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Acute sarcoidosis occurring during the course of systemic lupus erythematosus.

A diagnosis of systemic lupus erythematosus (SLE) was established in a 54-year-old woman with a compatible history, as well as patchy alopecia, anemia, arthralgias, and a positive LE cell preparation. Sixteen months later bilateral hilar and mediastinal lymph nodes appeared on chest roentgenogram. Sarcoidosis was diagnosed when hypercalcemia and noninfectious, noncaseating epithelioid granulomas were found in the skin and liver. The sarcoidosis remitted with corticosteroid therapy, but slowly advancing renal failure ultimately resulted in the patient's death. We believe the concurrence of SLE and sarcoidosis had not been previously reported in the English literature. Immune mechanisms are discussed.

Female↗

Studies of the pathogenesis of interstitial cystitis, obstructive uropathy, and intestinal malabsorption in a patient with systemic lupus erythematosus.

A patient with systemic lupus erythematosus (SLE) is described who displayed the unusual extraglomerular syndromes of interstitial cystitis with obstructive uropathy and severe secretory-type diarrhea with intestinal malabsorption. Pathologic investigations into the nature of these events revealed immunofluorescent deposits in blood vessel walls of the small intestine and urinary bladder. The patient's malabsorption state and cystitis with urinary obstruction regressed with therapy of the SLE. We are postulating that immune complexes from the circulation may deposit in blood vessel walls, and may be responsible for tissue injury in these sites and the clinical manifestations of this patient.

Adult↗

Plasmapheresis-immunoadsorption for treatment of systemic lupus erythematosus in a dog.

Systemic lupus erythematosus was diagnosed in a 3-year-old, male German Shepherd Dog. Clinical signs included lethargy, partial anorexia, fever, joint swelling, and skin and oral ulceration. The diagnosis was confirmed on the basis of an antinuclear antibody titer of 1:640 and immunofluorescence for immunoglobulin G at the dermal-epidermal junction in skin biopsy specimens. Treatment with prednisone and cyclophosphamide failed to induce remission. Intensive plasmapheresis-immunoadsorption, using purified Staphylococcus protein A in combination with low-dose prednisone therapy, resulted in sustained remission.

Animals↗

[Use of intravitreal ganciclovir for cytomegalovirus retinitis in a patient with systemic lupus erythematosus].

A patient with systemic lupus erythematosus (SLE) developed bilateral cytomegalovirus (CMV) retinitis and was treated with a total of 9 intravitreal ganciclovir injections at a dose of 400 micrograms/50 microliters in each eye under topical anesthesia. She was unable to receive systemic antiviral therapy because of bone marrow suppression and renal failure. The condition of both eyes improved after the intravitreal injections. Recurrence of CMV retinitis was not seen until death. Intravitreal injections of ganciclovir are useful even for the non-acquired immunodeficiency syndrome (AIDS) immunocompromised patients with CMV retinitis when the patients can not receive intravenous medications because of systemic complications.

Antiviral Agents↗

Peripheral neuropathy in systemic lupus erythematosus.

The prevalence of clinically apparent peripheral neuropathy in systemic lupus erythematosus is reported to be between 2% to 18%. The purpose of this prospective case-control study was to determine the prevalence of peripheral neuropathy (PN) using electrodiagnostic criteria. Subgroup analysis was performed to determine whether PN correlated with disease activity, renal involvement, or serum immune markers. Fifty-four systemic lupus erythematosus patients and 30 controls were recruited in the study. The right median, ulnar, peroneal, tibial, and sural sensory and motor nerve conduction studies were obtained. PN in our study was defined as any abnormal values in motor and sensory distal latency, sensory action potential, motor action potential, or conduction velocity affecting 2 or more nerves. Of the 54 patients studied, PN was present in 15 patients (27.8%) of which 4 were symptomatic. There was a significant correlation between PN and anti-SM antibody, and there was a trend showing decreased motor and sensory action potential amplitudes in our systemic lupus erythematosus group compared to the controls. This observation was also seen in an active disease group when compared to those with inactive disease. The amplitude of the action potential was more often affected than the distal latency, and sensory nerves were more susceptible than motor nerves. The sural sensory action potential amplitude appears to be the most sensitive indicator of PN which may be used as an index to monitor disease activity.

Adolescent↗

Hyperlipoproteinemia and multifocal neurologic dysfunction in systemic lupus erythematosus.

Two patients with systemic lupus erythematosus, including one with neuropathologic findings, had recurrent multifocal neurologic dysfunction and hyperlipoproteinemia. The lipoprotein disturbances were complex and variable over time. Deficient lipoprotein lipase was found in both patients and appeared to be related temporally to neurologic deterioration. One of these patients had neurologic disease and lipoprotein abnormalities 2 1/2 years before SLE could be documented serologically. These studies suggest that lipoprotein lipase deficiency may be a marker for the endothelial disorder causing cerebral vasculopathy in SLE.

Adolescent↗

Bilateral phrenic paralysis in a patient with systemic lupus erythematosus.

Respiratory manifestations of systemic lupus erythematosus (SLE) are frequent. They include respiratory muscle abnormalities, which have been implicated in the pathogenesis of the "shrinking lung syndrome" (SLS). We report the case of a patient with this syndrome, in whom diaphragmatic paralysis due to demyelinating phrenic lesions was diagnosed at the same time as SLE. Follow-up studies showed a favorable clinical and diaphragmatic outcome with corticosteroid therapy, but little change in spirometry. It is concluded that severe diaphragm palsy is possibly due to phrenic nerve lesions in SLE, and that the link between diaphragm dysfunction and the SLS is probably not a straightforward one.

Adult↗

Immune complexes in the pericardial fluid in systemic lupus erythematosus.

Two patients with systemic lupus erythematosus (SLE) had pericardial tamponade. The pericardial fluid from both patients contained "mixed" type cryoglobulins consisting of IgG, IgM, and C1q. There was a selective concentration of antinuclear antibodies in the pericardial fluid cryoglobulin of one patient. Pericardial fluid from the same patient contained immune complexes. Anti-DNA antibodies were found in the serum, pericardial fluid, and pericardial fluid cryoprotein. Immune complexes isolated from the pericardial fluid by polyethylene complexes isolated from the pericardial fluid by polyethylene glycol precipitation contained antinuclear antibodies including anti-DNA antibodies. Immune complexes may be important in the pathogenesis of pericarditis in SLE.

Adult↗

Atypical cutaneous findings in a patient with systemic lupus erythematosus.

A previously diagnosed systemic lupus erythematosus patient presented with arthralgia, skin rash and muscular weakness. When treated with high-dose corticosteroids and methotrexate she improved, except for a persistent lesion in the hand which evolved into a profound ulcer, along with tender subcutaneous nodules in the calf. A skin biopsy disclosed necrotizing vasculitis with giant cell granuloma revealing acid fast positive bacteria on ziels nilsen staining. A chest X-ray disclosed miliary tuberculosis (TB). The patient was diagnosed as miliary TB with prominent cutaneous involvement and treated with four anti-tuberculous drugs with slow resolution of her systemic, pulmonary and skin signs.

Adult↗

Clinical features of chorea associated with systemic lupus erythematosus.

Neurologic complications of systemic lupus erythematosus (SLE) are common, but chorea is rare. Three cases of chorea associated with SLE are presented as well as a review of 28 cases from the world literature. Chorea may be the first and at times the only sign prior to the establishment of diagnosis of SLE. Under such conditions the establishment of the correct diagnosis is difficult, and the chorea may be attributed to a more common etiology, Also the SLE-related chorea may recur in the same subject during the course of the disease. Finally, the chorea associated with SLE can be brought under control by use of haloperidol (HALDAL).

Adult↗

[Systemic lupus erythematosus in children].

Pediatric systemic lupus erythematosus (pSLE) is a chronic mutisystemic autoimmune disease with complex clinical manifestations. Although the presentation, clinical manifestations, immunological findings and treatment issues of pSLE are similar to those of adult SLE patients, there are special issues which need to be considered when dealing with SLE in children. During the last decade survival has improved remarkably as a result of earlier diagnosis, recognition of milder disease and better approaches to therapy. However, pSLE remains a potentially serious condition. Although the pathogenesis of SLE remains poorly understood, susceptibility involves a combination of environmental, hormonal and genetic factors. Better understanding of SLE pathogenesis will hopefully lead to more specific and less toxic therapies for this disease.

Child↗

Minimal-change nephrotic syndrome associated with systemic lupus erythematosus.

A case of systemic lupus erythematosus (SLE) associated with minimal-change nephrotic syndrome (MCNS) is described. A 41-year-old woman with SLE presented with symptoms of nephrotic syndrome. Renal biopsy revealed minor glomerular abnormalities without the deposition of immune complexes. The initial heavy proteinuria promptly decreased after the prednisolone dosage was increased and disappeared 4 weeks later. The patient had a relapse of nephrotic syndrome without exacerbation of immunoserological reactions when the prednisolone dose was subsequently decreased. Remission was achieved 5 days after methylprednisolone pulse therapy. T cell dysfunction, which is present both in SLE and MCNS, might have triggered MCNS during the course of SLE.

Adult↗

Polymyositis and polyarthritis associated with systemic lupus erythematosus in a dog.

Systemic lupus erythematosus with polymyositis and polyarthritis was diagnosed in a 7-year-old female Standard Poodle. Pertinent clinical signs included extreme muscular weakness, muscle wasting, atrial fibrillation, and dysphagia due to megaesophagus. Aspiration pneumonia secondary to the megaesophagus contributed to the death of the dog. Serum muscle enzyme activities were increased. Electromyographic findings included fibrillation potentials, positive sharp waves, increased insertional activity, and bizarre high-frequency repetitive potentials. Histopathologic findings in skeletal muscle included myofiber necrosis and phagocytosis; regeneration of myofibers; perivascular and interstitial infiltrations of macrophages, lymphocytes, and plasma cells; and type I and II myofiber degeneration and vacuolation.

Animals↗

Concomitant development of chronic active hepatitis and antibodies to ribosomal P proteins in a patient with systemic lupus erythematosus.

We describe a systemic lupus erythematosus (SLE) patient who for several years had typical SLE features and positive antinuclear antibodies (ANA), including anti-native DNA. Over the course of a year, 4 years after the SLE was diagnosed, the ANA disappeared and antibodies to cytoplasmic component ribosomal P protein (anti-P) appeared. Associated with the appearance of anti-P antibodies was the development of biochemical evidence of liver disease, later shown histologically to be chronic active hepatitis. The temporal relationship between the occurrence of anti-P antibodies and the development of liver disease raises the possibility of a role for anti-P antibodies in liver disease.

Adult↗

Laryngeal complications in a patient with inactive systemic lupus erythematosus.

Laryngeal complications in systemic lupus erythematosus (SLE) are rarely described. They range from hoarseness to life-threatening respiratory distress. To our knowledge, previous reports describe laryngeal involvement with SLE occurring only during periods of active disease. We saw a patient with inactive SLE in whom hoarseness and exertional dyspnea developed as a result of arytenoiditis and vocal cord paresis during steroid tapering. The condition responded dramatically to readjustment of her steroid dosage. Involvement of the larynx with SLE is a potentially life-threatening complication and may occur in patients with either active or inactive disease. It is an indication for close observation and steroid therapy in patients with SLE.

Airway Obstruction↗

Efficacy of topical tacrolimus for treating the malar rash of systemic lupus erythematosus.

The rash of systemic lupus erythematosus (SLE) is usually treated with topical corticosteroids, but prolonged use causes adverse cutaneous side-effects. We assessed the efficacy of topical tacrolimus for treating the skin lesions of SLE. Three patients with SLE affecting their facial skin applied 0.1% tacrolimus ointment on one side of their face twice daily for 3 weeks, in conjunction with a sunscreen cream. After 3 weeks, erythema on the treated side was ameliorated in all three patients compared with the untreated side. Although the study is preliminary, the results demonstrate that topical tacrolimus may be useful for treating the malar rash of SLE.

Adult↗

Acquired hemophilia A in a patient with systemic lupus erythematosus.

A patient with systemic lupus erythematosus (SLE) developed acquired hemophilia A. The patient, a 24-year-old Japanese woman, was referred to our hospital because of uncontrollable bleeding following a tooth extraction. Laboratory examination revealed prolonged APTT (116 seconds), reduced factor VIII activity (2.8 %) and the presence of factor VIII inhibitor at a titer of 46.5 Bethesda units/ml. Transfusion of prothrombin complex concentrate and activated prothrombin complex concentrate followed by administration of prednisolone and cyclophosphamide successfully arrested bleeding and reduced the factor VIII inhibitor level. Acquired hemophilia A is a rare but lethal condition. Rapid diagnosis and introduction of adequate therapies are critical.

Adult↗

[Aortal defects in systemic lupus erythematosus].

Five patients with significant lupus erythematosus (SLE) are described. The use of ECG, PCG and ultracardiosonography made it possible to verify the diagnosis of aortal valvular disease. On recognition of the damage to the aortal valves the question arises as to the genesis of its occurrence: does it occur because of the lupoid process proper or superaddition of bacterial endocarditis? The similar signs of SLE and bacterial endocarditis are described as are differential and diagnostic criteria. The main mechanisms by which the aortal valvular disease develops in SLE are depicted. Four out of the 5 patients described had bilateral sacroileitis and were not HIA-B27 carriers.

Adult↗