Acute aortic valvulitis as an initial presentation of systemic lupus erythematosus.

Valvular involvement in patients with systemic lupus erythematosus (SLE) is not uncommon but patients rarely present with it. The mitral valve is most commonly involved. We report a 36-year-old man who had an episode of acute fever, arthritis, and acute aortic insufficiency with a small vegetation at the tip of the aortic valve mimicking infective endocarditis, proven later to be due to SLE. SLE should be considered as one of the uncommon causes of acute aortic insufficiency.     

Silent renal involvement in systemic lupus erythematosus.

20 patients with active SLE without clinical evidence of renal involvement underwent percutaneous renal biopsy. 12 had varying proliferative changes on light microscopy. Of 19 ultrastructural examinations performed only 3 had no electron-dense deposits. Serum C3 and C4 levels were 63 +/- 8 and 8 +/- 2 mg% in patients with subendothelial deposits, compared to 142 +/- 27 and 27 +/- 6 mg%, respectively, in patients without deposits (p less than 0.01). All patients with diffuse proliferative changes had subendothelial deposits; however, one with normal light microscopy and another with focal proliferation also had them. It is concluded that no variant of lupus nephropathy can be excluded on clinical grounds alone.     

Neonatal lupus erythematosus with cardiac involvement in offspring of mothers with experimental systemic lupus erythematosus

Neonatal lupus erythematosus (NLE) syndrome is characterized by a transient dermatitis, a variety of systemic and hematological abnormalities, and isolated cases of congenital complete heart block. The latter has been reported to be due to the presence of autoantibodies specific to La (SS-B) and/or Ro (SS-A). As female mice with experimental systemic lupus erythematosus (SLE) induced by immunization with the human monoclonal anti-DNA antibody bearing the 16/6 Id produce variety of autoantibodies including anti-Ro and anti-La antibodies, we looked for NLE related symptoms in the murine model. Offspring of BALB/c mice with SLE possessed high levels of autoantibodies that declined gradually till reduced to normal levels at day 60 after delivery. Electrocardiograms recorded in groups of offspring from mothers with experimental SLE indicated that a high percentage of the offspring had defects in their conductive system including first-, second-, and third-degree heart block, significant bradycardia, and a wide QRS complex. In contrast, a normal pattern was observed in offspring of healthy mothers.     

Neuropsychiatric involvement in systemic lupus erythematosus: A review

The neuropsychiatric involvement in systemic lupus erythematosus (NPSLE) is a challenge for clinicians, both at a diagnostic and therapeutic level. Although in 1999 the American College of Rheumatology (ACR) proposed a set of definitions for 19 NPSLE syndromes, with the intention of homogenizing the terminology for research purposes and clinical practice, the prevalence of NPSLE varies widely according to different series and is estimated to be between 37 and 95%. This is due to multiple factors such as the unalike definitions used, the diverse design of the studies, type of population, race, type and severity of symptoms, and follow-up of the different cohorts of patients with SLE. In recent years, some authors have tried excluding minor neuropsychiatric manifestations in order to try to reduce this wide variation in the prevalence of NPSLE since they are very prevalent in the general population; others authors have developed various models for the attribution of neuropsychiatric events to SLE that can assist clinicians in this diagnostic process, and finally, some authors developed and validated in 2014 a new algorithm based on the definitions of the ACR that includes the evaluation of the patient's lupus activity together with imaging techniques and the analysis of cerebrospinal fluid (CSF), with the aim of trying to differentiate the true neuropsychiatric manifestations attributable to SLE. In 2010, the European League Against Rheumatism (EULAR) developed recommendations for the management of NPSLE. We found abundant literature published later where, in addition to the recommendations for the management of the 19 NPSLE syndromes defined by the ACR, additional recommendations are given for other neurological and/or psychiatric syndromes, conditions, and complications that have been associated to SLE in recent years. We review below the diagnostic and therapeutic management of the different entities.     

Predictors of renal involvement in patients with systemic lupus erythematosus

From a cohort of 109 patients (105 females and 4 males) treated for systemic lupus erythematosus (SLE), 20 patients (18.3%) developed new episodes of lupus nephritis and 89 patients (81.7%) remained free of renal involvement during the follow-up period. The mean duration of follow up was 39.1 +/- 54.4 months. Clinical characteristics associated with developing lupus nephritis were a high systolic blood pressure (> or = 130 mmHg), photosensitivity, cutaneous vasculitis and gastrointestinal (GI) symptoms. Laboratory abnormalities associated with the development of lupus nephritis were hemoglobin < 10 mg/dl, hematocrit < 30%, blood urea nitrogen > 12 mg/dl, serum creatinine > 1.3 mg/dl, ESR > 60, the third component of complement (C3) level < 0.45 and positive antidsDNA antibody. After a multivariable analysis, only high systolic blood pressure, cutaneous vasculitis, hemoglobin < 10 mg/dl and serum creatinine > 1.3 mg/dl remained as statistically significant risk factors for developing lupus nephritis.     

Respiratory involvement in systemic lupus erythematosus. A clinical and immunological study.

Thirty patients fulfilling conventional criteria for systemic lupus erythematosus and who presented with extensive pleural and pulmonary involvement were studied retrospectively. Four overlapping patterns of respiratory disease were identified and observations were made on their clinical presentation, radiographic abnormalities and response to treatment. A low incidence of severe renal disease was found in this series of patients and this was in keeping with the general finding of low serum binding using native DNA in a globulin Farr-binding technique (greater than 20% binding in only 4/21 (19%) of the series) and normal or elevated serum complement (C3) levels. Precipitating antibody detected by double diffusion and counter-current immunoelectrophoresis and probably reacting in most cases with single-stranded DNA was, however, detected in 66% of pretreatment serum samples tested. This evidence supports the idea that different types of anti-nuclear antibody may be associated with different clinical manifestations seen within a group of patients who broadly fulfil the criteria for SLE.     

A case of lupus erythematosus profundus followed by systemic lupus erythematosus presenting with severe intestinal involvement]

A 52-year-old female visited the outpatient department of Sapporo Medical University hospital in 1984 due to a refractory rash on the skin of the trunk. Histological findings of a skin biopsy specimen indicated a diagnosis of lupus erythematosus profundus (LEP). The eruption remained quiescent with moderate doses of prednisolone. In October 2003, she was suddenly admitted to our hospital with abdominal pain, fever and bloody stool. Lupus enteritis was diagnosed based on an elevated level of anti-DNA antibody, low complementemia and diffuse edematous change of the intestinal walls on CT scans. Although high doses of corticosteroids resulted in transient improvement, melena developed again on the 24th hospital day. Colonoscopy revealed deep ulceration at the rectum and a gastrografin enema indicated perforation. Accordingly, the involved rectum was resected and an artificial anus was constructed on the 50th hospital day. Examination of the resected specimen by microscopy showed that the ulceration approached at the depth of the subserosal layer with intense infiltration of inflammatory cells around the vessels. The pathogenesis of the rectal lesion might have been due to vasculitis associated with systemic lupus erythematosus (SLE). The disease did not recur under the administration of 10 mg of prednisolone daily until November 2004. Colonic function made a remarkably untroubled recovery after the artificial anus was closed in February 2005. The LEP was generally accompanied by a mild form of SLE. This case seemed to be rare in that SLE was associated with severe lupus enteritis and a refractory rectal ulcer developed from LEP. Patients with SLE and intestinal involvement should be carefully monitored in cooperation with a surgeon.     

Cardiopulmonary involvement in pediatric systemic lupus erythematosus: a twenty-year retrospective analysis.

BACKGROUND AND PURPOSE: Cardiovascular and pulmonary involvement is frequent among patients with systemic lupus erythematosus (SLE). It is important that the frequency and characteristics of pulmonary and cardiovascular involvement in childhood-onset SLE are understood. Thus, we conducted a retrospective analysis of childhood-onset SLE at a tertiary medical center in Taipei. METHODS: Children with SLE diagnosed at the National Taiwan University Hospital between 1985 and 2004 were evaluated by chart review. Records included the age at diagnosis, gender, family history, presenting manifestations with American Rheumatism Association criteria and initial laboratory data, other associated complications and duration of follow-up. RESULTS: A total of 157 cases were included. The male-to-female ratio was 18:82, with the mean age at diagnosis 12.2 years. Overall, pulmonary and cardiovascular involvements were recorded in 89 patients (56.7%) and 75 patients (47.8%), respectively. Among the more frequent lung disorders were pneumonia treated under hospitalization (in 36.9% of patients), increased pulmonary interstitial marking or infiltration (35.0%), and pleuritis (33.1%). The more common cardiovascular manifestations included cardiomegaly (in 33.8%), pericarditis (28.7%) and arrhythmia/conduction anomaly (12.7%). CONCLUSIONS: The frequencies of pulmonary and cardiovascular complications were high. Blood creatinine >1 mg/dL, hematuria and anemia with hemoglobin <12 g/dL obtained at diagnosis of SLE were associated with cardiovascular complications during the disease course, while anti-double stranded DNA at diagnosis was associated with pulmonary complications.     

Levamisole in systemic lupus erythematosus.

Two patients with systemic lupus erythematosus complicated with lupus nephritis were treated with levamisole, an immunomodulator. Clinical features, including urinary protein excretion and creatine clearance, were restored in one patient, also immunoparameters such as ANA, Ig-bearing cell number and PHA-skin test. The other patient did not respond to levamisole but did respond to cyclophosphamide.     

Psychiatric symptoms as features of systemic lupus erythematosus

An enquiry of the Dutch Lupus Patients Society revealed that 49% of the members had experienced psychiatric symptoms before systemic lupus erythematosus (SLE) was diagnosed and 33% had sought professional psychiatric help at that stage. To determine the frequency of SLE on psychiatric admission 2,121 patients were tested for the presence of SLE-specific antinuclear and anti-DNA autoantibodies. The results suggest that SLE is a cause of admission in psychiatric hospitals at the rate of 0.1-0.2% and routine screening of antinuclear and anti-DNA antibodies on admission is not an effective diagnostic approach.     

OPD4-positive T-cell lymphoma of the liver in systemic lupus erythematosus.

Primary malignant lymphoma of the liver occupying the right lobe, 14 x 9 x 7 cm in size, developed in a 30-year-old man with a 4-year history of autoimmune hemolytic anemia. The diagnosis of systemic lupus erythematosus (SLE) accompanying thrombocytopenia had been made clinically 10 months earlier. The liver biopsy specimen revealed diffuse proliferation of large lymphoma cells expressing the activated helper/inducer T-cell phenotype (LCA+, UCHL1+, OPD4+, LN3+, MT1-, L26-, MB1-, Leu M1-, Ki-1-, KP1-). The lymphoma was successfully treated by chemotherapy and irradiation. Intractable thrombocytopenia provoked fatal esophageal hemorrhage. At autopsy, no lymphomatous lesion was identified, and the hepatic right lobe contained an encapsulated necrotic lesion without any viable tumor cells. The bone marrow revealed marked hyperplasia of erythroid and megakaryocytic series. Extramedullary hematopoiesis was demonstrated in the liver, spleen and lymph nodes. This is the second case of primary hepatic T-cell lymphoma associated with SLE.     

Ultrastructural lesions in kidneys with minimal involvement by systemic lupus erythematosus

Summary We report observations of three bronchial carcinoids, using optical, UV fluorescent and electron microscopy. The scattered autofluorescent and silver reducing cells observed in these tumours are mastocytes and not tumour cells.

---

Zusammenfassung Bericht über 3 bioptisch gewonnene Bronchus-Carcinoide, welche lichtmikroskopisch, UV-Fluorescenz-mikroskopisch und elektronenoptisch untersucht werden. In 2 Fällen können zahlreiche, im Geschwulstgewebe verteilte, granulierte Zellen nachgewiesen werden, deren Granula eine positive Reaktion nach Masson-Hamperl zeigen. Dieselben Zellen lassen außerdem eine wechselnd ausgeprägte Autofluorescenz im UV-Licht erkennen. Nach Entfernung der Silbergranula und Färbung der gleichen Präparate mit metachromatischen Lösungen sind diese Zellen vorwiegend orthochromatisch. Die Ultrastruktur der Granula unterscheidet sich eindeutig von den Einschlüssen typischer Carcinoid-Zellen; es handelt sich um teils homogene, teils lamellär gestreifte Strukturen. An Hand der lichtoptischen, fluorescenzmikroskopischen und ultrastrukturellen Befunde werden die Elemente als Mastzellen bezeichnet, die in einer unreifen (orthochromatische, homogene Granula) und einer reifen (metachromatische, differenzierte Granula) Form vorliegen. Es kann angenommen werden, daß diese Zellen eine Beziehung zur Serotonin-Produktion in den Carcinoid-Zellen haben und deshalb nicht als eigentliche Geschwulstzellen interpretiert werden dürfen.

     

Lung involvement in systemic lupus erythematosus (SLE)--own experience

Pulmonary disorders in systemic lupus erythematosus are frequent and sometimes they are the first symptoms of the disease. SLE may cause a variety of clinical presentations and pathologic patterns, which can be difficult to diagnose. We observed 11 patients (9 women and 2 men) with pulmonary manifestations of SLE during last 18 years in our department. Mean age of patients was 47.7 +/- 13.4 years. There were no patients with drug induced SLE. Interstitial lung diseases (7/11) acute or chronic and pleural involvement (5/11) were the most frequent clinical presentation. In three cases airway disease presented as reduction of FEV1%VC index was detected. In one case "shrinking lung" syndrome was confirmed by muscle function (diaphragm relaxation time) and lung function tests. Pulmonary hypertension confirmed by echocardiography, was associated with interstitial lung disease or vasculitis. Six cases representing different lung involvement pattern of SLE were described in details.     

Childhood-onset systemic lupus erythematosus

OBJECTIVES: To describe the initial clinicolaboratory manifestations and short-term outcome in a series of Nigerian children with systemic lupus erythematosus (SLE). METHODS: A nonrandomized prospective study of consecutive cases of childhood-onset SLE. Baseline and follow-up clinicolaboratory data were collected and analyzed. Each patient was followed up for 12 months. RESULTS: Eleven children were studied. There were seven girls (F:M, 1.75). Mean ages at lupus onset and diagnosis were 10.0 +/- 2.53 years and 11.2 +/- 2.53 years, respectively. Mean time at onset of renal disease following SLE symptoms onset was 1.22 +/- 0.93 years. All cases were misdiagnosed prior to presentation; diagnosis was delayed in nine patients. Lupus activity was mild, moderate and severe in two, five and four patients, respectively. Hypertension (n = 5), nephrotic syndrome (n = 6), microerythrocyturia (n = 6) and acute renal failure (n = 7) were associated morbidities. Of the 27 presenting clinical features, 17 were nondiagnostic, while 10 were diagnostic. Fever (n = 9) was a major nondiagnostic symptom; major diagnostic manifestations were lupus nephritis (n = 11), arthritis (n = 10) and serositis (n = 7). Catastrophic antiphospholipid syndrome was diagnosed in three. The glomerular lesions were nonproliferative (n = 1), focal (n = 3) and diffuse (n = 7) proliferative lupus nephritis. Complete remission rate at end-point was 71.4%. Fourteen percent of the patients relapsed. Renal survival and mortality rates were 86.0% and 30.0%, respectively. CONCLUSION: In this study, severe renal and extrarenal comorbidities were common; mortality rate was also high. High frequency of misdiagnosis and delayed diagnosis were probably responsible for these.     

Neonatal lupus erythematosus in offspring of mothers with experimental systemic lupus erythematosus.

Neonatal lupus erythematosus (NLE) syndrome is a result of the transfer of autoantibodies produced by the mother, across the placenta, to the fetus. NLE is characterized by a transient dermatitis, a variety of systemic and hematological abnormalities, and isolated cases of congenital heart block. The latter has been reported to be due to the presence of autoantibodies specific to La (SS-B) and/or Ro (SS-A). As female mice with experimental SLE, induced by immunization with the monoclonal anti-DNA 16/6 Id, produce a variety of autoantibodies including anti-Ro and anti-La antibodies, we examined the relevance of NLE in the murine system. Offspring of SLE-afflicted BALB/c mothers possessed antibody titers to the 16/6 Id, ssDNA, and nuclear extract, which gradually declined until reduced to normal levels by day 60 after delivery. Antibody titers in the sera of the mothers remained elevated throughout this period. Electrocardiograms were recorded from groups of neonates from mothers with experimental SLE. The results indicated that a high percentage of the offspring had defects in their conduction system including first, second, and third degree heart block; significant bradycardia; and wide QRS complex. Normal patterns were observed in offspring of healthy mothers. Experiments done with mice that were exposed to SLE-related autoantibodies early in their development indicated that offspring to mothers with experimental SLE were neither protected nor more susceptible to disease induction by the 16/6 Id.