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Fulltext A comparison of thrombotic thrombocytopenic purpura in an inception cohort of patients with and without systemic lupus erythematosus

Letchumanan P, Ng HJ, Lee LH, Thumboo J

Rheumatology (Oxford), 2009 Apr;48(4):399-403.
PMID: 19202160 DOI: 10.1093/rheumatology/ken510

To compare the clinical presentation, response to therapy and outcome of thrombotic thrombocytopenic purpura (TTP) in an inception cohort of patients with and without SLE.

Matched MeSH terms: Lupus Erythematosus, Systemic/complications*
Fulltext Systemic lupus erythematosus and melioidosis

Che Rahim MJ, Mohammad N, Kamaruddin MI, Wan Ghazali WS

BMJ Case Rep, 2019 Jul 01;12(7).
PMID: 31266760 DOI: 10.1136/bcr-2019-229974

We reported a case of a young female patient presented with sepsis and diagnosed with melioidosis and systemic lupus erythematosus (SLE) within the same admission. She presented with 1-week history of productive cough, progressive dyspnoea together with prolonged fever, arthralgia, rashes and oral ulcers. She had septicemic shock, respiratory failure requiring intubation and ventilation in intensive care unit and subsequently developed acute renal failure requiring haemodialysis. Antibiotics and immunosuppressive treatment including low-dose intravenous cyclophosphamide were commenced. She had a remarkable recovery and was discharged after 6 weeks. There was no evidence of active SLE or relapse of melioidosis during clinic follow-ups.

Matched MeSH terms: Lupus Erythematosus, Systemic/complications*
Severe infections in systemic lupus erythematosus: disease pattern and predictors of infection-related mortality

Teh CL, Wan SA, Ling GR

Clin Rheumatol, 2018 Aug;37(8):2081-2086.
PMID: 29667100 DOI: 10.1007/s10067-018-4102-6

Infection is a major cause of morbidity and mortality among patients with systemic lupus erythematosus (SLE). To describe the pattern of serious infections in patients with SLE and to identify the predictors of infection-related mortality among SLE patients with serious infections, we prospectively studied all SLE patients who were hospitalized with infections in Sarawak General Hospital during 2011-2015. Demographic data, clinical features, and outcomes were collected. Cox regression analysis was carried out to determine the independent predictors of infection-related mortality. There were a total of 125 patients with 187 episodes of serious infections. Our patients were of multiethnic origins with female predominance (89.6%). Their mean age was 33.4 ± 14.2 years. The patients had a mean disease duration of 66.8 ± 74.0 months. The most common site of infection was pulmonary (37.9%), followed by septicemia (22.5%). Gram-negative organisms (38.2%) were the predominant isolates within the cohort. There were 21 deaths (11.2%) during the study period. Independent predictors of infection-related mortality among our cohort of SLE patients were flare of SLE (HR 3.98, CI 1.30-12.21) and the presence of bacteremia (HR 2.54, CI 0.98-6.59). Hydroxychloroquine was protective of mortality from serious infections (HR 9.26, CI 3.40-25.64). Pneumonia and Gram-negative organisms were the predominant pattern of infection in our SLE cohort. The presence of flare of SLE and bacteremia were independent prognostic predictors of infection-related mortality, whereas hydroxychloroquine was protective of infection-related mortality among SLE patients with serious infections.

Matched MeSH terms: Lupus Erythematosus, Systemic/complications*
Degraded microarchitecture by low trabecular bone score is associated with prevalent vertebral fractures in patients with systemic lupus erythematosus

Lai EL, Huang WN, Chen HH, Chen JP, Chen DY, Hsieh TY, et al.

Arch Osteoporos, 2020 03 27;15(1):54.
PMID: 32221755 DOI: 10.1007/s11657-020-00726-3

PURPOSE: Recently, trabecular bone score (TBS) has emerged as an important supplementary assessment tool in osteoporosis diagnosis and management. The high incidence of fragility fracture within the non-osteoporotic range of bone mineral density (BMD), among systemic lupus erythematosus (SLE) patients, highlights the crucial role of bone microarchitecture in osteoporosis. This study aimed to evaluate whether TBS identified existing vertebral fractures (VF) more accurately than BMD in SLE patients.
METHODS: This study enrolled 147 SLE patients from the Asia Pacific Lupus Collaboration (APLC) cohort, who had BMD and TBS assessed from January 2018 until December 2018. Twenty-eight patients sustaining VF and risk factors associated with increased fracture occurrence were evaluated. Independent risk factors and diagnostic accuracy of VF were analyzed by logistic regression and ROC curve, respectively.
RESULT: The prevalence of vertebral fracture among SLE patients was 19%. BMD, T-score, TBS, and TBS T-score were significantly lower in the vertebral fracture group. TBS exhibited higher positive predictive value and negative predictive value than L spine and left femur BMD for vertebral fractures. Moreover, TBS had a higher diagnostic accuracy than densitometric measurements (area under curve, 0.811 vs. 0.737 and 0.605).
CONCLUSION: Degraded microarchitecture by TBS was associated with prevalent vertebral fractures in SLE patients. Our result suggests that TBS can be a complementary tool for assessing vertebral fracture prevalence in this population.

Matched MeSH terms: Lupus Erythematosus, Systemic/complications
Fulltext Puerperal acquired factor VIII inhibitor causing a von Willebrand-like syndrome in a patient with anti-DNA antibodies

Jackson N, Hashim ZA, Zainal NA, Jamaluddin N

Singapore Med J, 1995 Apr;36(2):230-1.
PMID: 7676276

A 30-year-old Malay lady, with no previous or family history of bleeding, presented with severe gum bleeding 25 days post-partum. The factor VIII:c was 0.03 iu/ml with evidence of a slow-acting factor VIII inhibitor. Von Willebrand factor antigen (VWF:age) varied from less that 0.05 to 0.17 iu/ml, and there was absent ristocetin-induced platelet aggregation. Anti-nuclear and anti-DNA antibodies were present, but there were no other features of systemic lupus erythematosus. There was some clinical response to cryoprecipitate and tranexamic acid, and slight improvement with corticosteroid. Fifteen months later, the patient has no active bleeding problem, and her VWF-ag is increasing spontaneously. However, factor VIII:c is less than 0.01 iu/ml and her factor VIII inhibitor titre is still > 20 Bethesda units/ml.

Matched MeSH terms: Lupus Erythematosus, Systemic/complications
Fulltext Pediatric systemic lupus erythematosus. Retrospective analysis of clinico-laboratory parameters and their association with Systemic Lupus Erythematosus Disease Activity Index score

Nazri SKSM, Wong KK, Hamid WZWA

Saudi Med J, 2018 Jun;39(6):627-631.
PMID: 29915860 DOI: 10.15537/smj.2018.6.22112

OBJECTIVES: To elucidate the clinico-laboratory characteristics associated with pediatric systemic lupus erythematosus (pSLE) patients with higher Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score in a retrospective cohort of pSLE patients.

METHODS: A retrospective study involving 32 pSLE patients was conducted at Hospital Universiti Sains Malaysia, Kelantan, Malaysia between 2006 and 2017.

RESULTS: Within the group of 32 pSLE patients, 23 were girls and 9 were boys (3:1 ratio). The most common symptom was renal disorder (n=21; 65.6%) followed by malar rash (n=9; 28.1%), oral ulcers (n=7; 21.9%), prolonged fever (n=5; 15.6%) and arthritis (n=4; 12.5%). Antinuclear antibodies (ANA) were detected in all patients and 25 patients (78.1%) were positive for anti-double stranded DNA (anti-dsDNA) antibodies. Eighteen (56.3%) patients had active SLE (SLEDAI ≥6), and these patients were significantly associated with heavy pyuria (p=0.004), a high ANA concentration (1:160; p=0.040, 1:320; p=0.006), elevated ESR (p=0.006), low C3 levels (p=0.008), oral ulcers (p=0.010), heavy hematuria (p=0.017) and heavy proteinuria (p=0.017), lupus erythematosus (LE)-nonspecific lesion manifestations (p=0.019) and malar rash (p=0.044).

CONCLUSION: Pediatric systemic lupus erythematosus patients with higher SLEDAI score were most significantly associated with pyuria, high ANA titers, and elevated ESR.

Matched MeSH terms: Lupus Erythematosus, Systemic/complications
Fulltext Dietary calcium and bone mineral density in premenopausal women with systemic lupus erythematosus

Chong HC, Chee SS, Goh EM, Chow SK, Yeap SS

Clin Rheumatol, 2007 Feb;26(2):182-5.
PMID: 16565892 DOI: 10.1007/s10067-006-0258-6

The primary objective of this study was to determine the relationship between dietary calcium intake and bone mineral density (BMD) in premenopausal women with systemic lupus erythematosus (SLE) on corticosteroids (CS). The secondary aim was to identify other risk factors for osteoporosis in these patients. A cross-sectional sample of patients attending the SLE Clinic at a teaching hospital was recruited. BMD was measured using dual-energy X-ray absorptiometry. Daily dietary calcium intake was assessed using a structured validated food frequency questionnaire, in which patients were asked to estimate their food intake based on their recent 2-month dietary habits. Sixty subjects were recruited with a mean age of 33.70+/-8.46 years. The median duration of CS use was 5.5 years (range 0.08-24). The median cumulative dose of steroids was 17.21 g (range 0.16-91.37). The median daily dietary calcium intake was 483 mg (range 78-2101). There was no significant correlation between calcium intake and BMD, even after correcting for CS use. There were also no correlations between BMD and the duration of SLE, cumulative CS use, duration of CS use, smoking, alcohol intake, and SLE disease activity index score. Twenty-eight (46.7%) patients had normal BMD, 28 (46.7%) had osteopenia, and four (6.6%) had osteoporosis. Duration of SLE significantly correlated with cumulative CS dosage. In conclusion, 6.7% of these Asian premenopausal SLE women had osteoporosis and only 46.7% had normal BMD. Daily dietary calcium intake did not correlate with BMD.
Study site: SLE clinic, University Malaya Medical Centre (UMMC), Kuala Lumpur, Malaysia

Matched MeSH terms: Lupus Erythematosus, Systemic/complications
Characteristics and outcome of autoimmune liver disease in Asian children

Lee WS, Lum SH, Lim CB, Chong SY, Khoh KM, Ng RT, et al.

Hepatol Int, 2015 Apr;9(2):292-302.
PMID: 25788179 DOI: 10.1007/s12072-014-9558-0

BACKGROUND: Little is known about autoimmune liver disease (AILD) in Asian children. We studied the clinical features and predictors of outcome in childhood AILD in an Asian population.

METHODS: Retrospective review of AILD [autoimmune hepatitis type 1 and 2 (AIH1, AIH2), primary sclerosing cholangitis (PSC) and autoimmune sclerosing cholangitis (ASC)] seen at two pediatric liver units in Malaysia.

RESULTS: At presentation, 17 (56%) of the 32 children [19 females, 59%; median (range) age 7.7 (1.8-15.5) years] with AILD (AIH1 = 18, AIH2 = 5, PSC = 0, ASC = 9) had liver cirrhosis. At final review [median (range) duration of follow-up 4.8 (0.4-12) years], 24 patients (75%) survived with a native liver. Twenty-one (66%) were in remission; 19 (AIH1 = 11; AIH2 = 4, ASC = 4) were on prednisolone and/or azathioprine, one on cyclosporine and another on mycophenolate mofetil. Three (AIH1 = 3) were in partial remission. Of the two who underwent liver transplantation (LT; 6.5%; both ASC), one died of primary graft failure after LT. Six patients (19%) died without LT (acute liver failure, n = 1; end-stage liver disease, n = 5). The overall survival rate (native liver and survival post-LT) was 78%. A delay in seeking treatment adversely affected the final outcome [survival with native liver vs. LT or death (duration between onset of disease and treatment; median ± standard error) = 2.5 ± 2.9 months vs. 24.0 ± 13.3 months; p = 0.012].

CONCLUSIONS: Although remission was achieved in the majority of patients with prednisolone and/or azathioprine therapy, delay in seeking diagnosis and treatment adversely affects the outcome of childhood AILD in Malaysia.

Matched MeSH terms: Lupus Erythematosus, Systemic/complications
The spectrum of ocular involvement in patients with systemic lupus erythematosus without ocular symptoms

Soo MPK, Chow SK, Tan CT, Nadior N, Yeap SS, Hoh HB

Lupus, 2000;9(7):511-4.
PMID: 11035416 DOI: 10.1177/096120330000900706

The aim of the study was to determine the spectrum of clinical ocular involvement in patients with inactive systemic lupus erythematosus (SLE) who have no ocular symptoms. Patients with a diagnosis of SLE based on the 1982 revised American College of Rheumatology criteria and with no ocular complaints were recruited from the SLE clinic. Clinical data regarding their systemic disease and disease activity were recorded and a full ophthalmic examination carried out. 52 patients of mixed ethnicity comprising of 75% Chinese, 19% Malays and 6% Indian patients were recruited. Of these, 51 (98%) were female with a mean age of 34+/-11 (range 16-74 y). 16 (31%) patients had dry eyes while corticosteroid induced glaucoma and cataract was detected in 1 (2%) and 7 (14%) patients, respectively. No patients were found to have sight-threatening ocular conditions such as cotton wool spots, vasculitis, optic neuropathy or uveitis. Patients with clinically inactive disease were found not to have sight-threatening ocular diseases that are known to be associated with SLE. Although they have no ocular complaints, nearly one-third of these patients have dry eyes. Ocular examination may be unnecessary when the disease is clinically inactive and in the absence of ocular symptoms.
Study site: SLE clinic, University Malaya Medical Centre (UMMC), Kuala Lumpur, Malaysia

Matched MeSH terms: Lupus Erythematosus, Systemic/complications
Macrophage activation syndrome as an initial presentation of systemic lupus erythematosus with sensorineural hearing loss in a young male patient

Tan CL, Yahaya MH, Ahmad NS, Lim CH

BMJ Case Rep, 2020 Mar 12;13(3).
PMID: 32169986 DOI: 10.1136/bcr-2019-233330

An 18-year-old male adolescent presented with prolonged high spiking temperature, photosensitive rash, oral ulcers and reduced hearing bilaterally of recent onset. Examination revealed malar rash, vasculitis rash over bilateral palms and soles, oral and buccal ulcers, palpable posterior auricular and inguinal lymph nodes, and reduced hearing bilaterally. His further investigations noted pancytopaenia, elevated transaminases, hyperferritinaemia, low C3 and C4 levels, positive antinuclear antibody, double-stranded DNA and direct Coombs test, while bone marrow aspiration revealed active phagocytic activity suggestive of hemophagocytic syndrome. We made a diagnosis of systemic lupus erythematosus with macrophage activation syndrome. We treated him with pulse intravenous methylprednisolone and his condition improved drastically. Temperature resolved on the next day after intravenous methylprednisolone; bilateral sensorineural hearing loss improved to near-normal hearing after treatment. He remained well during follow-up with a tapering dose of prednisolone.

Matched MeSH terms: Lupus Erythematosus, Systemic/complications*