[Inhibition of the Haserick factor by rabbit immune serum against human lupus erythematosus serum. Disseminated lupus erythematosus. Clinical serology].
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Circulating antinuclear antibodies and in vivo bound immunoglobulins at the dermal-epidermal junction are frequently seen in patients with lupus erythematosus. The present study was designed to examine the distribution of the IgG subclasses of in vivo skin bound IgG and circulating antinuclear antibodies (ANA) in patients with systemic lupus erythematosus (SLE) or subacute cutaneous lupus erythematosus (SCLE). Immunofluorescence studies on skin biopsies showed IgG1 to be the predominant IgG subclass in SCLE patients, present in 20 of 21 (95%) of the specimens. IgG2 was present in 4 patients (19%), IgG3 in 1 (5%), and IgG4 in 7 (33%). The frequencies of IgG2, IgG3, and IgG4 skin staining were significantly higher in the seven SLE patients who were studied: IgG1 in 7/7 (100%), IgG2 in 7/7 (100%) and IgG4 in 6/7 (86%). Immunoblot analysis for the IgG subclasses was performed on serum of 29 patients with SCLE who had antibodies to SSA/Ro antigen. Twenty-seven (93%) of these patients were positive for IgG1 anti-SSA/Ro antibody, while the frequencies for IgG2, IgG3, and IgG4 anti-SSA/Ro were very low. These studies indicate that there is a difference in the IgG subclass antibody response in patients with SLE and SCLE. The presence of more than one subclass antibody may be indicative of systemic disease.
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Nail changes occur in about 25% of systemic lupus erythematosus (SLE) cases. Onycholysis has been reported as the most frequent abnormality in SLE. Nailbed hyperkeratosis may be observed in both SLE and discoid lupus erythematosus (DLE). Involvement of the nail apparatus in DLE is extremely uncommon and never restricted to it. We report on a patient in whom the clinical features on the proximal nailfold were similar to those observed on the skin of a patient with typical DLE. This has, to the best of our knowledge, not yet been reported. The patient also exhibited a very distinctive prominent subungual hyperkeratosis. Interestingly, the patient developed biological alterations suggesting a systematization of the disease. Only a combination of systemic corticoids, retinoids and antimalarials was able to achieve nail improvement and this partial resistance to therapy may be explained by the very unusual subungual hyperkeratosis.
A female infant with neonatal lupus erythematosus had scanty discoid lesions and concurrent lupus erythematosus profundus on the face. Depression of lupus erythematosus profundus lesions was still evident at 4 years of age.
A patient with chronic discoid lupus erythematosus was admitted with fever, arthralia, pleuropericarditis, and a history of leukopenia. He was initially believed to have systemic lupus erythematosus (SLE), but extensive evaluation showed negative immunologic studies and the presence of acid-fast organisms on pericardial biopsy specimens with cultures positive for Mycobacterium tuberculosis. Discoid lupus erythematosus patients with extracutaneous manifestations should be carefully studied for concurrent illness, especially when serologic evidence fo SLE is negative.
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A patient previously reported to have discoid lupus erythematosus as a neonate eventually developed systemic lupus erythematosus (SLE) at age 19 years. This case illustrates that although neonatal lupus erythematosus is usually self-limited, SLE may develop in selected patients in future years.
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We studied the autoimmune serologic features and histocompatibility antigen associations of 27 patients who had a widespread, nonscarring and often photosensitive form of histologically specific cutaneous lupus erythematosus. We designated this disorder as subacute cutaneous lupus erythematosus. Skin lesions from this disorder can be distinguished from scarring discoid lupus erythematosus lesions both on a morphologic and histopathologic basis. Antinuclear and anticytoplasmic antibodies (Ro or Ro and La) and circulating immune complexes were frequently present in patients with subacute cutaneous lupus erythematosus, whereas rheumatoid factor and anti-lymphocyte, anti-DNA, anti-nRNP, and anti-Sm antibodies were found less frequently. Patients having annular skin lesions represented a particularly homogeneous subgroup in which there was a striking concordance of anti-Ro antibodies and the HLA-DR3 phenotype. These studies clearly establish that the presence of these lesions can serve as a cutaneous marker for a distinct subset of patients with lupus erythematosus who share similar clinical, serologic, and genetic features.
Lupus nephritis may be considered the prototypic autoimmune disease which is initiated by immune complex deposition. Several patterns of localization of immunoreactants are observed in lupus nephritis, but few data are available to elucidate the corresponding immunopathogenetic mechanisms. Corticosteroids form the mainstay of therapy for lupus nephritis, provided that they achieve a prompt clinical remission which can be sustained with alternate day therapy. Cyclophosphamide may have fewer risks than extended high-dose prednisone and it has been shown to be more efficacious than corticosteroids in preventing end stage renal failure.
We present a cross-sectional, population-based neuropsychological study of systemic lupus erythematosus (SLE) patients identified within Tampere University Hospital district, Finland with 440,000 inhabitants. Patients with definite SLE in the age range of 16-65 years (n = 46) and matched controls (n = 46) underwent neurological examination and comprehensive neuropsychological testing. On the basis of medical examination, the SLE patients were divided into neuropsychiatric (NP+; n = 15) and nonneuropsychiatric (NP-; n = 31) cases. The neuropsychological test results revealed more prevalent cognitive impairment in the NP+ patients, indicating that this subgroup mostly accounts for neuropsychological changes in SLE. Most characteristic changes in NP+ were observed in domains of memory, psychomotor speed, and complex attention. This suggests nonspecific CNS involvement, which is in line with neurological manifestations of the disease.
Two Caucasian carriers for chronic granulomatous disease (CGD) developed cutaneous lupus erythematosus (LE) with clinically and morphologically characteristic appearance for chronic discoid lupus erythematosus (DLE) and lupus erythematosus tumidus (LET). Direct immunofluorescent examinations and ANA titers were positive in both young women. No systemic involvement due to the ACR criteria was evident. Their sons suffered from X-linked cytochrome-b negative CGD. The diagnosis of CGD was based on measurement of oxidative burst activity by nitroblue tetrazolium (NBT) slide test and by flow cytometry using dihydrorhodamine 123 (DHR). The absence of cytochrome b558 in neutrophilic granulocytes was confirmed photometrically and by flow cytometry using the 7D5 monoclonal antibody against cytochrome b. We report for the first time the association of the photosensitive LE subtype LET and the X-linked CGD carrier state. Tissue damage by UV radiation and a reduced antimicrobial capacity may lead to recurrent immune stimulation and may together with genetic predisposition explain the occurrence of cutaneous LE in female carriers of CGD.
OBJECTIVE: To verify (1) how many patients with cutaneous lupus erythematosus (CLE) fulfill 4 or more American Rheumatism Association (ARA) and European Academy of Dermatology and Venereology (EADV) criteria for classification of systemic lupus erythematosus (SLE); (2) which criteria are mostly fulfilled; (3) the severity of the disease in patients fulfilling criteria; (4) how many patients with systemic involvement fail to fulfill 4 ARA and EADV criteria. METHODS: We studied 207 patients with chronic and subacute CLE, classified according to ARA and EADV criteria. RESULTS: Twenty-four patients with localized discoid (L-DLE; 21.8%), 22 with disseminated discoid (D-DLE; 30.5%) and 7 with subacute CLE (SCLE; 28%) had 4 or more ARA criteria. With EADV criteria, these figures fell to 7 (6.4%), 7 (9.7%) and 6 (24%), respectively. Only 3 L-DLE (2.7%), 5 D-DLE (6.9%) and 3 SCLE cases (12%) defined as SLE by ARA criteria and 1, 3 and 3, respectively, by EADV criteria had a renal or neurological disorder, hemolytic anemia and/or thrombocytopenia, vasculitis or serositis. ARA criteria did not classify 7 patients with a similar visceral involvement, while EADV criteria failed in 11 patients. CONCLUSION: In our patients, ARA criteria showed a sensitivity of 88%, a specificity of 79%, a positive predictive value of 56% and a negative predictive value of 96%. EADV criteria showed a sensitivity of only 64%, but a specificity of 93%, a positive predictive value of 61% and a negative predictive value of 94%. ARA criteria should not be used in CLE patients as they are too sensitive, poorly specific and altogether misleading. EADV criteria are more specific, but less sensitive.