HYPOSPLENISM IN SYSTEMIC LUPUS ERYTHEMATOSUS

Hyposplenism, which is suggested by a typical peripheral bloodsmear and by the absence of splenic activity in a ^99m To sulphurcolloid scan, has been recently found to be associated withvarious diseases. This condition increases the susceptibilityof patients to certain bacterial infections principally by pneumococci,meningococci and Haemophilus influenzae. The association ofSLE and hyposplenism has not often been reported before; thuswe see fit to report another such case. The administration ofpolyvalent pneumococcal vaccine is recommended in this condition. KEY WORDS: Hyposplenism, Systemic lupus erythematosus     

SEVERE THORACIC SYSTEMIC LUPUS ERYTHEMATOSUS

The effects of corticosteroid administration to eight patients with severe thoracic systemic lupus erythematosus (SLE) were assessed in a prospective study over a mean period of 26 months by serial measurements of respiratory function, ESR, and a clinical score. Initial assessment of respiratory function showed severe restrictive ventilatory defects and impairment of carbon monoxide uptake not wholly attributable to the small lung volumes. ESR and clinical score showed high correlation coefficients with FEV₁ in all patients, with VC and TLC in seven patients, and with T_LCO in four patients, indicating that changes of respiratory function were reflecting the activity of the disease. This study shows that in patients with severe thoracic SLE it is valid to use serial measurements of respiratory function to assess the response to treatment and that pronounced and sustained improvement of respiratory function can be expected.     

SYSTEMIC LUPUS ERYTHEMATOSUS PRESENTING AS PSEUDOCYESIS

A patient with SLE whose initial manifestation was with a raredelirious disorder manifested as pseudocyesis is described.The psychiatric manifestations were considered to be due toSLE and treated with pulse steroid therapy. Special attentionis focused on treatment and MRI. KEY WORDS: Systemic lupus erythematosus, Pseudocyesis, Neuropsychiatric lupus, Central nervous system     

SYSTEMIC LUPUS ERYTHEMATOSUS AND ABDOMINAL PAIN

Abdominal pain is a common symptom in systemic lupus erythematosus.We report two patients with recurrent severe attacks of abdominalpain, often necessitating hospital admission, which were notdiagnosed until laparotomy. In one an active arteritis of themesenteric arteries was seen, with infarction of the jejunumand ileum, and in the second infarction of the ileum with haemorrhageand conglutination of red cells, but no true arteritis. KEY WORDS: Systemic lupus erythematosus, Abdominal pain     

PROTEIN-LOSING ENTEROPATHY IN SYSTEMIC LUPUS ERYTHEMATOSUS

Two patients with systemic lupus erythematosus (SLE) presented with anasarca, pleural effusions and severe hypoalbuminema, Both were demonstrated to have protein-losing enteropathy, a rare complication of SLE. Other causes of gastrointestinal protein loss were excluded. There were marked similarities in both cases including circulating ANF with speckled staining, anti-(U1)RNP antibodies and low serum complement levels. Complete remission was achieved in both with prednisolone. Anti-(U1)RNP may be a marker for a subset of SLE in which protein-losing enteropathy is a major manifestation.     

NEUROPSYCHIATRIC MANIFESTATIONS OF SYSTEMIC LUPUS ERYTHEMATOSUS

Forty-six episodes of major neuropsychiatric manifestations of systemic lupus erythematosus occurring in 35 patients over a ten-year period were reviewed. The frequency of central nervous system SLF was lower than that reported from overseas referral centres. Glucocorticosteroid treatment did not precipitate psychiatric problems in this group. Whilst neuropsychiatric recovery was the rule, such features indicated a subgroup of SLE with high short-term mortality.     

LUPUS AND NON-LUPUS CUTANEOUS MANIFESTATIONS IN SYSTEMIC LUPUS ERYTHEMATOSUS

Abstract Mucocutaneous manifestations occur frequently in systemic lupus erythematosus (SLE). Common non-lupus dermatoses may be confused with lupus rashes, with important clinical consequences. A study of 84 consecutive patients with SLE was performed to determine the comparative frequency of lupus and non-lupus mucocutaneous abnormalities, the comparative sensitivity of routine histology and immunofluorescence in the diagnosis of lupus rashes, and the association of skin manifestations with other clinical and serological features. Thirty-five patients had dermatoses attributable to SLE (mean 3.7 per patient) and 58 had derrnatoses which were not directly attributable to SLE (mean 2.1 per patient), highlighting the need for accurate diagnosis of skin rashes in SLE patients. Routine histology confirmed the clinical diagnosis of typical cutaneous lupus in 100% of malar lupus rashes and in approximately 90% of subacute cutaneous and discoid lupus rashes. Direct immunofluorescence of the affected skin provided supportive evidence of cutaneous lupus in 60% of malar lupus rashes and approximately 50% of subacute cutaneous and discoid lupus rashes. This reaffirmed the poor sensitivity of immunofluorescence, compared with routine histology, in the diagnosis of lupus rashes. The association of subacute cutaneous lupus with anti-Ro antibodies was confirmed. (Aust NZ J Med 1987; 17: 501–506).     

GOUT AND HYPERURICAEMIA IN SYSTEMIC LUPUS ERYTHEMATOSUS

Coexistent gout and systemic lupus erythematosus (SLE) havereceived increasing attention. An additional case is reportedand published cases reviewed. Common clinical features of thisgroup include nephritis, diuretic and corticosteroid therapyand a tendency to be older and more frequently male. Prevalenceof hyperuricaemia (HU) was assessed in 38 patients with SLE.HU was found in 29% and was closely associated with renal involvementparticularly proteinuria and diuretic therapy. The presentationof gout in SLE may be modified or suppressed by anti-inflammatorytherapy and may be misinterpreted as SLE arthritis. KEY WORDS: Hyperuricaemia, Diuretics, Renal failure, Steroids     

CO-EXISTENT TOPHACEOUS GOUT AND SYSTEMIC LUPUS ERYTHEMATOSUS

The association of gout and systemic lupus erythematosus (SLE)is extremely uncommon and, to date, only one such case has beenreported. We describe a patient who, 12 years after the onsetof SLE, developed tophaceous olecranon bursitis ^*Present address: Salisbury General Infirmary, Salisbury, Wilts.SP2 7SX.     

ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES IN SYSTEMIC LUPUS ERYTHEMATOSUS

Antineutrophil cytoplasmic antibodies (ANCA) with specificityfor proteinase-3 (PR3) are associated with Wegener's granulomatosis,and ANCA directed to myeloperoxidase (MPO) with other idiopathicvasculitides. Inflammation of small-sized blood vessels is ahallmark of systemic lupus erythematosus (SLE). We evaluatedthe prevalence of ANCA in SLE, their antigenic specificities,and their possible relation to clinical disease patterns andactivity. Plasma samples from 84 patients with SLE were testedfor ANCA during remission. Plasma samples from the 25 patientswho relapsed during a follow-up of 32 months were serially analysedfor ANCA in a 6 month period preceding and including the relapse.The presence of ANCA was assessed by indirect immunofluorescence(IIF) and ELISA for antibodies to PR3, MPO, lactoferrin (LF),elastase (HLE) and cathepsin-G (CG). We related the presenceof ANCA to disease patterns, activity and duration. ANCA byIIF were difficult to interpret due to the presence of antinuclearantibodies (ANA). By ELISA, we found no anti-PR3 or anti-HLE.Anti-MPO (n = 7), anti-LF (n = 13) and anti-CG (n = 10) weredetected, generally in low titres. The presence of ANCA of definedspecificity was not associated with specific clinical subsets.The prevalence of ANCA was higher in patients who developedrelapses than in those who did not (P < 0.01). However, levelsof ANCA did not fluctuate in the period preceding the relapse.ANCA of various specificities occur in SLE. Their presence isnot associated with specific clinical disease entities. Thehigher frequency of ANCA in relapsing patients compared to thosewho do not relapse may suggest that ANCA are involved in diseaseexpression. Their diagnostic significance is limited. KEY WORDS: ANCA, SLE, Disease activity, ELISA     

晚发系统性红斑狼疮的临床特点分析

目的分析晚发系统性红斑狼疮（SLE）患者的临床表现及治疗情况,并与早发SLE患者进行比较,以探讨晚发SLE的临床特点。方法收集2000～2004年于我院就诊的晚发SLE患者21例,并随机选取了同期40例发病时年龄〈50岁的患者作为早发SLE组,分析比较两组的临床表现、实验室指标、治疗情况。结果晚发SLE占所有SLE患者的6．18％。晚发SLE组性别比例女：男差异有统计学意义（3．2,P〈0．01）,且一些临床表现较少见：关节炎（P〈0．01）;蝶形红斑（P〈0．05）;肾脏受累（P〈0．01）。实验室指标中类风湿因子阳性率更高（P〈0．05）,而低补体C3血症较早发SLE组低（P〈0．01）。治疗方面,晚发SLE组较少使用大剂量皮质类固醇和免疫抑制剂。结论晚发SLE的临床表现多较轻,治疗上较少需要使用大剂量皮质类固醇和免疫抑制剂。     

PRIMARY NOCARDIAL MENINGITIS IN SYSTEMIC LUPUS ERYTHEMATOSUS

Nocardia is an important but often overlooked opportunisticinfection agent in immunocompromised hosts. Nocardiosis is primarilypulmonary; central nervous system involvement, usually in theform of brain abscess, is less common. Primary nocardial meningitiswithout associated brain abscess and pulmonary lesion is extremelyrare and poses a formidable diagnostic challenge. We reporton a patient with systemic lupus erythematosus who presentedwith a clinical picture of chronic meningitis which failed torespond to empirical antibiotic treatment. She deterioriatedand succumbed. Post-mortem examination showed nocardial meningitiswithout associated brain abscesses. Nocardial meningitis shouldbe considered a differential diagnosis of subacute meningitisin immunocompromised hosts. KEY WORDS: Nocardia, Nocardiosis, Immunocompromised hosts     

LUPUS ANTICOAGULANT AS A PROGNOSTIC MARKER IN SYSTEMIC LUPUS ERYTHEMATOSUS

To evaluate the prognostic significance of lupus anticoagulant(LA), 37 SLE patients with LA and 37 age- and sex-matched SLEpatients without LA were followed up for a median of 22 years,of which 16 years (median) after the initial LA-testing. Deepvenous thrombosis was observed in 20 (54%) patients with LA.Of these patients, 90% had the first episode within 8 yearsafter the onset of SLE symptoms, as compared to only one ofthe six LA-negative patients with deep venous thrombosis (P0.0001). Cerebral artery occlusions were more common in patientswith LA (P 0.016), but typically appeared as a late phenomenon.Nephritis or neuropsychiatric manifestations, previously associatedwith a poor outcome in SLE, did not correlate with the presenceof LA. However, higher mortality was associated with both LA(P 0.021) and a history of deep venous thrombosis (P 0.004),as well as with nephritis (P 0.038). The most common cause ofdeath in both LA positives and negatives was vascular occlusion. In conclusion, it appears that the first episode of deep venousthrombosis in patients with LA is typically seen early in thecourse of disease, and that LA and a history of deep venousthrombosis are both associated with increased mortality. KEY WORDS: Antiphospholipid antibodies, Lupus anticoagulant, Lupus erythematosus, Survival, Thrombocytopenia, Thrombosis