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A rare case of systemic lupus erythematosus with initial manifestation of myocarditis is reported here, found in a 24-year-old female who presented first with an episode of exertional dyspnea, later developing life-threatening arrhythmia, and left ventricular dysfunction. There were no classical clinical manifestations of SLE such as arthragia or malar rashes as initial symptoms. Subsequently, the only clue was traced with positive ANA screening test. Central nervous system manifestations were found and proteinuria was detected. We ordered intravenous "pulses" of methylprednisolone, followed by maintenance of daily glucocorticoid because the criteria of SLE seemed applicable. The patient responded well and rapidly. This initial manifestation for SLE is so rare and unusual that it has not previously been described.

Lupus is a mysterious disease without definite etiology and pathogenesis. The heterogeneous clinical manifestation of systemic lupus erythematosus (SLE) increases the delay and difficulty of diagnosis and the complexity of pathogenesis. Even in twins with identical genome and similar environmental milieu, the incidence of concordance for SLE is not high. Identical twins, concordant for SLE with discordance to disease onset and expression, are reported. Besides genetics, multiple factors are responsible for the pathogenesis of SLE.

A 20-year-old male was admitted with fever and hemoptysis. Agammaglobulinemia was found, with bronchiectasis and sinusitis. Clinical and laboratory evidence included immunological examinations, bone marrow and small intestinal biopsies. Results suggested a diagnosis of X-linked agammaglobulinemia. After treatment with antibiotics and intravenous human immunoglobuline, the clinical symptoms demonstrated progressive improvement. The case is reported along with a review of the literature.

Background: Systemic lupus erythematosus (SLE) has traditionally been considered a disease of women, and is uncommon in men. In recent years, several large clinical series of male lupus patients have been reported. As no known data are available for lupus in males from Taiwan, a retrospective analysis of data from male lupus patients was done to determine whether these patients differed from other series of male or female SLE patients in the literature., Methods: Sixty-one male lupus patients, diagnosed and followed in Tri-Service General Hospital, between 1983 and 1993, were studied and their data analyzed, retrospectively., Results: The mean age of diagnosis was 30 +/- 17 (mean +/- SD, range: 13-81) years. The peak age of diagnosis was between 13 and 40 years. The mean duration of follow-up was 36 +/- 36 (range: 2-256) months. The 1-, 5- and 10-year survival rates were 84%, 76% and 75%, respectively. The frequency of clinical manifestations were renal disease, 75%; malar rash, 70%; arthritis, 60%; fever, 56%; photosensitivity, 48%; pleuritis, 39%; pericarditis, 31%; alopecia, 31%; mucosal ulcers, 29%; neuropsychiatric disease, 26%; discoid lupus, 21%; vasculitis, 15%; Raynaud's phenomenon, 10%; and lymphadenopathy, 2%. The frequency of abnormal laboratory findings were antinuclear antibodies (ANA), 95%; hypocomplementemia, 77%; antibodies to double-stranded DNA (anti-dsDNA), 57%; leukopenia, 44%; lupus erythematosus (LE) cells, 39%; anti-Ro, 39%; anti-Smith antibodies (anti-Sm), 19%; thrombocytopenia, 18%; rheumatoid factor, 17%; anti-ribonucleoprotein antibody (anti-RNP), 14%; autoimmune hemolytic anemia, 8%; false-positive venereal disease research laboratory test (VDRL), 6% and anti-La, 4%., Conclusions: In a review of the 61 ethnic Chinese male lupus patients, a higher frequency of renal disease, malar rash and photosensitivity, but a lower frequency of arthritis and lymphadenopathy, compared to previous reports of Caucasians. There were no significant immunological differences from other series of male lupus, except a lower frequency of anti-dsDNA. In general, poor prognosis was noted for male lupus patients here.

A patient with ankylosing spondylitis and coexisting IgA nephropathy and leukocytoclastic cutaneous vasculitis is described. Renal biopsy demonstrated mesangial proliferative glomerulonephritis with prominent IgA, C3 and fibrin deposition in the glomeruli. Simultaneously, leukocytoclastic cutaneous vasculitis with prominent IgG, IgA and C3 deposition of dermal vessel wall was also observed in the skin biopsy specimen. Such associations have been previously reported in only four cases. This report once again indicates that antigenic mucosal stimulation may play an important role in the pathogenesis of ankylosing spondylitis.

Central nervous system (CNS) toxoplasmosis is an important infectious complication of acquired immunodeficiency syndrome (AIDS) which appears to result from reactivation of a previously acquired infection and requires prolonged treatment. A 31-year-old male presented in a drowsy mental state and with an unstable gait. Computerized tomographic (CT) scan and magnetic resonance imaging (MRI) showed multiple nodular lesions in the cerebrum and cerebellum; the seropositivity for the human immunodeficiency virus (HIV-1) and high serum IgG toxoplasma titers were also demonstrated. A presumptive diagnosis of CNS toxoplasmosis was based on neurological signs and neuroradiological findings. This was confirmed by improvement in both clinical and neuroradiological pictures during treatment with pyrimethamine and clindamycin. Four months later, however the patient died of intracranial hemorrhage and massive upper GI bleeding.

Background: From both a microscopic and a metabolic view, experimental animal models are very important for study of the pathogenesis of osteoarthritis. A new, different operative procedure was used in rabbit models, and the pathologic findings were evaluated., Methods: Twelve New Zealand white rabbits were divided into six groups; eight were used as animal models and four, for drug efficacy study. Transection of the anterior cruciate and medial collateral ligaments on the left knee joint, and sham-operation were performed on the right knee joint. Rabbits were sacrificed post surgery from 4, 6, 8 and 12 weeks. Eight parameters from gross to microscopic findings were used in order to evaluate osteoarthritic changes. Indomethacin and aspirin were chosen for the drug efficacy experiment; the two rabbits of each group were sacrificed at the end of the sixth week post-surgery., Results: According to pathological findings, this operative procedure can produce osteoarthritic changes, visible both microscopically and macroscopically. There were osteoarthritic changes in the fourth week post-surgery group and, obviously, in the eighth week; these persisted until 12th weeks post-surgery. Neither indomethacin nor aspirin showed any effect in preventing osteoarthritis progression., Conclusions: Transection of the anterior cruciate and medial collateral ligament rabbit model can produce osteoarthritic lesions in the knee joint. This model can be used for further biochemical and metabolic studies.

The association of malignant lymphoma with positive antinuclear antibody is uncommon, and their relationship is not clear. Here a 35-year-old man is presented who initially had a high titer of antinuclear antibody; later, peripheral T cell lymphoma was found. It is suggested than an unexplained high antinuclear antibody titer may warrant searching for a malignancy.

Background: Renal involvement in systemic lupus erythematosus commonly leads to renal failure and death. We conducted a study to evaluate the efficacy and side effects of intermittent intravenous treatment with cyclophosphamide of patients with lupus nephritis., Methods: Twenty-four patients with lupus nephritis were recruited at Tri-Service General Hospital from 1988 to 1992. Cyclophosphamide was administered intravenously monthly for three months, and then every three months. 24-hour urinary protein, creatinine clearance, serum creatinine, blood urea nitrogen, C3 and C4 levels, serum albumin, hemoglobin, and dosage of prednisolone were recorded before each treatment. During treatment, the side effects were monitored., Results: Two patients progressed to renal failure within one year and one patient after three years. Fifteen patients completed therapy for one year. Among these 15 patients, the levels of hemoglobin, serum albumin, and C3 significantly increased at six months, then became stable; the level of C4 increased at 12 months. In contrast, the dosage of prednisolone decreased significantly at six months. There was no significant difference of creatinine clearance, 24-hour urinary protein, serum creatinine, and blood urea nitrogen before and after treatment. The most common side effects were nausea and vomiting. No severe side effect necessitated discontinuing therapy with cyclophosphamide., Conclusions: Intermittent intravenous therapy in lupus nephritis with cyclophosphamide can significantly increase levels of hemoglobin, serum albumin, C3 and C4, and keep renal function stable. Poor response was found in a subset of patients. The side effects were mild in the present study.

Background: The influence of renal morphology and clinical factors at biopsy on the development of renal failure in patients with lupus nephritis remain controversial. We investigated the relation between renal histologic finding and clinical manifestations, and evaluated prognostic factors and short-term prognosis among patients with lupus nephritis., Methods: Seventy patients with lupus nephritis were enrolled in the study from 1982 to 1992 at the Tri-Service General Hospital. Renal biopsy specimens from these patients were assessed according to the World Health Organization (WHO) classification, activity and chronicity indices, and clinical parameters. Survival was analyzed by using the day of renal biopsy as the starting point. The end point of renal survival was the date when patient started to receive regular hemodialysis., Results: In pathological finding, one patient was grouped as Class I (1.4%); 14, as Class II (20%); 15, as Class III (21.4%); 29, as Class IV (41.4%); 9, as Class V (12.9%), and 2, as Class VI (2.9%). The scores of activity indices were highest in Class IV. The blood levels of C3 and C4 in Class V were significantly higher than Class IV. The values of BUN and 24-hour urine protein in Class II were significant lower than Class IV. Patients who progressed to renal failure had significantly higher numbers of death, higher serum creatinine and chronicity index, less creatinine clearance, and higher numbers of hypertension at the time of biopsy. Nephrotic syndrome was not associated with renal failure. Patient and renal survivals did not differ among WHO classifications. The patient and renal survivals were 84%, 60% and 85%, 72% at one and five years, respectively. Seventeen patients (24.2%) progressed to end-stage renal disease and 21 patients (30%) died during the study period. The leading causes of death were sepsis and renal failure., Conclusions: WHO classification had little correlation with clinical and renal information. At the time of biopsy the elevated serum creatinine and hypertension were good predictors for end-stage renal disease. Poor patient and renal survivals were found in this study.