspicion of concurrent presence of POEMS syndrome.Bone marrow examination revealed plasma cell dyscrasia and plasmacytoma in trephine biopsy confirmed the diagnosis.Here, we emphasize on autoimmune cause of hypoglycemia. BIRDEM Med J 2022; 12(1): 70-73

Diagnostic value of monospecifc DFS70 antibodies in systemic utoimmune rheumatic diseases

The detection in serum of monospecifc antibodies that induce a dense fne-speckled fluorescence when interacting with the DFS70 / LEDGF / p75 nuclear antigen is negatively associated with the development of systemic autoimmune rheumatic diseases (SARD) and increases the diagnostic specifcity of the screening study of antinuclear antibodies (ANA) using indirect immunofluorescence on HEp-2 cells (IIF-HEp-2). The results of assessing the clinical signifcance of anti-DFS70 antibodies vary depending on the test systems and the selection of patient groups. The aim of this work is to study the frequency of detection of monospecifc anti-DFS70 antibodies in blood serum in healthy individuals and patients with SARD. Sera of 74 healthy donors and 59 patients with SARD were studied (27 – systemic lupus erythematosus – SLE, 15 – Sjogren's syndrome – SjS, 17 – rheumatoid arthritis – RA). Classical antinuclear antibodies (ANA) and anti-DFS70 antibodies were determined by IIF using a mixture of standard and genetically engineered DFS70-KO HEp-2 cells that do not express DFS70 / LEDGF / p75 as a substrate. 14.9% of healthy donors and 83.1% of SARD patients (96.3% – SLE, 100.0% – SS, 47.1% – RA) were seropositive for antinuclear factor (ANF). Classical ANA with homogeneous, speckled, nucleolar, cytoplasmic, mixed types of fluorescence and the absence of anti-DFS70 antibodies were found in all ANF-positive patients with SARD and in 8.1% of healthy donors. Monospecifc anti-DFS70 antibodies without classical ANA were detected in 6.8% of healthy individuals and were absent in SARS. Among ANF-positive healthy donors, the frequency of isolated detection of anti-DFS70 antibodies was 45.5%. The detection of monospecifc anti-DFS70 antibodies can be considered as a potential predictive marker for excluding the diagnosis of SARD in ANF-positive patients with no or unclear clinical signs of these diseases.

Clinical and Histopathological Features of Scleroderma-like Disorders: An Update

Scleroderma-like disorders include a set of entities involving cutis, subcutis and, sometimes, even muscular tissue, caused by several pathogenetic mechanisms responsible for different clinical–pathological pictures. The absence of antinuclear antibodies (ANA), Raynaud’s phenomenon and capillaroscopic anomalies constitutes an important element of differential diagnosis with systemic sclerosis. When scleroderma can be excluded, on the basis of the main body sites, clinical evolution, any associated pathological conditions and specific histological features, it is possible to make a correct diagnosis.

ANA positivity and complement level in pleural fluid are potential diagnostic markers in discriminating lupus pleuritis from pleural effusion of other aetiologies

ObjectiveLupus pleuritis is the most common pulmonary manifestation of systemic lupus erythematosus (SLE). We aimed to compare various biomarkers in discriminating between pleural effusions due to lupus pleuritis and other aetiologies.MethodsWe determined in 59 patients (16 patients with SLE and 43 patients without SLE) pleural fluid levels of high-mobility group box 1, soluble receptor for advanced glycation end products (sRAGE), adenosine deaminase (ADA), interleukin (IL) 17A, tumour necrosis factor-α, antinuclear antibodies (ANA), and complements C3 and C4.ResultsWe found significant differences in the pleural fluid level of sRAGE, ADA, IL-17A, C3 and C4, and in the proportion of ANA positivity, among lupus pleuritis and other groups with pleural effusion. Specifically, ANA positivity (titre ≥1: 80) achieved a high sensitivity of 91%, specificity of 83% and negative predictive value (NPV) of 97% in discriminating lupus pleuritis from non-lupus pleural effusion. A parallel combination of the level of C3 (<24 mg/dL) and C4 (<3 mg/dL) achieved a sensitivity of 82%, specificity of 89% and NPV of 93% in discriminating lupus pleuritis from non-lupus exudative pleural effusion.ConclusionsIn conclusion, ANA, C3 and C4 in pleural fluid are useful in discriminating lupus pleuritis from pleural effusion due to other aetiologies with high NPV.

P039 Evolutionary course of Juvenile Idiopathic Arthritis

Background Juvenile idiopathic arthritis (JIA) is a heterogeneous group of rare conditions that begin before the age of 16. Seven categories have been defined by the International League of Associations for Rheumatology (ILAR) classification. The aim of our work is to describe the demographic, clinical, and radiological characteristics and therapeutic of patients with JIA. Materials and methods A retrospective, descriptive study of patients followed for AIJ in the rheumatology department of Mohamed LamineDebaghine during a period of 15 years (2005–2020). Patients were included if satisfied ILAR criteria. Results Ten patients with JIA were included, 5 of whom were females. The mean age of onset was 9.20 ± 3.15 (range: 5–14 years). The subtypes were as follows: 4 patients had RF-negative polyarthritis, one case had RF-positive polyarthritis, one with systemic arthritis form, 2 cases of oligoarthritis, 1 case of psoriatic arthritis, and enthesitis-related arthritis. Three children have coxitis. ACPA was positive in one case and antinuclear antibodies were negative. Eight patients have developed osteoarticular destruction. Pulmonary involvement was noted in a patient who had RF-positive polyarthritis. Corticosteroid therapy was prescribed in 9 patients, 5 of whom developed complications related to steroids, including growth retardation, which was observed in 3 cases. Nine JIA patients received methotrexate. The use of etanercept and IL-1 inhibitors were required in 7 and 1 cases, respectively. Corticosteroid infiltrations were performed in 9 patients. Total hip replacement was indicated in 3 patients. Conclusion Through our study, we note a predominance of the polyarticular form. Osteoarticular destruction seems to be frequent, particularly in the hip. The use of biotherapy and the total hip prosthesis proved the severity of juvenile idiopathic arthritis in our patients.