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A case of chilblain lupus erythematosus during pregnancy is described in a 28-year-old woman. Symptoms and signs were present by 8 weeks of her first pregnancy and had resolved by 10 days after delivery. The condition is discussed with particular emphasis on the relationship with pregnancy.
A case of systemic lupus erythematosus with deforming hand arthropathy (DHA) is reported in a woman observed for 15 years. DHA was presented with stable ulnar deviation, deformation of the fingers. This resulted in functional insufficiency of the hand of the third degree.
Systemic lupus erythematosus (SLE) is an inflammatory disease characterized by deposition of autoantibodies and pathogenic immune complexes in the cells and tissues of an organism, causing lesions. The heart is one of the most frequently involved organs, valvar involvement being common (27%) and of great clinical importance. Some studies suggest that valvar involvement is associated with the presence of a specific autoantibody, the antiphospholipid antibody (aPL). In these circumstances, major complications may occur such as arterial or venous thrombosis, thrombocytopenia, repeated spontaneous abortions and presence of lupus anticoagulant antibody. Among the semiologic methods for cardiac evaluation, echocardiography is the main instrument for investigating the anatomical and functional involvement of the heart in the great majority of systemic diseases like SLE. We report the case of a patient with SLE and aPL syndrome who underwent surgery for mitral valve replacement with a biological prosthesis and died nine months later due to massive intracardiac thrombosis and prosthesis dysfunction.
A patient with systemic lupus erythematosus (SLE) developed attacks of unilateral cataplexy precipitated by laughter. Unilateral cataplexy has not been described previously in detail and its association with SLE is unique. The clinical details, investigations, and diagnostic criteria are discussed and a causal relationship between cataplexy and SLE is suggested.
Two patients with systemic lupus erythematosus had aortic valvular stenosis resulting from massive thrombotic deposits on the valve. This condition has not been described previously, but a similar process has been reported to have caused mitral stenosis in a patient.
Four patients with systemic lupus erythematosus (SLE) developed an unusual form of occlusive retinal arterial disease. The most prominent clinical features of this disorder were deposition of yellow-white material in retinal arterial walls and evidence of multifocal retinal arterial occlusion. Fluorescein angiographic findings included nonperfusion of the obstructed arteries and the retinal capillary beds fed by them, and fluorescein leakage at the sites of involvement of the retinal arteries. This ocular complication of SLE is presumably a manifestation of the widespread systemic vascular problems seen in this disorder. It may be more common in patients with lupus involving the CNS.
Two patients with systemic lupus erythematosus (SLE) and hyperkalemia were studied. The hyperkalemia was disproportionate to the degree of renal excretory impairment. The usual causes of hyperkalemia were excluded. Basal levels of plasma renin activity (PRA) and plasma aldosterone (PAC) were low. The responses of PRA and plasma aldosterone to the combined stimulus of ambulation and furosemide were blunted. Plasma levels of 18-hydroxycorticosterone (18-OH-B) were normal. The hyperkalemia in both patients could be attributed to hyporeninemic hypoaldosteronism (HH). In one patient, the hyperkalemia was corrected by the administration of fludrocortisone. In the second patient, treatment of lupus nephritis with azathioprine, prednisone, and plasmapheresis normalized both the serum creatinine and the serum potassium.
Two patients with systemic lupus erythematosus developed monarticular infectious arthritis, in which Neisseria meningitidis was recovered from knee synovial fluid. In one instance, the sole manifestation of meningococcal disease was a chronic, indolent, erosive monarthritis. In the second, a febrile, bacteremic illness presented with an acute, septic arthritis.
The diagnosis of systemic lupus erythematosus (SLE) often depens on positive tests for antinuclear antibody, LE cells, and antibody to DNA. In fact, the presence of such serological markers is virtually required for the diagnosis of active disease. This report describes 5 well-studied patients with SLE who initially presented with negative serology despite active multisystemic disease. When followed from 10 months to 7 yr, negative serologic markers, including the ANA, LE cell test, and anti-DNA were seen to change from negative to positive, analogous to the seronegative rheumatoid patient who may become seropositive. In a few cases of lupus, therefore, negative serologic tests do not always exclude SLE, and serial testing at periodic intervals may be indicated.
A patient with systemic lupus erythematosus developed acute mitral regurgitation due to ruptured chordae tendineae, requiring mitral valve replacement. Typical changes of Libman-Sacks endocarditis were observed in the excised mitral valve. Immunofluorescent studies revealed antinuclear antibody and deposits of immunoglobulins and complement within small vessels.
The autoimmune disease systemic lupus erythematosus (SLE) is caused by a failure of B cell tolerance. Recent studies in mouse models of SLE have identified several distinct tolerance checkpoints that must each function appropriately to protect against disease. However, studies of B cell repertoire selection in humans are essential to understand which checkpoints are defective in human autoimmune diseases.
A patient with systemic lupus erythematosus (SLE)/erosive arthritis overlap is described who was also shown to have late latent treponemal infection. The possibility of serological reactivation is discussed. Since antiphospholipid antibodies are the basis of the reagin tests for syphilis as well as being present in patients with SLE, difficulties with diagnosis may ensue.
The association between systemic lupus erythematosus and human immunodeficiency virus infection is extremely rare. We report a case of this association and discuss the possible reasons for its rarity, together with the difficulties encountered in the interpretation of the clinical and laboratory features.
Four patients with systemic lupus erythematosus (SLE) had a vesicobullous eruption that histologically resembled dermatitis herpetiformis. Immunofluorescence microscopy patterns in the skin studied in three patients, however, were characteristic of SLE. Treatment of the underlying collagen-vascular disease improved the eruption, while recurrence coincided with exacerbation of the systemic disease. This distinctive lesion is an important parameter of SLE activity.
An adolescent female with systemic lupus erythematosus (SLE) with diffuse proliferative lupus nephritis subsequently contracted Hodgkin's lymphoma, mixed cellularity type. Prior to the diagnosis of Hodgkin's lymphoma, hypogammaglobulinemia developed with IgG deficiency. The nephritis, which improved on steroid therapy, remitted entirely after nitrogen mustard, vincristine, procarbazine, and prednisone (MOPP) therapy. The hypogammaglobulinemia also remitted after chemotherapy for Hodgkin's lymphoma. The literature concerning the association of SLE and Hodgkin's lymphoma is reviewed. The significance of this concurrence is discussed in regard to the possible pathophysiology and the clinical management.
Three cases of systemic lupus erythematosus (SLE) with clinical presentation similar to a rheumatic polymyalgia (RP) are described. There was no evidence of underlying myopathy in any of the cases thus suggesting that the synovitis is the pathogenic mechanism. The late onset and the poor serologic expression are the relevant clinical signs which could delay or hinder the diagnosis.
A combination of systemic lupus erythematosus (SLE) and fatal pulmonary hypertension occurred in a patient who, to our knowledge, had the highest pulmonary artery pressure (120/65 mm Hg) reported without any clinical or autopsy findings of pulmonary interstitial disease or vasculitis. The gradual development of pulmonary hypertension over years is a rare complication in patients with SLE.
Eleven patients with systemic lupus erythematosus (SLE) also had headaches and/or visual hallucinations typical of those found in migraine. These migrainous symptoms were commonly associated with exacerbations of SLE and abated as disease activity subsided. In some cases corticosteroids were more effective than conventional antimigraine therapy in controlling headaches and scotomas. The data suggest that migraine-like phenomena may arise as a result of vascular dysfunction in SLE.