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Pediatric lupus nephritis presenting with terminal renal failure

Besouw MT, Vande Walle JG, Ilias MI, Raes AM, Prytula AA, Claeys L, et al.

Acta Clin Belg, 2016 Dec;71(6):455-457.
PMID: 27169353

A 12-year-old Congolese girl presented with acute renal failure, edema, hypertension, hemoptysis, hematuria, and proteinuria after a history of throat infection. Renal ultrasound showed kidneys of normal size, with increased echogenicity of the cortical parenchyma and decreased corticomedullary differentiation. Other additional investigations showed pancytopenia with decreased complement (low C3 and C4). Antinuclear antibodies were strongly positive, including anti-double stranded DNA. Renal biopsy confirmed severe grade IV lupus nephritis. She was treated with high-dose steroids, mycophenolate mofetil and hydroxychloroquine, in addition to hemodialysis. After one week of intensive treatment, diuresis recovered and dialysis could be stopped after six sessions. We describe an uncommon case of severe lupus nephritis, presenting with terminal renal failure. Since the rarity of this disease presentation, other more common diagnoses have to be considered. Once the diagnosis of lupus nephritis is established, a choice has to be made between the different induction treatment protocols. The patient's ethnic background and other supportive therapies, such as the need for dialysis, can help to make this choice.

Matched MeSH terms: Lupus Nephritis/complications*
Persistent hypertension in lupus nephritis and the associated risk factors

Shaharir SS, Mustafar R, Mohd R, Mohd Said MS, Gafor HA

Clin Rheumatol, 2015 Jan;34(1):93-7.
PMID: 25373448 DOI: 10.1007/s10067-014-2802-0

Arterial hypertension (HPT) burden up to two third of systemic lupus erythematosus (SLE) patients and contributes to accelerated atherosclerosis and cardiovascular (CV) risk. We aim to determine the prevalence of HPT among lupus nephritis (LN) patients who were in complete remission (CR) for a minimum of 6 months, with estimated glomerular filtration rate (eGFR) of >60 mL/min/1.73 m(2). This is a cross-sectional study of 64 LN patients who attended Nephrology/SLE Clinic at The National University of Malaysia Medical Centre (UKMMC). Persistent hypertension (blood pressure (BP) ≥140/90 mmHg for at least two occasions), CR for a minimum of 6 months and eGFR of >60 mL/min/1.73 m(2) were identified. Univariate and multivariate analyses were performed to determine the demographic and disease characteristics associated with HPT. Thirty-four of them (53.1 %) were hypertensive. Persistent HPT was associated with disease duration, acute kidney injury and high BP at the onset of LN, longer duration interval to achieve CR, number of relapses and cyclosporine A (CyA) use. There were no associations between histological classes, nephrotic range proteinuria, body mass index and waist circumference with HPT. Factors independently associated with HPT were disease duration OR 1.06 [95 %CI (0.91-1.24)], longer duration interval to achieve CR OR 1.104 [95 %CI (1.02-1.19)], number of relapses OR 2.53 [95 % CI (1.01-6.3)] and CyA use OR 5.3 [95 % CI (1.14-23.9)]. The prevalence of HPT among LN is high despite in remission. Aggressive treatment is important to achieve early CR and to prevent relapses.
Study site: Nephrology/SLE clinics, Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM), Kuala Lumpur, Malaysia

Matched MeSH terms: Lupus Nephritis/complications*
Multifocal osteoarticular tuberculosis in a systemic lupus erythematosus (SLE) patient: a rarity or an underdiagnosed condition?

Lim SL, Ong PS, Khor CG

Mod Rheumatol Case Rep, 2020 07;4(2):237-242.
PMID: 33086999 DOI: 10.1080/24725625.2020.1754567

Tuberculosis (TB) and its association with rheumatic diseases have been widely recognised. Occurrence of multifocal skeletal involvement constitutes <5% of all skeletal TB cases. We present a Malay patient with multifocal osteoarticular TB (OATB). A 35 year-old SLE woman with background usage of corticosteroid therapy and Azathioprine presented with lupus nephritis flare. Renal biopsy revealed diffuse proliferative lupus nephritis and intravenous (IV) Cyclophosphamide 0.5 g/m2 (850 mg) was initiated. One week later, patient complained dorsum of left hand and right knee swelling. On physical examination, patient was afebrile and the left hand swelling was cystic in consistency while right knee was warm and tender. Erythrocyte Sedimentation Rate (ESR) was 50 mm/hr and C-Reactive Protein (CRP) was 9.4 mg/L. Her Mantoux test was positive with 20 mm induration. Wrist radiograph and chest radiograph was normal. Musculoskeletal ultrasound showed 4th extensor compartment tenosynovitis with Doppler signal and right knee effusion with synovial proliferation. Extensor tenosynovectomy and right knee aspiration was performed. Left hand excised tissue and right knee synovial fluid for acid-fast bacilli (AFB) stain, TB PCR, bacterial and fungal cultures were negative. Urgent histopathological examination of the excised tissue showed necrotising granulomatous inflammation. Patient was empirically started on TB treatment and subsequent mycobacterial culture confirmed the diagnosis of TB. The joints swelling resolved after one month of TB treatment. Multifocal OATB is an infrequent form of extrapulmonary TB and diagnosing OATB requires high index of suspicion particularly in SLE patient on immunosuppression. Prompt investigations are essential to the diagnosis of this rare condition for early initiation of anti-tuberculous therapy.

Matched MeSH terms: Lupus Nephritis/complications
Fulltext Renal cell carcinoma with proliferative lupus nephritis

Wong KW

BMJ Case Rep, 2015 Jan 16;2015.
PMID: 25596289 DOI: 10.1136/bcr-2014-208060

We report a case of renal cell carcinoma diagnosed after a patient was treated successfully with intravenous cyclophosphamide for her active proliferative lupus nephritis (classes III and V). After the intravenous cyclophosphamide regimen, the patient was asymptomatic with persistent microscopic haematuria, and no proteinuria. The renal cell carcinoma was located on the left kidney; incidentally, this was where the initial renal biopsy was done to diagnose lupus nephritis.

Matched MeSH terms: Lupus Nephritis/complications*
Fulltext Psychiatric disorder in Malaysians with systemic lupus erythematosus

Chin CN, Cheong I, Kong N

Lupus, 1993 Oct;2(5):329-32.
PMID: 8305928 DOI: 10.1177/096120339300200510

All 79 patients who attended a University Systemic Lupus Erythematosus (SLE) Clinic over a 6 month period were assessed using the Clinical Interview Schedule for psychiatric disorder. Using the ICD-9 Classification, 40 were found to have psychiatric disorder, 26 having depressive neurosis, six anxiety neurosis, five endogenous depression and three dementia. The group with psychiatric disorder had significantly poor family support as well as lack of a confidant compared to the group without psychiatric disorder (P < 0.01). There was no difference between the group with psychiatric disorder and those without psychiatric disorder in terms of age, duration of illness, ethnicity and severity of SLE. Psychiatric disorder is common affecting more than half the subjects and depression was the most frequent diagnosis.
Study site: SLE clinics, Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM), Kuala Lumpur, Malaysia

Matched MeSH terms: Lupus Nephritis/complications
Systemic lupus erythematosus among male patients in Malaysia: how are we different from other geographical regions?

Shaharir SS, Kadir WDA, Nordin F, Bakar FA, Ting MWH, Jamil A, et al.

Lupus, 2019 Jan;28(1):137-144.
PMID: 30458692 DOI: 10.1177/0961203318812676

BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease which predominantly affects females. The disease characteristics in male SLE patients are reported to be distinct and may vary across ethnicities and geographical regions.
OBJECTIVE: To determine and compare the clinical phenotype and organ damage between male and female patients with SLE in Malaysia.
METHODOLOGY: This was a cross-sectional study involving SLE patients from Universiti Kebangsaan Malaysia Medical Centre from June 2016 until June 2017. Information on their socio-demographics and disease characteristics were obtained from the clinical records. Disease damage was assessed using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) damage index (SDI) scores. The disease characteristics, autoantibody profiles and organ damage were compared between male and female patients, and multivariable analysis using male sex as dependent variable was then performed.
RESULTS: A total of 418 patients were recruited and a total of 59 (14.1%) patients were male. Male patients presented with lower SLE ACR criteria at initial presentation but a significantly higher number of them had renal involvement (lupus nephritis) (78.0% versus 63.8%, p = 0.04). Male patients had less musculoskeletal involvement (45.8% versus 63.0%, p = 0.02) and tended to have lesser mucocutaneous involvement. Immunologic profile revealed that a lower number of male patients had positive anti-Ro antibody (22.7% versus 44.7%, p = 0.04) and they tended to have positive lupus anticoagulant antibody (27.6% versus 14.3%, p = 0.06). Presence of organ damage (SDI score ≥ 1) was significantly higher among males (55.9% versus 39.6%, p = 0.02) with higher renal damage (25.4% versus 9.2%, p = 0.004) and cardiovascular event of ischaemic heart disease or stroke (20.3% versus 7.0%, p = 0.004). They were also inclined to develop damage much earlier as compared to female patients, 3 (interquartile range (IQR) 7.5) versus 5 (IQR 7) years, p = 0.08. The occurrence of disease damage was independently associated with male gender with odds ratio of 1.9 (95% confidence interval 1.1-3.5), p = 0.02.
CONCLUSION: Male patients with SLE have more severe disease with renal damage and cardiovascular event.

Matched MeSH terms: Lupus Nephritis/complications
Predictors of thickened carotid intima media thickness among well controlled lupus nephritis patients in a Malaysian tertiary centre

Shaharir SS, Mohamed Said MS, Kong NC

Reumatismo, 2012;64(6):341-9.
PMID: 23285477 DOI: 10.4081/reumatismo.2012.341

OBJECTIVES: To investigate the prevalence of thickened carotid intima media thickness (CIMT) and its associated risk factors in patients with lupus nephritis (LN) who were in remission.
METHODS: This was a cross sectional study in which consecutive LN patients who were in remission and attending our Nephrology/SLE Clinic were included. Their demographic profile, traditional cardiovascular risk factors and treatment medications were evaluated by clinical interview and review of medical records. Carotid intima media thickness (CIMT) was measured using B Mode carotid ultrasonography. CIMT was considered to be abnormally thickened if it exceeded the 75th percentile matched for age-and sex-matched normal controls. The associated factors for thickened CIMT were examined.
RESULTS: A total of 39 patients with a mean remission duration of 29 ± 24.3 months and on a mean prednisolone dose of 9.10 ± 7.83 mg daily completed the study. Six patients (15.4%) had thickened CIMT. On univariate analysis, male gender, patient age, older age at diagnosis, higher serum CRP levels, greater proteinuria and higher mean cumulative azathioprine dose were associated with thickened CIMT (P<0.05). Lower mean cumulative doses of cyclosporine A (CyA) and mycophenolic acid (MPA) (P<0.05) each were associated with thickened CIMT. Using regression analysis, the associated factors of CIMT were older age at diagnosis and proteinuria.
CONCLUSIONS: Lupus factors particularly age at diagnosis and proteinuria were the associated factors of thickened CIMT. Larger prospective trials are indicated to confirm our findings.

Matched MeSH terms: Lupus Nephritis/complications