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Lupus erythematosus and lupus valgaris, i.e., cutaneous tuberculosis, are confusing skin lesions in the history. Here, we reported on a Chinese lupus patient manifested with multiple nodular erythematous rashes on both upper extremities, which turned out to be cutaneous mycobacterial infection.
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.
The presence of the discoid lupus erythematosus (DLE) skin lesion in a patient with systemic lupus erythematosus (SLE) has been suggested to be a marker of less frequent and less severe renal disease. The clinical and laboratory features of seventeen patients who were seen in a dermatology practice and who had DLE as a manifestation of SLE (DLE-SLE) are reported. DLE preceded the diagnosis of SLE in eight patients. In six patients, the onset was concurrent, whereas in three the SLE was present prior to the discoid skin lesions. Five of the patients had lesions of subacute cutaneous lupus erythematosus (SCLE), and Raynaud's phenomenon occurred in eight patients. Clinical evidence of a renal abnormality was present in six patients (hematuria in three, proteinuria in five, and abnormal renal function in two). Antibodies to nuclear and/or cytoplasmic components were abnormal in all patients. Antibody subsets did not correlate well with clinical findings; only half of those with Raynaud's phenomenon demonstrated a positive ribonucleoprotein; only one patient with SCLE demonstrated anti-Ro (SSA) antibody, but four of the six patients with a renal abnormality had an elevated anti-native deoxyribonucleic acid antibody titer. The cutaneous lesions were eventually widespread in all patients, although two had initial disease that was localized to the head and neck. Although renal disease occurs in this group, it is less common and usually milder than in previous groups of unselected SLE patients.
Familial cases of lupus anticoagulant were rarely described. We report the observation of two sisters with lupus anticoagulant, one with thrombocytopenic purpura and the other with systemic lupus erythematosus.
Lupus anticoagulants are closely related to systemic lupus erythemathosus (SLE) and to thrombotic events. We describe a 12 year-old girl with a bilateral intramuscular haemorrhage of the gastrocnemius muscles as her main initial presentation of juvenile SLE. Laboratory work-up revealed lupus anticoagulant-hypoprothrombinaemia syndrome (LAHS) with very low levels of factor II due to autoantibodies. She showed a good initial clinical and laboratory response to prednisone therapy, however steroid dependency developed. To the best of our knowledge, this is the first case reported of juvenile SLE presenting with LAHS.
OBJECTIVE: To identify factors predictive of significant neuropsychiatric (NP) damage in systemic lupus erythematosus (SLE). METHODS: One hundred and thirty patients with SLE were followed at the University of Maryland Lupus Clinic from 1992 until 2003. NP manifestations were defined according to the revised American College of Rheumatology (ACR) nomenclature and case definitions for NP-SLE syndromes. Disease activity was measured using the SLE Disease Activity Index (SLEDAI), organ damage using the Systemic Lupus International Collaborating Clinics Damage Index SLICC/ACR (SDI); NP damage (NPDI) was measured with the corresponding domain of the SDI. At end of study period, 64 patients exhibited no NP damage (NPDI = 0) and 66 patients developed significant NP damage (defined as NPDI > or =1). The baseline features for these two patient groups were compared, and variables found to be significantly different were examined by multivariable analyses to determine their contribution to NP damage. RESULTS: Significant NP damage is common in SLE; mortality is infrequent and the cause of death is unrelated to NP damage. Independent predictors of significant NP damage were disease activity, Caucasian ethnicity and the presence of antiphospholipid antibodies and anti-Ro/SSA antibody. Certain clinical features at baseline predicted specific NP damage. For example, higher disease activity at baseline was predictive of psychosis and cognitive impairment, anti-dsDNA was predictive of polyneuropathy, and antiphospholipid antibodies were predictive of seizures and cerebrovascular accidents. CONCLUSIONS: In this longitudinal SLE cohort, significant cumulative NP damage occurred. Early aggressive therapy targeted towards NP manifestations may prevent the occurrence of NP damage.
The hY RNAs form a macromolecular complex with the 60-kDa Ro protein and may in addition be bound by the La protein. In this study, we examine the autoantibody responses to the intact protein-RNA complexes in 18 subacute cutaneous lupus (SCLE), 10 discoid lupus (DLE), and 18 neonatal lupus erythematosus (NLE) sera. All SCLE and NLE serum samples precipitated all four of the Ro hY RNAs, whereas none of the DLE serum samples precipitated the hY RNAs. Among the SCLE and NLE sera, there was a significant association between the amount of Ro hY RNAs precipitated and the concurrent presence of anti-La antibodies in the sera (p = 0.008), consistent with the hypothesis that both the 60-kDa Ro and the La proteins can bind the Ro hY RNAs. There was no correlation between the amount of hY RNAs precipitated and the titer of antibodies to the 60-kDa Ro protein in enzyme-linked immunosorbent assay (ELISA). This may be due to a difference in the epitopes formed by the antigen in the respective assays. The autoantibody response to Ro in SCLE and NLE, which is generally detectable by both immunodiffusion and ELISA, is directed to all the subsets of the Ro protein-hY RNA complexes. The autoantibody response in DLE, though frequently detectable by ELISA, is not of sufficient concentration or affinity to precipitate the Ro protein-RNA complexes. The autoantibody response to Ro in SCLE and NLE may include antibodies to epitopes created by the complexing of the 60-kDa Ro protein with hY RNA.
A 34-year-old male systemic lupus erythematosus patient (SLE) with cutaneous vasculitis developed renal failure after switching anti-nuclear antibody (ANA) specificity. He developed cutaneous lupus erythematosus with homogeneous and speckled type ANA and a high titer of anti-DNA antibody without renal involvement at 21 years of age. After developing lupus nephritis at the age of 27, the original ANA disappeared gradually. Two years later, a discrete speckled type ANA titer elevated abruptly to as high as 1:640 with low complementemia and without DNA antibody. Within five years, he suffered renal failure. This case of SLE suggests a direct correlation with ANA pattern and organ involvement.
A patient with systemic lupus erythematosus was first diagnosed after she had developed the Budd-Chiari syndrome. We believe that the Budd-Chiari syndrome was part of her tendency for thrombosis, induced by the presence of a lupus anticoagulant. The association between enhanced thrombosis and the lupus anticoagulant is discussed, and the need for prompt recognition and administration of anticoagulant therapy is emphasised.
A poor prognosis was observed in patients who had end-stage renal disease (ESRD) as a result of systemic lupus erythematosus (SLE). This was true even in patients in whom SLE disease activity was transiently quiescent during the period of hemodialysis. Six of 9 patients with ESRD and SLE died with active SLE and/or sepsis 1-28 months following the onset of dialysis. In 5 of the 6 patients, acute inflammatory activity of SLE flared within 1 month of the patient's death. Four patients died with superimposed sepsis, but only 2 of the 4 were receiving high-dose concomitant immunosuppressives for more than 1 week prior to death. Infected hemodialysis vascular access sites were implicated as the source of septicemia in 3 of 4 infectious deaths. The 3 surviving patients had minimal lupus activity prior to the development of ESRD, a possible marker for stability in SLE patients who require hemodialysis. Our results suggest that hemodialyzed lupus patients with nonautologous vascular access sites may be at continued increased risk for life-threatening inflammatory and septic complications.
The putative distinct diagnostic and pathogenic potential of aDNA-Ab subtypes, differing in their affinity or epitope specificity, was subject of several studies with controversial results. Comparing five assays, characterized by different reaction conditions and nature/source of dsDNA, we investigated the abovementioned problem in a retrospective study on 100 systemic lupus erythematosus (SLE) patients and 100 controls (other CTD, autoimmune hepatopathies). As demonstrated, only assay 3 (Farrzyme, TBS, UK) and 5 (Farr-RIA, Trinity Biotech, Ireland) are really suitable to detect primarily high avidity aDNA-Ab. Both were significantly linked to lupus nephritis (specificity 84%) and highly specific for SLE (95 and 96%). Thereby, assay 3 was found to be the first solid phase ELISA probably suitable to replace the Farr-RIA. Classical ELISAs (assay 1, Orgentec, Germany, and 2, Bindazyme, TBS, UK), detecting aDNA-Ab more or less independent from their avidity, or tests with only intermediate specificity for high avidity Ab (assay 4, ELIAdn, Sweden Diagnostics, Germany), were less specific for SLE (83, 79, 91%, respectively) and not associated with renal involvement (specificity 54-57%). At least in the patients studied here, obvious antigen-related differences could not be observed. With slight differences, all assays were suitable to monitor disease activity and therapy in SLE, agreeing with the ECLAM score in about 70-80% of cases. For lupus nephritis, aC1q-Ab are as specific as high avidity aDNA-Ab and capable to close a diagnostic gap in some cases. Thus, to enhance the specificity (up to 98%) and to consider the distinct diagnostic/pathogenic potential of aDNA-Ab subtypes in SLE, under routine clinical laboratory conditions it should be recommended to combine a sensitive screening test with a more specific second assay.
We describe two patients whose initial presentation of systemic lupus erythematosus (SLE) was accompanied by haemorrhagic episodes and significant coagulopathy. Further investigation demonstrated positive lupus anticoagulant and decreased Factor II (prothrombin) activity. Both patients were diagnosed with lupus anticoagulant-hypoprothrombinemia syndrome (LAC-HPS) as a result of non-neutralizing antibodies directed against Factor II. LAC-HPS is a rare clinical entity that can occur in association with SLE, transient viral infections, drug reactions or even in healthy individuals. Mixing studies, which can be affected by other coagulation factor inhibitors, play an important role in the diagnosis of LAC-HPS. Factor VII level was decreased in the second patient, a finding that has not previously been reported in association with SLE. In both patients, bleeding stopped promptly and coagulation studies improved significantly with high dose corticosteroids. We discuss the pathogenesis, diagnosis and management of LAC-HPS in patients with SLE.
The patient was a 13-year-old girl. In August 2000, she presented with a fever, together with diarrhea, vomiting, arthralgia, nasal bleeding and malaise, and was examined by another physician. Because her platelet count was low, and there were positive reactions for anti-nuclear antibodies, anti-DNA antibodies and platelet-associated IgG, idiopathic thrombopenic purpura, and systemic lupus erythematosus (SLE) was suspected. From January 2001, when she caught measles, she reported abdominal pain, and urinalysis indicated urinary protein and occult blood, and the left kidney was found hydronephrotic. At the same time left ureter stenosis and dilatation were demonstrated. Symptoms were disappeared by hydration and treatment with NSAIDs, but 2 months later fever and erythematous patches seen on both cheeks led to the proper diagnosis of SLE, and she was admitted to our hospital. Intravenous pyelography revealed hydronephrosis on left kidney, constriction and dilatation of the left ureter, and intracystic endoscopy showed erythema at the orifice of the left ureter. The pathological examination indicated the presence of vasculitis, and finally lupus cystitis was diagnosed. Intravenous cyclophosphamide (IVCY)-pulse therapy was introduced to a total of 8 times over the period of a year, and maintenance therapy with predonisolone and azathioprin was also used. After completion of the IVCY-pulse therapy, the hydronephrosis and constriction of the ureter were disappeared. No side effects of IVCY-pulses were observed, and the patient is now in remission. We reported a case of childhood SLE complicated with lupus cystitis and successfully treated by IVCY-pulse therapy and maintenance predonisolone and azathioprin.
Recent reviews have suggested a higher frequency of the lupus anticoagulant or related antiphospholipid antibodies in patients with systemic lupus erythematosus (21% to 65%) than was found in earlier studies (6% to 18%). In our study of 60 consecutive patients, we found the frequency of the lupus anticoagulant by Russell viper venom time was 6.7% (95% confidence interval, 16.2 to 1.8) and by anticardiolipin antibody assay was 25% (95% Cl, 37.0 to 15.7), compared with 0% (p = not significant) and 2.5% (p = 0.002), respectively, in the normal control population. The Russell viper venom time (p = 0.0001 by t-test) and anticardiolipin antibody levels (p = 0.01) were significantly associated with presumed thrombotic events (stroke, deep venous thrombosis, and digital gangrene). No association with miscarriage or pulmonary hypertension was detected. The Russell viper venom time was more specific than the anticardiolipin antibody level in the prediction of past presumed thrombotic events, miscarriage, or pulmonary hypertension (100% compared with 84%, p = 0.01).
The Lupus Anticoagulant (LA) is an immunoglobulin directed towards the phospholipid portion of the prothrombin activator complex. This immunoglobulin because first identified in the plasma of patients with Systemic Lupus Erythematodes, was named as Lupus Anticoagulant. Although initially described in patients with SLE, it was subsequently observed in other diseases and also in patients without any manifest disease. SLE or similar diseases are present in 35% of LA (+) patients. The LA prevalence in SLE patients has been found as 34% whereas ACA prevalence was found as 44%. While searching for the presence of LA and the levels of APA in cases having unexplained recurrent fetal losses, a family (a mother and 3 daughters) was discovered, whose each member has been diagnosed as SLE at different times and with different symptoms. Data suggesting the presence of LA and high APA levels were determined in all the members of the family and also it was realised that cause who had recurrent fetal losses had the highest APA levels. In addition to immunoassay methods to detect APA, examination of coagulation tests in patients with unexplained thrombosis and/or fetal losses, would be of great help.
In the practice of neurology, the type of clinical involvement suggests the site of the lesion and the mechanism beneath. Sometimes, the symptoms can be delusive, turning the diagnostic approach to a wrong path and raising the necessity of an algorithm considering the less probable entities. Dysimmunity as mechanism of neurological disease involving both the neuromuscular junction and peripheral nerves is frequently insidious and difficult to suspect on clinical basis alone. We report the case of a 67-year-old male with atypical Eaton-Lambert syndrome and mononeuropathy probably in relation with lupus-like entity. The patient has also high titers of anticardiolipin antibodies and lupus anticoagulant. We are considering the diagnostic algorithm before an isolated and atypical neurological presentation and reviewing the main neurological manifestations in lupus-like and autoimmune systemic disease. We raise the difficulty to classify an inflammatory connective tissue disease in the absence of other pathologic features than autoantibodies and isolated neurological symptoms and discussing the main therapeutic issues.
Severe hemorrhagic diathesis due to lupus anticoagulant complicated by hypoprothrombinaemia resulting from prothrombin autoantibodies is a rare disorder and is often associated with systemic lupus erythematosus (SLE). We report a case in which a 15-year-old girl with SLE developed marked haemorrhagic manifestations due to menorrhagia and nosebleeds. The acute bleeding episode was treated with SAGM, tranexamic acid and recombinant factor VIIa. Lupus anticoagulant, cardiolipin antibodies and antiprothrombin antibodies were successfully depressed within weeks after corticosteroid therapy was begun.
The frequency of "Lupus anticoagulant" (LA), was studied in 51 patients with systemic lupus erythematosus (SLE), 15 patients with chronic immune thrombocytopenic purpura (ITP) and 3 other patients with prolonged partial thromboplastin time (PTT), two of which had suffered episodes of CVA, and the other had a diagnosis of Paroxysmal Nocturnal Hemoglobinuria. Lupus anticoagulant was determined in each patient by the plasma recalcification time and the Russell's viper venom clotting time. Eight patients with SLE, (15.6%) 6 with chronic ITP (40%) and the three patients with prolonged PTT were positive for LA. All patients with LA were female, whose ages ranged from 19 to 59 years, and all except two patients were under steroid therapy. Thrombocytopenia was the most frequent manifestation in the patients with LA, followed by recurrent fetal death and thrombosis. Only the patients with ITP had hemorrhagic complications and one of them also had CVA in one occasion. The immunosupressory therapy may have played a role in diminishing the frequency of LA in the patients studied.