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PubMed1983

Lupus cystitis: primary bladder manifestations of systemic lupus erythematosus.

Clinical and radiographic findings of cystitis are reported in six patients with systemic lupus erythematosus. All patients had clinical manifestations of cystitis; radiography showed the bladder capacities were reduced and the bladder walls were thickened and irregular. Ureterohydronephrosis was found in some patients. Abnormal clinical and roentgenographic findings were reversed with high-dose corticosteroid therapy. These patients had concomitant widespread multisystem manifestations, especially of the central nervous system and gastrointestinal tract. No explanation for the bladder findings could be found other than lupus erythematosus. Urinary bladder involvement may be a primary manifestation of systemic lupus erythematosus, sometimes overshadowed by other major organ system disease. Corticosteroids appear to reverse most of the acute manifestations but the long-term prognosis is not known and may not be favorable.

Adrenal Cortex Hormones↗

PubMed2002

Complete atrioventricular block in a woman with systemic lupus erythematosus.

The complete atrioventricular (AV) heart block is a rare complication in the course of systemic lupus erythematosus. We describe a case of a young woman with systemic lupus erythematosus and lupus cardiomyopathy who was admitted into our hospital with a syncopal attack showing on the electrocardiogram a paroxysmal complete AV heart block. The syncopal attack was resolved with a pacemaker implantation.

Electrocardiography↗

PubMed1989

Systemic lupus erythematosus in Indian children.

Twenty cases of systemic lupus erythematosus (SLE) in prepubertal children (less than 14 years of age) were seen over a period of 14 years. The male:female ratio was 1:2.3, and the mean age at onset was 9.37 years. Fever with joint involvement was the commonest presenting manifestation (60%), followed by nephrotic syndrome (25%). Notable clinical features included a high incidence of renal involvement (75%), significant hypertension (45%) and reversibility of acute renal failure (2 cases). The other organs and systems involved included: mucocutaneous manifestations (60%), cardiovascular system (30%), respiratory system (25%), neuropsychiatric manifestations (45%), and anemia (75%). Raynaud's phenomenon and thrombocytopenia were rare while leucopenia was not seen in a single patient. Immunological abnormalities noted were 100% positivity for antinuclear antibodies, and 87.5 and 75% positivity for antibodies to double-stranded and single-stranded DNA, respectively. Hypocomplementemia was seen in 75% of patients tested.

Adolescent↗

PubMedDate not supplied

Severe retinopathy in systemic lupus erythematosus associated with IgG anticardiolipin antibodies.

A six-year-old boy with systemic lupus erythematosus developed insidiously a monolateral serous retinal detachment leading to severe visual loss. Serial determinations of anticardiolipin antibodies showed the presence of IgG anticardiolipin antibodies only at the time of ocular involvement. This case underlines the need for regular ophthalmologic evaluation in children with active systemic lupus erythematosus and supports the previous hypothesis of a relationship between antiphospholipid antibodies and retinal involvement in systemic lupus erythematosus.

Antibodies, Anticardiolipin↗

PubMed1984

[Bullous eruption in systemic lupus erythematosus].

A 39 years old black woman with systemic lupus erythematosus developed tense bullae on erythematous bases on the flexor surfaces of the forearms and oral cavity. Some blisters healed with atrophy and pruritus was a striking feature. The diagnosis of systemic lupus erythematosus was based upon the following criteria: the typical blush in the butterfly area, alopecia, a painful macular papular eruption on the palms and fingers, fever, arthralgia, anemia, leukopenia, elevation of erythrocyte sedimentation rate and positive ANA. Histologic examination showed a subepidermal bulla formation and perivascular inflammatory infiltrate containing lymphocytes and eosinophils. By indirect immunofluorescence no autoantibodies were detected. Direct immunofluorescence showed deposition of linear IgG. High doses of prednisone brought about clinical remission but there was no response to sulfone. The patient is on maintenance dose. The authors discuss the differences between the three diseases and conclude that the bullous eruption cannot be classified.

Adult↗

PubMed1994

Papulonodular dermal mucinosis in systemic lupus erythematosus.

A 38-year-old woman with systemic lupus erythematosus presented to the dermatology department with diffuse nodules and plaques on her face, trunk and extremities. A lesional biopsy revealed large dermal mucin deposits. Papulonodular dermal mucinosis is characterized histologically by diffuse dermal mucin without the classic epidermal or inflammatory changes seen in lupus erythematosus (LE). This rare variant of LE should be distinguished from other cutaneous mucinoses.

Adult↗

PubMedDate not supplied

Crithidia luciliae assay for antibodies to native DNA in procainamide-induced lupus erythematosus.

Sera from 49 patients with procainamide-induced lupus erythematosus or circulating antinuclear antibodies were found to be negative for antibodies to native DNA by the CL immunofluorescence assay. This finding suggests that the CL assay is helpful in discriminating between procainamide-induced and naturally occurring systemic lupus erythematosus. The results further confirm the relatively high degree of specificity of the CL assay for SLE.

Adult↗

PubMed1978

Association of systemic lupus erythematosus and SLE-like syndromes with hereditary and acquired complement deficiency states.

The most prominent association of rheumatic diseases with hereditary complement deficiency is systemic lupus erythematosus (SLE) and discoid lupus erythematosus with homozygous C2 deficiency in females. The lupus disease in these patients differ from classic lupus in 1) the increased incidence of discoid lesions, 2) the low incidence of renal disease, 3) the low or absent titers of antibodies to native DNA, and 4) the infrequent finding of immunoglobulin and complement in skin lesions. The strong positive linkage disequilibrium between C2 deficiency and HLA genes raises the possibility that genes other than those determining C2 levels may have the primary role in determining predisposition to disease in these patients. However, the finding of similar diseases in certain patients with hereditary angioedema and SLE-related syndrome who have acquired deficiency of the early components of complement supports a primary role of the C2 deficiency gene in predisposing to lupus disease in these patients.

Arthritis, Rheumatoid↗

PubMed2001

A candidate gene analysis of three related photosensitivity disorders: cutaneous lupus erythematosus, polymorphic light eruption and actinic prurigo.

BACKGROUND: Polymorphic light eruption (PLE) is a common inherited photosensitivity disorder, which may predispose to several related but distinct conditions, including subacute cutaneous lupus erythematosus (SCLE), discoid lupus erythematosus (DLE) and actinic prurigo (AP). OBJECTIVES: To examine specific candidate genes for shared susceptibility alleles between these related phenotypes. METHODS: Eighty-five caucasian patients with annular SCLE or DLE were recruited, in addition to 102 first-degree relatives. The prevalence of PLE in both the patient and relative groups was determined by detailed interview and clinical examination. Eighty-five patients with pure PLE and 59 patients with AP were also recruited. Candidate genes were analysed by typing of single nucleotide polymorphisms of IL10 (-1082 G/A and -819 C/T), FCGR2A (131 R/H), SELE (128 S/R), ICAM1 (241 G/R and 469 E/K), IL1A (+ 4845 G/T), IL1B (-511 C/T and + 3954 C/T), IL1RN (+ 2018 T/C) and TNF (-308 G/A) using polymerase chain reaction (PCR) with sequence-specific primers and 5'-nuclease PCR. RESULTS: A significant association was found between SCLE and the rare TNF -308 A allele when compared with patients with DLE (P = 0.043), PLE (P = 0.001), AP (P < 0.001) and healthy controls (P < 0.001). However, there was strong linkage disequilibrium between TNF -308 A and the HLA A*01, B*08, DRB1*0301 haplotype. A negative association was also found between SCLE and the IL1B + 3954 T allele (P = 0.039), but the significance was lost on correction for multiple testing. CONCLUSIONS: We have demonstrated the association of SCLE with the rare TNF -308 A allele, which may be pathogenic or, alternatively, a marker allele for the extended HLA A*01, B*08, DRB1*0301 haplotype that is associated with a number of autoimmune conditions. Although many of the other loci that we chose failed to demonstrate an association, a candidate gene approach remains the most logical one, and the most likely to yield positive results in the future.

Alleles↗

PubMed2006

Failure of physiologic doses of pure UVA or UVB to induce lesions in photosensitive cutaneous lupus erythematosus: implications for phototesting.

BACKGROUND: Phototesting studies in cutaneous lupus erythematosus have yielded variable results, with most trials reporting photo-induction of lesions by both UVA and UVB in substantial numbers of patients. OBJECTIVES: To determine the minimal erythema dose in patients with subacute cutaneous lupus erythematosus (SCLE) and controls. PATIENTS/METHODS: We phototested nine patients with SCLE and 14 skin type-matched controls, using repetitive dosing of UVA1 and UVB, but with filters that removed most of the shorter UVC and longer infrared and visible light. In addition, DNA was isolated from anticoagulated blood to genotype the TNF-alpha 308 region in each patient and control. RESULTS: We were unable to demonstrate a difference in minimal erythema dose (MED) between patients and controls, or any correlation of MED with either TNF genotype or systemic drug therapy for SCLE. In addition, no SCLE skin lesions were induced in the nine patients with either UVA or UVB, and one patient cleared a skin lesion after low-dose UVA1 irradiation. CONCLUSIONS: The potential role of wavelengths outside the UVA and UVB range in the photo-induction of cutaneous lupus skin lesions needs to be investigated, and there is a need to standardize phototesting equipment and procedures for patients with cutaneous lupus erythematous.

Case-Control Studies↗

PubMed1995

Case report: testosterone treatment of systemic lupus erythematosus in a patient with Klinefelter's syndrome.

Systemic lupus erythematosus occurs with much greater frequency in females than in males, and in some reports, researchers suggested that treatment with androgenic hormones might have therapeutic effects in this disease. The authors report a case of systemic lupus erythematosus in a hypogonadal male with Klinefelter's syndrome who was treated with testosterone in doses sufficient to normalize the serum level of this hormone to the adult male range. Hematologic and serologic abnormalities, including elevated levels of anti-DNA antibodies and depressed complement levels, returned to normal within 9 months of increasing the testosterone dose. The findings in this patient indicate that androgenic steroids can exert significant effects on immune parameters, and suggest that effects of androgens on the immune system may contribute to the sexual dimorphism of autoimmune disease.

Adolescent↗

PubMed1994

Dendritic cells in cutaneous lupus erythematosus: a clue to the pathogenesis of lesions.

In view of the critical role of dendritic cells in immune mediated skin diseases, we have investigated the membrane antigen patterns and ultrastructure of cutaneous dendritic cells in eight patients with chronic discoid lupus erythematosus and five with subacute cutaneous lupus erythematosus. In the lesional epidermis, the expression of HLA-DR antigens by epidermal dendritic cells was reduced, as compared with perilesional, clinically normal skin. In addition, only few CD1a+ dendritic cells (Langerhans' cells), along with some CD11c+ and CD14+ cells (presumable precursors of Langerhans' cells), were found in atrophic areas of lesional epidermis. In contrast, the number of Langerhans' cells in non-atrophic areas of lesional epidermis was similar to that in perilesional skin. On electronmicroscopy, epidermal Langerhans' cells appeared depleted of organelles and dendrites and contained tubuloreticular inclusions. In the lesional dermis, both CD1a+ and CD36+ dendritic cells were found, associated with CD4+ and CD8+ T-cells, respectively. Moreover, CD11c+ and CD14+ cells were found around capillaries in the papillary dermis on electronmicroscopy. Indeterminate cells (dendritic cells with features of Langerhans' cell lineage, but apparently without Birbeck granules) and dendritic macrophages were found, associated with lymphocytes and mast cells. No cells with intermediate/transitional features between these two dendritic cell types were found. Conversely, peculiar dendritic cells--with short and blunt dendrites and cytoplasm containing many flat, rough cisternae, moderately well developed Golgi apparatus and no lysosomes--were found in the same location as the CD11c+ and CD14+ cells identified by light microscopy. These findings might be interpreted as follows: 1 the alterations in cytological differentiation and expression of functionally meaningful molecules by epidermal Langerhans' cells in cutaneous lupus erythematosus lesions suggest an impairment of their immunological efficiency; 2 in the lesional dermis of cutaneous lupus erythematosus, a CD4+ T-cell/CD1a+ dendritic cell-based, delayed-type immune response is possibly modulated by a suppressor T-cell circuit in which CD36+ dendritic cells may act as accessory cells; 3 CD11c+ and CD14+ cells with peculiar ultrastructure are possible precursors of both CD1a+ indeterminate cells and CD36+ dermal dendrocytes in the dermis.

Adolescent↗

PubMed1988

AA amyloidosis associated with systemic lupus erythematosus.

A 54-year-old female with a 23-year history of systemic lupus erythematosus was admitted because of loss of renal function and nephrotic syndrome. Renal biopsy showed deposition of AA amyloid as demonstrated by Congo red staining and reactivity with protein AA-specific antibodies. Immune deposits were present in the mesangium and the glomerular basement membrane, but histopathological changes consistent with lupus nephritis were not detected. The rare association of systemic lupus erythematosus and amyloidosis is discussed in view of the characteristics of the acute phase reaction in systemic lupus erythematosus.

Amyloidosis↗

PubMed2003

[Systemic lupus erythematosus with disease onset after age 65].

OBJECTIVES: Systemic lupus erythematosus with disease-onset in the elderly has rarely been studied (only one report about 21 patients with disease onset at 65 and older). Is the management of this pathology modified in this population? METHODS: Seventeen hospitalised cases of lupus patients with disease onset at 65 or older are retrospectively reported between 1988 and 2000. The results are compared with those of younger subjects. RESULTS: The female to male ratio is 1.83. Mean age at disease onset is 71.9 +/- 3.5 years. Mean duration of follow-up is 3.5 +/- 2.4 years. Main initial symptoms are: deterioration of general status (41%), arthritis (35%), cutaneous manifestations (35%), thrombo-embolism (24%) and pleuritis (18%). Malar rash is uncommon (12%). Nephropathy is never a revealing symptom and is rarely serious during the disease's evolution. Like in neurologic manifestations, the etiology has to be discussed in relation to associated co-morbidities. Concerning haematologic features, lymphopenia is found in 82% of the cases with a questionable specificity. Antinuclear antibodies are constant, anti-dsDNA antibodies are found in 82% of the cases, antibodies to extractable nuclear antigens in 50%, and anticoagulant circulating activity in 59%. Prognosis is difficult to assess in such a limited series but 5-years survival probability is 83%. Glucocorticoid lead to 50% of major complications. CONCLUSIONS: This study focuses on the particular initial manifestations of systemic lupus erythematosus in the elderly (deterioration of general status, thrombosis, unusual cutaneous symptoms), and on the specificity of differential diagnosis and treatment.

Age Distribution↗

PubMed1989

Neonatal lupus erythematosus: report of a case.

We present the first report of neonatal lupus erythematosus from Taiwan. A female baby, born to a mother with documented systemic lupus erythematosus (SLE), developed cutaneous lupus lesions after phototherapy for hyperbilirubinemia. She had additional clinical features of hemolytic anemia and thrombocytopenia. Detailed serological and immunogenetic studies were performed. Transplacental passage of both anti-SSA/Ro and anti-SSB/La antibodies were demonstrated and their disappearance at the age of 6 months correlated with regression of clinical symptoms. This patient inherited human leukocyte antigen (HLA) A11, Bw60 Cw3, DR2 and Aw33, Bw57, Cw7, DRw6 from her father and mother, respectively. A long-term follow-up is required for observing whether she will develop SLE in the future.

Adult↗

PubMedDate not supplied

Hyposplenia in a patient with systemic lupus erythematosus.

During the course of systemic lupus erythematosus in a young female patient the peripheral blood smear incidentally showed the features of hyposplenism. Silent lupus vasculitis with splenic infarction was suspected as the cause of this.

Adult↗

PubMed1978

Acute abdominal complications of systemic lupus erythematosus.

Four patients with acute abdominal complications of systemic lupus erythematosus requiring laparotomy are presented. The complications consisted of intestinal ischaemia, without perforation, in 3 patients and omental infarction in the fourth. In 2 patients the abdominal emergency was the first manifestation of the collagen disease. Tissue removed from 3 patients showed a marked vasculitis on histological examination. The pathogenesis and management of acute abdominal complications of systemic lupus erythematosus are discussed and the literature reviewed.

Abdomen, Acute↗

PubMed1992

[Cutaneous lupus erythematosus and cardiolipin antibodies. Incidence and clinical significance].

In recent years, the importance of antiphospholipid antibodies in systemic lupus erythematosus and various other dermatological and internal diseases has been recognized. Characteristic symptoms associated with these antibodies are venous and arterial thrombosis, recurrent fetal loss, thrombocytopenia, and haemolytic anaemia. Two antiphospholipid antibody subgroups that are clinically relevant can be discerned: anticardiolipin antibodies and lupus coagulant. In this study, 51 clinically well-characterized patients with predominantly cutaneous lupus erythematosus were screened for the presence of anticardiolipin antibodies. Anticardiolipin antibodies could be detected in only three patients. These data suggest that, in patients with cutaneous lupus erythematosus, anticardiolipin antibodies should be measured only in the presence of symptoms associated with antiphospholipid antibodies.

Antiphospholipid Syndrome↗