Acute pancreatitis as an initial symptom of systemic lupus erythematosus： A case report and review of the literature

Acute pancreatitis as an initial symptom of systemic lupus erythematosus (SLE) is rare. We present a report of a 46-year-old female patient who had fever, abdominal pain and vomiting, elevated pancreatic enzyme levels, hypocalcemia, hypoxemia, and various other laboratory abnormalities. She was first diagnosed with acute severe pancreatitis and then with SLE after further investigations. After a 2-mo treatment with somatostatin, the patient recovered.     

Aplastic anemia complicating systemic lupus erythematosus – report of a case and review of the literature

We report a case of osteoblastoma in the uncommon location of the L5 lumbar vertebra, detailing the clinical and radiologic aspects. Although the plain radiographs of the patient were normal, computed tomographic scans of the lumbar region confirmed the diagnosis of osteoblastoma or osteoid osteoma of the L5 vertebra. The patient was referred to the orthopedic department for operation. Histologic examination revealed osteoblastoma of the L5 vertebra. Following surgery, the patient's pain resolved completely. Although osteoblastoma is extremely rare in the spine, it should be included in the differential diagnosis as a cause of chronic back pain, especially in young males with painful scoliosis and/or radicular-type leg pain. The tumor is often not readily apparent on plain radiographs. Therefore, advanced radiological investigation is necessary to establish the correct diagnosis.     

Are RASopathies new monogenic predisposing conditions to the development of systemic lupus erythematosus? Case report and systematic review of the literature

Objective

RASopathies (Noonan syndrome (NS) and Noonan-related syndromes) are neurodevelopmental syndromes resulting from germline mutations in genes that participate in the rat sarcoma/mitogen-activated protein kinases (RAS/MAPK) pathway (PTPN11, SOS1, RAF, KRAS or NRAS, and SHOC2). Some monogenic conditions are associated with the development of systemic lupus erythematosus (SLE), and a few reports described the association of SLE with NS. We aim to search for a relationship between RASopathy and the development of SLE.

Methods

We reported for the first time a case of 13-year-old boy with NS with loose anagen hair (NSLAH) resulting from mutation in SHOC2 who developed an autoimmune disorder that fulfilled four American College of Rheumatology (ACR) criteria for the classification of SLE (polyarthritis, pericarditis, antinuclear antibodies, and anti-DNA antibodies). The case report then prompted a literature review by a systematic search for English and French articles on the subjects of RASopathies and SLE that had English abstracts in PubMed from 1966 to 2012.

Results

We identified seven additional patients with RASopathy and SLE. The male-to-female ratio was 1:1 and age at onset of SLE ranged from 5 to 32 years. The most common features were polyarthritis (7/8 patients), autoimmune cytopenia (4/8 patients), and pericarditis (4/8 patients) while only one patient presented with skin involvement.

Conclusion

The association of two rare diseases in eight patients suggests that RASopathies may be associated with the development of SLE, which is characterized by a higher male-to-female ratio, a lower rate of skin involvement, and a higher rate of pericarditis than “classic” SLE.     

Progressive disseminated histoplasmosis in systemic lupus erythematosus—an unusual presentation of acute tenosynovitis and a literature review

Progressive disseminated histoplasmosis is a disease where Histoplasma capsulatum affects multiple organs due to the inability of host cellular immunity to control the infection. Progressive disseminated histoplasmosis mainly involves the bone marrow, liver, and lungs. We report an unusual initial presentation of progressive disseminated histoplasmosis presenting as acute tenosynovitis in a systemic lupus erythematosus (SLE) patient. This report highlights the point that H. capsulatum may present as focal lesions and a high level of suspicion is needed to make the diagnosis, especially in SLE patients. We specifically reviewed reported cases of progressive disseminated histoplasmosis in SLE patients, and a review of the literature is presented.     

Kikuchi-Fujimoto’s disease associated with systemic lupus erythematosus: case report and review of the literature

Kikuchi-Fujimotos disease (KFD) or histiocytic necrotising lymphadenitis is a benign and self-limited disease, of unknown aetiology, which affects mainly young women. It presents with localised lymphadenopathy, predominantly in the cervical region, accompanied by fever and leukopenia in up to 50% of the cases. KFD has been rarely described in association with systemic lupus erythematosus (SLE), and its diagnosis can precede, postdate or coincide with the diagnosis of SLE. We present a patient with the diagnosis of SLE characterised by arthritis, leukopenia, malar rash, photosensitivity and positive ANA, besides cervical lymphadenopathy whose biopsy was compatible with KFD, which improved after using prednisone. Although the presence of lymphadenopathy is not uncommon in SLE patients, particularly in the phases of disease activity, the concomitance with KFD has rarely been reported in the literature. Its recognition is necessary because one can avoid laborious investigation for infectious and lymphoproliferative diseases.     

A case report of systemic lupus erythematosus combined with Castleman’s disease and literature review

Although lymph node enlargement is common in active systemic lupus erythematosus (SLE), lymph node examination is frequently ignored in the diagnosis of SLE. Clinical presentation and abnormal laboratory findings are often sufficient for SLE diagnosis, not to mention that the specific histological finding of lymph node necrosis in SLE is rarely seen, and the follicular hyperplasia is usually considered as nonspecific. However, since the late 1990s, a few cases of SLE lymphadenopathy have been reported exhibiting a Castleman’s disease (CD) morphology, which was discovered in lymph node biopsies. Here we report a similar case of SLE combined with CD in a 23-year-old girl who displayed systemic symptoms, including systemic lymphadenopathy and abnormal laboratory findings indicating the active phase of SLE. A biopsy of neck lymphnodes showed histopathological features of CD. The patient responded very well to the prednisolone treatment. Based on the related literature review, we would like to stress the possibility of CD in patients with SLE lymphadenopathy.     

An unusual presentation of sclerosing mesenteritis as pneumoperitoneum： Case report with a review of the literature

Sclerosing mesenteritis is a rare condition that involves the small or large bowel mesentery. An unusual presentation of this condition, which led to difficult preoperative assessment and diagnosis, is described. This report is followed by a comprehensive review of the literature.     

Left ventricular non-compaction in a juvenile systemic lupus erythematosus girl with Hashimoto’s thyroiditis: Case report and review of the literature

BackgroundLeft ventricular noncompaction (LVNC) is a structural abnormality of the left ventricular myocardium of unknown cause. Cardiovascular system involvement is an important manifestation in juvenile systemic lupus erythematosus (jSLE) and is a leading cause of morbidity and mortality.Aim of the workTo present a case of jSLE with LVNC and Hashimoto's thyroiditis.Case presentationA 15-year-old Tunisian girl with a history of Hashimoto's thyroiditis presented with paroxysmal chest pain, exertional dyspnea, headache, and polyarthralgia and was diagnosed with jSLE. Clinical examination revealed a febrile patient (39 °C). There was no tachypnea, no evidence of right heart failure, no audible murmurs on cardiac auscultation, and no edema of the lower limbs. Laboratory investigations revealed leucopenia (3500/mm³), anemia (10 g/dl), elevated erythrocyte sedimentation rate (ESR)(50 mm1^sth), C-reactive protein (20 mg/l). Infections were excluded with multiple negative blood cultures and negative anti-streptolysin O antibodies. The antinuclear antibody (ANA) was positive homogenous at 1/10,000 and both anti-native DNA and anti-SSA were positive. The electrocardiogram (ECG) showed sinus tachycardia, the transthoracic echocardiography revealed pericarditis associated with a non-compacted LV and a small pericardial effusion. Thus, a cardiac magnetic resonance imaging (CMRI) was performed confirming LVNC with a hypertrabeculated sub-endocardium. There was no proteinuria, hypocomplementemia or cerebral vasculitis on cerebral magnetic resonance imaging (MRI). The patient received high-dose corticosteroids associated with beta-blocker therapy with a favorable outcome.ConclusionA rare case of LVNC which was accidentally discovered in a patient with jSLE and Hashimoto's thyroiditis is documented. Follow-up is needed for the development possible complications.     

Systemic lupus erythematosus with simultaneous onset of Kikuchi-Fujimoto’s disease complicated with antiphospholipid antibody syndrome: a case report and review of the literature

Histiocytic necrotizing lymphadenitis, called Kikuchi-Fujimotos disease (KFD), is an idiopathic, self-limited condition rarely associated with systemic lupus erythematosus (SLE). The cause of concomitant KFD and SLE is still unknown. We describe a 19-year-old man simultaneously diagnosed with both KFD and SLE complicated with deep vein thrombosis (DVT). To the best of our knowledge, this is the first case report of KFD associated with SLE complicated with antiphospholipid antibody syndrome (APS). Our patient was successfully treated with intravenous pulse methylprednisolone, anticoagulation with heparin, oral hydroxychloroquine, azathioprine, and low-dose aspirin.     

Periodic fever as the manifestation of primary Sjogren’s syndrome: a case report and literature review

A 56-year-old male had periodic fever for 5?years and suffered from auditory hallucination and hearing impairment for 3?years. Xerostomia, xerophthalmia, elevated anti-SSA/Ro tilter, positive Schirmer’s test, and lymphocyte infiltrate of mucoserous gland in lip biopsy of this case confirmed the diagnosis of primary Sjogren’s syndrome (pSS). We review literature for fever and neuropsychiatric involvement in pSS case series. Though fever is present in 6–41?% pSS cases, periodic fever has not been reported. Auditory hallucination was rare in cases with pSS. The literature review alerts clinicians that fever and neurological manifestations were not uncommon in pSS cases.     

Transient myopia with severe chemosis as an initial manifestation of systemic lupus erythematosus

A 24-year-old woman suffered from blurred vision and periorbital edema with remittent fever. She was diagnosed as having systemic lupus erythematosus (SLE), complicated with myopia and retinopathy and severe chemosis. Antiphospholipid syndrome (APS), hemophagocytic syndrome, and liver involvement were also proven. We considered that APS might cause chemosis as a result of thrombosis-induced perfusion failure in the conjunctiva. In such cases, APS should be considered and anticoagulation therapy associated with steroid therapy should be initiated. In systemic lupus erythematosus (SLE), chemosis, severe hepatitis, and hemophagocytic syndrome (HPS) are rare complications. It is well known that many cases of SLE are complicated with antiphospholipid syndrome (APS), which causes arteriovenous thrombosis. We report a case of SLE with transient myopia and severe chemosis complicated with severe hepatitis and HPS. As this patient had antiphospholipid antibodies, these ocular complications were considered to be related to APS. Received: August 23, 2000 / Accepted: November 24, 2000     

MicroRNA-126 and 146a as potential biomarkers in systemic lupus erythematosus patients with secondary antiphospholipid syndrome

BackgroundMicroRNAs (miRs) are noncoding gene regulators that may have a role as diagnostic or prognostic biomarkers in systemic lupus erythematosus (SLE) and its complications. SLE is an autoimmune disease that may be associated with secondary antiphospholipid syndrome (APS).Aim of the workTo evaluate the plasma levels of both miR-146a and miR-126 as well as serum alpha interferon (α IFN) in Egyptian SLE patients with and without secondary APS and to investigate their potential role in disease pathogenesis and their utility as biomarkers for APS.Patients and methods88 SLE patients including 30 cases with secondary APS and 40 matched healthy individuals were enrolled in this study. SLE disease activity index (SLEDAI) was assessed. The plasma levels of miR-146a and miR-126 were determined by Realtime polymerase chain reaction (PCR) in all participants.ResultsThe mean age of the patients was 31.3 ± 9.6 years with disease duration 1–17 years. Plasma miR-146a was significantly lower and miR-126 significantly higher in SLE compared to controls. MiR126 was also higher in secondary APS patients compared to patients without. Serum IFN-α ws significantly higher in patients (71.2 ± 19.7 pg/ml) compared to control (43.2 ± 9.7 pg/ml) (p < 0.001). MiR-126 at a cut off of 2.66 can discriminate between SLE patients with and without secondary APS with a sensitivity of 76.67% and specificity of 81.01% ((95% CI 0.685–0.902, P < 0.001).ConclusionCirculating miR-126 could be a potential noninvasive biomarker in SLE associated with secondary APS. Further studies are needed in view of the limited data on the expressions of microRNA in APS.     

Systemic lupus erythematosus,celiac disease and antiphospholipid antibody syndrome: a rare association

Association of celiac disease with systemic lupus erythematosus is rare, even though HLA B8 and DR3 are commonly associated with these diseases. We read with great interest a similar case reported by Hrycek and Siekiera in this journal and wish to highlight another case of ours, which had celiac disease, systemic lupus erythematosus and antiphospholipid antibody syndrome, an association which has never been described before.     

Impact of antiphospholipid syndrome on disease characteristics and outcome in patients with systemic lupus erythematosus

Aim of the workTo assess the impact of antiphospholipid syndrome (APS) on systemic lupus erythematosus (SLE) clinical characteristics and disease outcome.Patients and methods216 SLE patients were classified according to the presence of APS into: SLE group (n = 109) and SLE-APS (n = 107). Evaluation of clinical, laboratory tests, immunological tests, SLE disease activity index 2000 (SLEDAI-2 K) and SLE damage index (SDI) were done.ResultsThe 216 patients mean age was 32.1 ± 8.4 years and the F:M was 8.4:1. There was a significantly higher frequency of abortion, neurological and cardiac manifestations (p < 0.001, p = 0.008, p = 0.016 respectively) in SLE-APS group. The SDI was significantly higher in SLE-APS patients (p < 0.001) with special attention to the presence of cerebrovascular accident (CVA) (p = 0.01), pulmonary hypertension (p = 0.04), cardiomyopathy (p = 0.034) and venous thrombosis (p < 0.0001). The frequency of thrombosis and active SLEDAI-2 K visits were significantly related to higher damage in SLE-APS (p < 0.001 and p < 0.001 respectively). Damage in SLE group was associated with hypocomplementemia (p = 0.015) and thrombosis (p = 0.049). Factors associated with damage in all SLE patients were male gender (p = 0.024), serositis (p = 0.02), neurological involvement (p < 0.001), thrombotic events (p < 0.001), cumulative doses of oral (p = 0.02) and pulse (p = 0.004) steroids and frequency of cyclophosphamide (CYC) use (p = 0.003). Predictors of damage included male gender, APS, neurological manifestations, use of steroids and CYC (p = 0.045, OR = 8.5; p < 0.0001, OR = 4.3; p = 0.001, OR = 6.3; p = 0.047, OR = 1.03; p = 0.005, OR = 2.96, respectively).ConclusionAPS adversely affect SLE disease course with prominent impact on end organ damage especially cerebral, cardiopulmonary and vascular events denoting the need for strict control of disease activity and early diagnosis.     

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.     

Reversible dementia in systemic lupus erythematosus without antiphospholipid antibodies or cerebral infarction

Dementia is a very rare neurological manifestation of systemic lupus erythematosus (SLE) and has a deep link with antiphospholipid antibodies (APL) and cerebral infarction in its development. However, nonvascular dementia irrelevant to APL or cerebral infarction has not been reported in patients with SLE until now. We describe a case of reversible dementia in an SLE patient without APL or cerebral infarction which was successfully treated with corticosteroid and cyclophosphamide. There are two significant points in this case. One is that humoral factors other than APL might be involved in the development of dementia. Secondly, reversible dementia without APL or cerebral infarction may respond more favorably to immunosuppressive therapy.     

Histopathological analysis of cerebral hemorrhage in systemic lupus erythematosus complicated with antiphospholipid syndrome

Abstract

A 53-year-old woman was admitted due to akinetic mutism and fever. On admission, brain computed tomography (CT) scan revealed a large hemorrhagic lesion in the left anterior lobe that required surgical operation for total removal. Her neuropsychiatric manifestation was not attenuated after the operation, and was gradually ameliorated by high doses of corticosteroid thereafter, suggesting a diagnosis of lupus psychosis. Histopathological examination revealed a necrotizing vasculature, sometimes with a disrupted lamina elastica interna, completely filled with thrombi and infiltrated with inflammatory cells and their debris. It is therefore suggested that the large frontal lobe hematoma in our patient was induced by thrombosis and arteritis in the subarachnoid space.     

Erythematosus plaques with macrophage infiltration as an initial manifestation of macrophage activation syndrome in a patient with systemic lupus erythematosus

The diagnosis of macrophage activation syndrome (MAS) in patients with systemic lupus erythematosus (SLE) may be challenging as it can mimic the clinical features of the underlying disease or be confused with an infectious complication. In this report, a Japanese woman in her forties had diverse clinical features of MAS at initial presentation of SLE, where erythematosus plaques with histiocytic infiltrates focally surrounding degenerated collagen might be the earliest indicator of MAS.     

Pregnancy in systemic lupus erythematosus and antiphospholipid syndrome

Systemic lupus erythematosus (SLE) is the autoimmune disease that most commonly compromises pregnancy. Moreover, the relationship between SLE and pregnancy is in both directions. However, the current experience indicates that pregnancy in patients with SLE should not be regarded as an unacceptable high risk condition for the mother or her baby provided that careful planning of conception and multidisciplinary monitoring and treatment are carried out.