First Presentation Of Systemic Lupus Erythematosus Research Articles

Life threatening macrophage activation syndrome as the initial presentation of systemic lupus erythematosus: A case report and review of the literature

BackgroundMacrophage activation syndrome (MAS) is characterized by activation of hemophagocytic cells and is a very rare initial manifestation of systemic lupus erythematosus (SLE). Aim of the workTo describe the features of a challenging case of SLE initially presented with MAS. Case reportA previously healthy 20-year-old female was referred to the Rheumatology clinic with a 25-days’ history of persistent fever, general fatigue, and significant weight loss. Clinical examination revealed a high-grade fever (41 °C), significant hair loss and general malaise. Laboratory investigations revealed leucopenia (1.5 × 109/L), neutropenia (0.7 × 109/L), lymphopenia (0.6 × 109/L), normocytic normochromic anemia (9 g/dL), thrombocytopenia (95 × 109/L), elevated liver enzymes, hyperbilirubinemia (2.6 mg/dl); hypertriglyceridemia (306 mg/dL), hyperferritinemia (16000 ng/mL), elevated D-Dimer (4.7 mg/L), elevated lactate dehydrogenase (1379 U/L) and direct and indirect Coombs' tests were positive. Erythrocyte sedimentation rate was 28 mm/1st h. Immunological screening was positive for antinuclear antibody (ANA) and anti-double stranded deoxyribonucleic acid (anti-dsDNA). Serum C3 and C4 complement factors were low and C reactive protein was negative. Soluble CD25 was elevated (9800 pg/ml). Bone marrow biopsy showed hypocellular bone marrow with normal megakaryocytes and erythroid series with evidence of hemophagocytosis. A diagnosis of SLE initially presented with MAS was made. The patient received intravenous methylprednisolone for 5 consecutive days followed by prednisolone and cyclosporine A and intravenous immunoglobulin. The condition deteriorated with progressive coagulopathy, thrombocytopenia and hyperferritinemia. Subsequently, the conscious level of the patient deteriorated, multiorgan failure developed and the patient died. ConclusionMAS is a serious condition that may be the first presentation of SLE.

P35 Lupus masquerading as Cholangiocarcinoma: One of the many faces of multifaceted lupus

Introduction/BackgroundSystemic lupus erythematosus (SLE) is a complex autoimmune condition which can present with a wide variety of clinical features. This variation can lead to delayed diagnosis or misdiagnosis. Gastroenetrological pathology in SLE is rare but primary autoimmune hepatitis, pancreatitis, lupus enteritis, cholestatic biliary disease and protein losing enteropathy have all been reported. We would like to share the first known presentation of SLE as cholangiocarcinoma.Description/MethodA 62-year-old, previously fit and well, gentleman presented with a 1 month history of painless jaundice, a palpable gallbladder (Courvoisier syndrome), dark urine, pale stool, generalised rash, pruritus, weight loss and anorexia. The presence of these red flag features raised concerns. Initial investigation with Endoscopic retrograde cholangiopancreatography (ERCP) revealed distal common bile duct (CBD) stricture which was further explored with computerised tomography of thorax, abdomen, pelvis and magnetic retrograde cholangiopancreatogram (MRCP) both raising the suspicion of a cholangiocarcinoma in the presence of constitutional symptoms. Several biopsies and brushings taken during multiple ERCP’s failed to identify clear mitotic cells, IgG 4 staining remained negative. Further investigation with Positron emission tomography – computerised tomography (PET-CT) showed significant FDG uptake around the upper part of the stent with final working diagnosis of Cholangiocarcinoma and a Whipple’s procedure was planned.In the interim, the patient developed swollen painful hands, scleritis and a yellow blistering rash. He was referred urgently onto the Rheumatology team after receiving the positive immunology results: ANA (1:1280), Anti double stranded DNA and Rheumatoid Factor (RF) along with hypocomplementemia (C3&C4).Discussion/ResultsSubsequent work up under rheumatology was done considering the possibilities of diagnosis of Systemic lupus erythematosus (SLE), Lupus related autoimmune hepatitis and reactive arthritis as a result of underlying malignancy. Patient’s immunology and other clinical features such as skin rash were strongly supportive of SLE as a primary diagnosis. He was initially treated with low dose prednisolone with dramatic improvement in arthritis and gradual improvement in liver function test. Following MDT discussions with the hepato-biliary team it was felt that lupus was the cause of the biliary stricture which settled well with steroid treatment. A final ERCP was arranged to remove the stent and take final brushings. Methotrexate was started at this point. His SLE remains well controlled on methotrexate and his liver function tests, including bilirubin, remain within the normal range. Patient has now been completely asymptomatic and is discharged from the care of hepatobiliary team with long term follow up for his SLE with rheumatology.Key learning points/ConclusionThis is extremely rare first presentation of SLE presenting with painless jaundice and biliary stricture which improved both biochemically and clinically with treatment of SLE. The key learning points included:Urgent review by rheumatology can facilitate timely diagnosis and preventable admissions/interventions such as major intraabdominal procedure in the form of whipple’s disease planned in our case.Cancer remains a major cause of mortality and morbidity in rheumatological conditions like SLE. Hence initial diagnosis can become extremely difficult if an overlap between these two happens.Treatment options can be limited initially and steroids remain the main stay of treatment till mitotic lesions have been ruled out and disease modifying agents can be used without any contraindications. But disease control for our conditions remains challenging in presence of cancers.

Predictors of Mortality in Pulmonary Haemorrhage during SLE: A Single Centre Study Over Eleven Years.

BACKGROUND:Pulmonary haemorrhage (PH) is a serious complication during Systemic Lupus Erythematosus (SLE).AIM:The aim was to present data on 12 patients of SLE with classic symptoms and signs of PH admitted throughout eleven years.METHODS:This retrospective study was carried out at King Abdul Aziz Specialist hospital in Taif-a tertiary care hospital in the western region of Saudi Arabia. The data was analysed from the case files of SLE patients who had episodes of PH throughout 11 years (January 2007 to December 2017).RESULTS:Twelve patients (10 females and 2 males) were found to have diffuse pulmonary haemorrhage during their SLE in the study period. Of 12 patients with confirmed pulmonary haemorrhage (hemoptysis, hypoxemia, new infiltrates on chest radiography, fall in haemoglobin and hemorrhagic returns of bronchoalveolar lavage with hemosiderin-laden macrophages) 4 patients had PH as the first presentation of SLE and 8 patients developed this complication during the disease. All patients presented with shortness of breath and hemoptysis. The most common extra-pulmonary involvement in the study cohort was renal (83%), which ranged from clinical nephritis, nephrotic syndrome to acute renal failure. All patients were managed in intensive care of the hospital, and of 12 patients, 9 (75%) required mechanical ventilation. All patients were uniformly treated with pulse Methylprednisolone; 9 received Cyclophosphamide, 6 received IVIG, and 4 received Plasmapheresis. Only 3 patients (25%) survived despite maximum possible support during their mean hospital stay of 18 ± 5 days.CONCLUSION:The requirement of mechanical ventilation and the association of renal and neuropsychiatric complications predicted mortality in patients with pulmonary haemorrhage.

A Rare Case of Macrophage Activation Syndrome Presenting as the First Manifestation of Systemic Lupus Erythematosus.

Macrophage activation syndrome (MAS) itself is a rare, potentially life-threatening complication of a rheumatic disease, mostly seen in juvenile idiopathic arthritis. It infrequently occurs in systemic lupus erythematosus (SLE), and it is extremely rare to be the first presentation of SLE. In a study of 511 patients with SLE, 7 cases (1.4%) of MAS were identified. In all the cases, MAS was simultaneous to the presentation of SLE in this article, we report a case of a patient with MAS who presented with fever, rash, and high ferritin level up to 16911 ng/mL. A high degree of suspicion is required that high fever and rash can be clues to MAS. Early diagnosis is necessary since mortality rates remain high for untreated cases.

Pyoderma Gangrenosum in a Patient With Systemic Lupus Erythematosus: Case Report

Pyoderma gangrenosum (PG) is a rare and unusual manifestation of systemic lupus erythematosus (SLE). It deserves special mention whenever a case of PG is seen in any patient population, due to its rarity. It has an unusual association with SLE, with few documented cases. A case of a 53-year-old male patient, who had an unusual first presentation of SLE in 1995, with mainly hematological and renal manifestations of SLE and with a lack of any dermatological or musculoskeletal features until 2012 is presented here. The patient developed two ulcerating lesions on right leg and was diagnosed with PG following biopsy, which was successfully treated with corticosteroids and appropriate wound management. J Med Cases. 2014;5(8):455-458 doi: http://dx.doi.org/10.14740/jmc1845w

A Teenager Diagnosed as Lymphocytic Hypophysitis Associated with Systemic Lupus Erythematosus (SLE)

A 15-year-old female was admitted to our hospital with intermittent fever, cough and sputum for one week, and diagnosed as pneumonia. Because of no clinic remission with antibiotic and antiviral treatment for one week and following with facial erythema, severe anemia, and neutropenia, appropriate screening laboratory examinations showed severe lower complements (C3, C4) and positives autoantibody such as ANA, ds DNA, Anti-Sm etc, Therefore, SLE was diagnosed with muti-organs involved including lungs, blood, liver, kidney, medical peritonitis, and central nervous system presented as irritable and moody. Because the patient complained of dizziness, fatigue, anorexia, hands trembling and showed a very indifferent mood, endocrinological tests were took. The results revealed very low levels of sex hormones, thyroid hormones and serum cortisol, and manifested as hypopituitarism. Magnetic resonance imaging (MRI) of pituitary region demonstrated a mild enlargement of the pituitary stalk. SLE associated with Lymphocytic hypophysitis (LYH) was suspected. And the clinical symptoms and laboratory tests were ameliorated after glucocorticoid treatment (involve twice methylprednisolone (MPIV) pulse therapy and oral 40 mg/d of MPIV) combined with cyclophosphamide therapy. Although, LYH associated with SLE have been reported, a 15-year teenager, as seen in this case, is a rare first presentation of SLE.

Risk factors of mortality for salmonella infection in systemic lupus erythematosus.

To investigate the risk factors of mortality for Salmonella infection in patients with systemic lupus erythematosus (SLE). Between 1995 and 1999 we reviewed 37 cases of Salmonella infection in 31 patients with SLE from a total of 1191 hospitalized patients with SLE at a medical center in Taiwan. Contrasting cases of patients who died with those who survived, we compared clinical and laboratory characteristics of SLE at the time of Salmonella infection, with special attention to potential risk factors (sex, age, complete blood count and differential count, erythrocyte sedimentation rate, C-reactive protein, complements, Salmonella species, infection site, reinfection, SLE presenting with Salmonella infection, associated non-Salmonella infection, etc.). The mean age at the onset of SLE in the 8 mortality cases was significantly higher than the 23 cases of survivors (p < 0.05). Other factors significantly related to death included associated infections other than Salmonella species, reinfection of Salmonella species, and cases of SLE presenting with Salmonella infection. Reinfection and SLE presenting with Salmonella infection were the most important risk factors of mortality for SLE with Salmonella infections: relative risk (CI) 84 (4.3-1638.8) and 63 (3.1-1296.5), respectively. Patients with SLE who are older or have associated infections other than Salmonella have an increased mortality rate when they have concurrent Salmonella infection. Patients with Salmonella infection occurring concurrently with the first presentation of SLE and patients with SLE reinfected with Salmonella species are at higher risk of mortality.