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PubMed1995

Pulmonary involvement in systemic lupus erythematosus.

Pulmonary disorders in systemic lupus erythematosus involve a variety of clinical presentations and pathologic patterns, which can be difficult to diagnose due to systemic dysfunction, infection, or complications of therapy. The causes of dyspnea in systemic lupus erythematosus are multifactorial, and the clinical manifestations of lung disease widely vary. Biopsy is frequently relied on to evaluate and diagnose pulmonary disease in systemic lupus erythematosus. The patient who has systemic lupus erythematosus-associated lung disease is effectively treated with various immunosuppressive drugs, in conjunction with careful evaluation of the patient's systemic involvement, drug-induced complications, and the ever-present threat of infection.

Adult↗
PubMed2002

Lupus erythematosus panniculitis: a clinicopathologic study.

BACKGROUND: Lupus erythematosus panniculitis is a clinical variant of lupus erythematosus which involves the deep dermis and the subcutaneous fat. The purpose of this study was to ascertain the clinical profile of Asian patients with this condition. METHODS: This was a retrospective study of all histologically confirmed lupus panniculitis seen at our center between 1992 and 1997. The age, sex, past history/subsequent diagnosis of systemic lupus erythematosus (SLE), presence of clinical discoid lupus erythematosus (DLE) changes on overlying skin, direct immunofluorescence, serologic, and histologic findings were analyzed. RESULTS: There were 12 cases of lupus panniculitis, two of which were in patients already diagnosed with SLE and one in which the patient subsequently evolved into SLE. The mean age at diagnosis was 31.3 years. The face (50%), upper limbs (33%), and scalp (25%) were the most common sites of involvement. Thirty-three per cent had clinical evidence of DLE on the overlying skin, whilst 67% had histologic features of DLE on the overlying skin. A lupus band was present in 36%. Antinuclear antibody (ANA) was positive in three of 11 cases; these were in the two patients who already had SLE and in the only patient who progressed to SLE. All of the cases showed fat necrosis and, in the majority of cases, there was associated lobular and paraseptal inflammation. Thirty-three per cent showed lymphocytic vasculitis and 75% had mucin deposition. None had lymphoid nodules, subepidermal hyalinization, or calcification. CONCLUSIONS: Lupus panniculitis affects a younger age group in Asians as compared with the Western population. Although about one-third of patients show clinical evidence of overlying DLE, two-thirds of patients show histologic evidence of DLE. It tends to have a mild disease course in the majority of cases.

Adolescent↗
PubMed1995

Association of systemic lupus erythematosus and gluten enteropathy.

Systemic lupus erythematosus presents various clinical manifestations. The coexistence of systemic lupus erythematosus and celiac sprue has been rarely reported. We present a patient who had systemic lupus erythematosus with malabsorption demonstrating characteristic clinical and pathologic findings of celiac sprue (gluten enteropathy).

Celiac Disease↗
PubMed1999

The cutaneous pathology associated with seropositivity for antibodies to SSA (Ro): a clinicopathologic study of 23 adult patients without subacute cutaneous lupus erythematosus.

Antibodies to Ro/SSA are found in patients with subacute cutaneous lupus erythematosus (SCLE), complement deficiency lupus erythematosus, systemic lupus erythematosus (SLE), neonatal lupus erythematosus, and Sjögren syndrome (SS). Most studies describing the cutaneous pathology associated with anti-Ro antibodies have been of patients with SCLE. Over a 42-month period, we encountered skin biopsy specimens from 23 anti-Ro-positive patients who did not have SCLE: 15 had SLE variably manifesting as SCLE-like rashes; malar erythema; a dermatomyositis-like rash; vascular disease involving cutaneous, cardiac, peripheral, and central nervous systems; restrictive pulmonary disease; periorbital edema; and myositis. Two patients had primary Sjögren syndrome, one had primary antiphospholipid antibody syndrome, and two had rheumatoid arthritis; all five had clinical evidence of cutaneous vasculopathy encompassing livedo, perniosis, and palpable purpura. Three additional patients presented with folliculocentric purpura without other stigmata to permit classification as a specific connective tissue disease. In the SLE patients, biopsy specimens of photodistributed eruptions showed an interface dermatitis accompanied by superficial vascular plexus density reduction. Vasculopathic reactions in patients with and without SLE comprised neutrophilic, lymphocytic, or pauciinflammatory thrombogenic subtypes. Although at times a marker of SCLE, the identification of anti-Ro antibodies may isolate a subset of patients at higher risk of multiorgan vasculopathy, myositis, and progressive pulmonary disease. We postulate that many of the features seen in these patients reflect the sequelae of antibody mediated endothelial cell injury.

Adult↗
PubMed1984

Neonatal lupus erythematosus: a review.

Neonatal lupus erythematosus is an uncommon type of lupus that in addition to cutaneous lesions may have systemic involvement. It is generally a benign condition unless complicated by complete heart block, which is irreversible and which carries a high mortality rate in the first year of life. Patients' mothers often show clinical and/or laboratory features of connective tissue diseases.

Diagnosis, Differential↗
PubMed1995

Diagnostic evaluation of the lupus band test in discoid and systemic lupus erythematosus.

BACKGROUND: The usefulness of the lupus band test (LBT) in the diagnosis of cutaneous lupus erythematosus remains controversial. The study was done to determine the sensitivity, specificity, and predictive value of the LBT in discoid lupus erythematosus (DLE) and systemic lupus erythematosus (SLE). METHODS: During the study period, 32 patients with SLE and 28 patients with DLE were included. The final classification of cases was based on the American Rheumatism Association Criteria (ARA) for SLE, histopathology, and consensus of the clinical staff. Thirty controls, 15 each for DLE and SLE, were chosen from a group of patients, who had lesions clinically simulating DLE and SLE, but could be excluded by histopathology, laboratory tests, and follow-up. Histopathologic examinations and direct immunofluorescence (DIF) tests were done on lesional skin. RESULTS: In DLE, the sensitivity of the LE band test was 58% and the specificity 87%. The positive predictive value was 95% and negative predictive value was 32%. In SLE the sensitivity was 93% and specificity 87%. The positive predictive value was 64% and negative predictive value 98%. CONCLUSIONS: The high negative predictive value of LBT in SLE suggests that it is valuable in excluding diseases clinically similar to SLE.

Adolescent↗
PubMed1995

Neurologic characteristics of childhood lupus erythematosus.

Records of 108 patients with lupus erythematosus beginning in childhood (1953-1990) were reviewed; 25 had recorded neurologic findings. This is the largest group of childhood lupus erythematosus patients with neurologic disease that has been reported. The average age of children at the time of diagnosis of lupus was 154 months. There were 22 girls and 3 boys in the group. All patients met at least four of the 1982 American Rheumatism Association criteria for the classification of systemic lupus erythematosus. Average age at onset of neurologic difficulties was 168 months. In 4 patients, the neurologic symptoms preceded the diagnosis: 1 month (spastic diplegia), 1 month (bilateral weakness and spasticity), 24 months (chorea), and 26 months (chorea), respectively. Four patients had neurologic symptoms coincident with the diagnosis of lupus erythematosus. In those patients whose symptoms followed the diagnosis of lupus erythematosus, the average elapsed time until symptoms appeared was 33 months; the single lowest and highest outliers were discounted. Most frequent findings were headache (16/25) and behavioral aberrations (10/25). All behavioral manifestations were depression except in 1 patient. Other prevalent findings included hemichorea or chorea (7/25), cerebrovascular accident with hemiplegia or diplegia (7/25), seizures (5/25), visual loss (3/25), and cranial neuropathy (2/25). Vertigo and myelopathy occurred in 1 patient each. All patients were treated primarily with corticosteroids and azathioprine; in the presence of active disease, the drug dosages were increased with significant improvement in neurologic symptoms. Resolution usually occurred from days to months; most improved in a few days to a few weeks; 3-4 months was the longest period until symptoms subsided.

Adolescent↗
PubMed2005

Management of "refractory" skin disease in patients with lupus erythematosus.

Skin disease in patients with lupus erythematosus can be subdivided into two broad categories-those lesions that, when biopsied, demonstrate an interface dermatitis and those that do not demonstrate an interface dermatitis. The skin lesions that are represented by the interface dermatitis include discoid lupus erythematosus (DLE), subacute cutaneous lupus erythematosus (SCLE), and acute cutaneous lupus erythematosus. Many patients with these cutaneous lesions can be managed with "standard" therapies, including sunscreens, protective clothing and behavioral alteration, and topical corticosteroids with or without an oral antimalarial agent. These standard therapies are often not used appropriately, resulting in a situation in which the patient is felt to have refractory disease. This chapter discusses these therapies and defines what is meant by refractory disease and how the author approaches these patients.

Antimalarials↗
PubMed1998

Treatment of systemic lupus erythematosus: an update.

Systemic lupus erythematosus predominantly affects women and is more common in blacks. Although survival rates have improved, over one half of patients with systemic lupus erythematosus have permanent damage in one or more organ systems. Arthritis and cutaneous manifestations are most common, but renal, hematologic and neurologic manifestations contribute largely to morbidity and mortality. Treatment approaches emphasize using a combination of drugs to minimize chronic exposure to corticosteroids.

Female↗
PubMed2006

Cyclophosphamide versus methylprednisolone for treating neuropsychiatric involvement in systemic lupus erythematosus.

BACKGROUND: Neuropsychiatric involvement in systemic lupus erythematosus is complex and several clinical presentations are related to this disease such as: convulsions, chronic headache, transverse myelitis, vascular brain disease, psychosis and neural cognitive dysfunction. This systematic review is an update of a review performed in 2000. OBJECTIVES: To assess the efficacy and safety of cyclophosphamide and methylprednisolone in the treatment of neuropsychiatric manifestations of systemic lupus erythematosus. SEARCH STRATEGY: We searched EMBASE, LILACS, Cochrane Central Register of Controlled Trials (CENTRAL) and MEDLINE up to and including May 2005. Additional articles were sought through handsearching in relevant journals. There were no language restrictions. SELECTION CRITERIA: All randomised controlled trials that compared cyclophosphamide to methylprednisolone were included. Patients of any age and gender were included as long as they fulfilled the criterion of the American College of Rheumatology for the diagnosis of systemic lupus erythematosus and presented with any one of the following neuropsychiatric events: convulsions, organic brain syndrome and cranial neuropathy. Outcome measures included the following: a) overall mortality (primary event); b) motor and psychiatric deficit (primary event); c) clinical improvement (secondary event). DATA COLLECTION AND ANALYSIS: Data was independently extracted by two reviewers and cross-checked. The methodological quality of each trial was assessed by the same two reviewers. Details of the randomisation (generation and concealment), blinding, and the number of patients lost to follow-up were recorded. Dichotomous data was presented as relative risks with corresponding 95% confidence intervals and a clinical relevance table was produced. MAIN RESULTS: We found one randomised controlled trial of 32 patients comparing cyclophosphamide versus methylprednisolone for the treatment of neuropsychiatric involvement in the systemic lupus erythematosus. A significantly greater number of people responded to treatment in the cyclophosphamide group. Treatment response was found in 94.7% (18/19) of patients using cyclophosphamide compared with 46.2% (6/13) in the methylprednisolone group at 24 months (RR 2.05, 95% CI 1.13, 3.73) The NNT for response to treatment is 2. Cyclophosphamide use was associated with a reduction in prednisone requirements. A significant decrease in the number seizures per month was observed in the cyclophosphamide group. All the patients in the cyclophosphamide group had electroencephalographic improvement. No significant differences in adverse effects between the groups were found. It was not possible to extract more data from the study because there was a small number of patients in the others clinical subgroups of neurological manifestations and the authors did not provide sufficient information for data extraction. AUTHORS' CONCLUSIONS: This systematic review found one randomised controlled trial with a small number of patients in the different clinical subgroups of neurological manifestation. It seems that cyclophosphamide is more effective in the treatment of neuropsychiatric involvement in systemic erythematosus lupus compared with methylprednisolone. However, properly designed randomised controlled trials that involve large, representative numbers of individuals, with explicit clinical and laboratory diagnosis criteria, sufficient duration of follow-up and description of all relevant outcome measures are necessary to guide practice.

Cyclophosphamide↗
PubMed1994

Significance of autoimmune haemolytic anaemia and immune thrombocytopenia (Evans' syndrome) in systemic lupus erythematosus.

Patients with systemic lupus erythematosus (SLE) have an extremely variable prognosis and mortality. The purpose of this report is to highlight the importance of looking for lupus parameters in patients with autoimmune haemolytic anaemia (AIHA) and/or immune thrombocytopenia (ITP) as this represents a subgroup of systemic lupus erythematosus (SLE) patients with a fairly good prognosis. We report a case of an 8-year-old Malay boy who was admitted to hospital with fever and gum bleeding. Physical examination revealed a malar rash, oral ulcers, petechial haemorrhages and bruises over the limbs, generalised lymphadenopathy and hepatosplenomegaly. Laboratory investigations confirmed the diagnosis of SLE. The patient's serum showing the presence of antinuclear factor, antiphospholipid antibodies and a biological false-positive test for syphilis. Immunological and haematological parameters were in keeping with combined AIHA and ITP (Evans' syndrome). No organ involvement was present and the patient responded well to corticosteroid therapy. This case demonstrates the importance of making an early diagnosis of SLE with haematological complications, in order to ensure full benefit of therapy and emphasises the good prognosis expected in this subgroup of SLE patients.

Anemia, Hemolytic, Autoimmune↗
PubMed1986

Circulating T- and B-cell abnormalities in cutaneous lupus erythematosus.

Seven patients with subacute cutaneous lupus erythematosus (SCLE), 11 patients with discoid lupus erythematosus (DLE), and 17 age-, sex-, and race-matched controls were examined for the number of circulating peripheral blood T and B cells, immunoglobulin (Ig) synthesizing cells, and Ig secreting cells. A significant alteration in T-cell subsets was detected only in SCLE patients who manifested a decreased number of OKT8 cells. Circulating B cells were significantly increased only in the SCLE patients. The percentage of Ig synthesizing and secreting cells was significantly increased in both the DLE and the SCLE groups. The somewhat unexpected increase in Ig synthesizing and secreting cells in the DLE patients could not be accounted for by antimalarial treatment or the concurrence of the HLA-DR3 phenotype. There was, however, a weak but significant correlation between the degree of B-cell activation in the DLE patients and the number of American Rheumatism Association criteria for the classification of systemic lupus erythematosus (SLE) possessed by these patients. These data demonstrate that abnormalities in B-cell function similar to those seen in SLE can also be found in a group of lupus erythematosus patients whose clinical disease expression is limited predominantly to the skin.

B-Lymphocytes↗
PubMed1995

Coronary artery bypass grafting in patients with systemic lupus erythematosus. Report of 2 cases.

Systemic lupus erythematosus is an autoimmune disease that often involves the cardiovascular system. Coronary artery narrowing in patients with lupus erythematosus is severe, progressive, and related to the duration of the disease rather than to the age of the patient. Steroid use in such patients has improved their life expectancy but seems to be increasing the incidence of coronary involvement. Consequently, a larger number of systemic lupus erythematosus patients may be candidates for myocardial revascularization in the future. We report our experience with myocardial revascularization in 2 women with severe systemic lupus erythematosus, incapacitating angina, and severe obstructive coronary artery disease. One of the women required balloon angioplasty 19 months after coronary artery bypass grafting and remains asymptomatic nearly 3 years later. The other patient is free of symptoms 9 months after surgery. Our results with these 2 patients are encouraging. Long-term follow-up should yield further information regarding the benefits of myocardial revascularization and coronary angioplasty in patients with systemic lupus erythematosus.

Adult↗
PubMed2004

Neonatal lupus erythematosus with cholestatic hepatitis.

Neonatal lupus erythematosus is an uncommon passive autoimmune disease in which there is transplacental passage of anti-Ro/SSA and/or anti-La/SSB or anti-U1RNP maternal autoantibodies. Its common clinical manifestations include cardiac disease, notably congenital heart block, cutaneous lupus lesions, and hematologic problems. During the past decade, it has become clear that hepatobiliary disease may also occur as a manifestation of neonatal lupus erythematosus. We report a case of neonatal lupus erythematosus in a male infant who had lupus hepatitis with jaundice in addition to cutaneous lupus, anemia, and thrombocytopenia. Other diseases in the differential diagnosis of conjugated hyperbilirubinemia, including metabolic, infectious, and inherited anatomic conditions were all ruled out. The infant had a high titer of antinuclear antibodies (titer 1:640) with a speckled pattern, anti-Ro/SSA and anti-La/SSB antibodies, and no anti-dsDNA antibodies. Treatment with prednisolone (2 mg/kg/day) for 14 days resulted in dramatic improvement of the thrombocytopenia. Hemoglobin and bilirubin returned to normal 2 months later, and transaminases were normal by 10 months of age.

Anemia↗
PubMed2002

[Acquired haemophilia in a patient with systemic lupus erythematosus].

INTRODUCTION: In patients with lupus, the most common acquired circulating anticoagulant is antiprothrombinase which is responsible for thrombosis. The presence of antibodies directed against factor VIII is rarely found in systemic lupus erythematosus. A case of acquired haemophilia in a patient with lupus is reported. CASE REPORT: A 30 year-old woman with systemic lupus erythematosus developed a right coxalgia and ecchymotic skin lesions which were prominent on the right arm and forearm. Laboratory values were as follows: positive antinuclear antibodies > 1: 2 560, anti-DNA antibodies (300 IU/ml), prolonged activated partial thromboplastin time, reduced factor VIII activity (1 p. 100) and the presence of antibodies against factor VIII. Magnetic nuclear resonance of the right hip confirmed the presence of an intramuscular hematoma. The patient was initially treated with intravenous pulse and oral corticosteroids, intravenous immunoglobulins and intravenous cyclophosphamide. Clinical and biological improvement was promptly obtained. DISCUSSION: In our patient with systemic lupus erythematosus, bleeding revealed acquired haemophilia with antibodies against factor VIII. It should be pointed out that the association between lupus and haemophilia is uncommon and that at present no standardized treatment can be recommended.

Adult↗
PubMedDate not supplied

Atrial fibrillation associated with systemic lupus erythematosus and use of methylprednisolone.

Systemic lupus erythematosus is known to have cardiac manifestations consisting of pericarditis, myocarditis, endocarditis, and coronary vasculitis. Pericarditis is the most common cardiac manifestation of systemic lupus erythematosus. Myocarditis may be suspected in patients presenting with unexplained tachycardia, conduction disturbances, unexplained systolic dysfunction with or without heart failure, or arrhythmias. The development of arrhythmias in systemic lupus erythematosus could be secondary to pericarditis, myocarditis, or ischemia caused by coronary vasculitis. The development of atrial fibrillation in systemic lupus erythematosus is not commonly reported. There have been few reports on the patients developing atrial fibrillation after being started on methylprednisolone therapy. Described here is a case of the development of atrial fibrillation in a newly diagnosed 37-year-old patient with systemic lupus erythematosus who was started on intravenous methylprednisolone therapy.

Adult↗
PubMed1992

Neonatal lupus erythematosus: a case report.

Neonatal lupus erythematosus is a syndrome characterized by congenital heart block and/or cutaneous lupus erythematosus in the presence of maternal autoantibodies. We report a female newborn with cutaneous lesions of the neonatal lupus erythematosus born to a clinically asymptomatic mother. She presented with erythematous maculopapules on the face, neck and upper trunk at birth. Complete blood cell count showed leukopenia and thrombocytopenia. Antinuclear antibodies, anti-Ro/SSA and anti-La/SSB antibodies were positive in the mother and infant. The skin lesions became annular, erythematous and slightly scaling three weeks later. A skin biopsy performed at seven weeks of age showed edema of the dermis and perivascular mononuclear cell infiltration. Immunofluorescent studies showed immune complex deposition of IgG an IgM in the basal zone of epidermis. She received supportive treatment and sun avoidance. Hematologic abnormalities recovered by nine weeks of age. Skin lesions faded gradually during the following four months, leaving residual hypopigmentation and telangiectasia.

Female↗
PubMed1992

Peripheral vascular disease in patients with systemic lupus erythematosus.

Patients with systemic lupus erythematosus may develop premature atherosclerosis, notably coronary artery disease. A group of 10 patients with peripheral vascular disease presenting with intermittent claudication or gangrene were studied from a group of 563 patients followed prospectively at the Wellesley Hospital Lupus Clinic. These 10 patients were compared with the next lupus clinic patient matched for age and sex, with respect to demographic characteristics and risk factors. The patients and controls did not differ significantly in lupus activity criteria count, partial thromboplastin time, the number with antibody to cardiolipin, number receiving steroids or mean steroid dose, family history of atherosclerosis, hyperlipidaemia, smoking, hypertension or use of oral contraceptives. The risk factors for developing peripheral vascular disease were a longer duration of systemic lupus erythematosus and a longer duration of use of steroids. Eight of the 10 patients had coexistent coronary artery disease or transient ischaemic attack.

Adult↗