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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2016  |  Volume : 27  |  Issue : 3  |  Page : 610-613
An unusual presentation of juvenile lupus nephritis

1 Department of Nephrology, Gandhi Medical College, Hyderabad, Telangana, India
2 Department of Pathology, Nizams Institute of Medical Sciences, Hyderabad, Telangana, India

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Date of Web Publication13-May-2016


The incidence of juvenile lupus varies widely ranging between 4 and 250 per 100,000 population. Most common organ involvement in juvenile lupus is kidney. Neurological, cutaneous and hematological involvements are also involved. Skeletal muscle involvement in the form of myositis is rare. Myositis as presenting manifestation in juvenile lupus is also unusual. Herein, we report one such case wherein myositis preceded the onset of lupus nephritis

How to cite this article:
Bottu M, Yadla M, Burri S, Uppin M. An unusual presentation of juvenile lupus nephritis. Saudi J Kidney Dis Transpl 2016;27:610-3

How to cite this URL:
Bottu M, Yadla M, Burri S, Uppin M. An unusual presentation of juvenile lupus nephritis. Saudi J Kidney Dis Transpl [serial online] 2016 [cited 2022 Jun 25];27:610-3. Available from: https://www.sjkdt.org/text.asp?2016/27/3/610/182441

   Introduction Top

About 15-20% of the population develops lupus before 18 years of age. Although the age criteria for the definition of juvenile systemic lupus erythematosus (JLE) was reported to be between 14 and 20 years, many studies have considered 16 years of age at the time of diagnosis.[1]Prepubertal gender ratio was repor- ted to be 4:3, which increases to 4:1 after puberty and further increases up to 8-9:1 in adults. Even though the clinical features are more often nonspecific and constitutional in nature, it is severe than in adults with an aggressive involvement of renal and central nervous system. The frequency of organ involvement in JLE varies widely among different population. In Indian scenario, renal involvement is common and severe. Lupus nephritis affects 20-80% of juvenile-onset lupus and 10-50% of them would progress to end-stage renal disease. Myositis is rare in JLE and is unusual as the presenting manifestation of lupus. Herein, we report one such case wherein myositis preceded the onset of lupus nephritis.

   Case Report Top

A 16-year-old girl presented with progres- sive quadriparesis of 20 days duration. General examination was normal. Musculoskeletal exa- mination showed decreased power in all the limbs (3/5) with sluggish deep tendon reflexes. Evaluation showed hemoglobin of 10 g/dL, blood urea of 32 mg/dL, and serum creatinine of 0.8 mg/dL. The liver function tests were within normal limits. Complete urine exami- nation showed nil protein and the sediment was bland. Her creatine phosphokinase (CPK) was 5436 IU/mL. Muscle biopsy from quadri- ceps showed perifascicular atrophy, diffuse lymphocytic infiltration suggestive of inflam-matory myositis [Figure 1]. Her serology for antinuclear antibody, anti-Smith, and anti- U1RNP were strongly positive. Anti-dsDNA was consistently negative. In view of the pos- sibility of autoimmune myositis, she was initiated on parenteral steroids followed by oral steroids. A routine chest radiograph showed cardiomegaly and haziness in both the lower zones. Two-dimensional (2D)-echo showed moderate pericardial effusion without signs of tamponade along with moderate pul- monary arterial hypertension. High-resolution computed tomography chest showed bilateral ground-glass opacities in both the lung zones. Pulmonary function tests could not be done. Pericardial fluid analysis was transudative. In view of the systemic organ involvement inclu- ding cardiopulmonary involvement, she was advised cytotoxic therapy which was refused. She was placed on oral steroids and advised a regular follow-up. She took oral steroids at 1.5 mg/kg/day for about three months and then stopped on her own.
Figure 1. Muscle biopsy on adenosine triphosphatase stain showing perifascicular atrophy

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One year later, she presented with progres- sive anasarca and breathlessness. On examina- tion, there was tachypnea, tachycardia, bilateral pitting pedal edema, muffled heart sounds, and bilateral crepitations in basal areas. Her blood pressure was 90/70 mm Hg. On evaluation, she was found to have hemoglobin of 8 g/dL, blood urea of 86 mg/dL, and serum creatinine of 3.6 mg/dL. Complete urine examination showed 3+ albumin with bland urine sediment. Twenty-four hours h urine protein was 5.2 g, serum albumin was 1.7 g/dL. Chest X-ray showed haziness in basal zones, and 2D-echo showed massive pericardial effusion with tamponade. An emergency pericardiocentesis was done. She was initiated on renal replace- ment therapy in view of anuria. Over the next 48 h, her blood pressure improved to 120/70 mm Hg. There was an improvement in urine output and serum creatinine decreased to 1.8 mg/dL. After the stabilization of her condition, renal biopsy was done. This showed lupus membranous nephropathy [Figure 2]. She was initiated on intravenous cyclophosphamide the- rapy. At the end of six months follow-up, she is asymptomatic, urine showed 1+ albumin.
Figure 2. Histopathology of renal biopsy showing thickening of the basement membrane

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This is a case of JLE with myositis and asymptomatic cardiopulmonary involvement as the presenting feature of lupus which pre- ceded the onset of membranous lupus nephritis by one year.

   Discussion Top

Although the literature is sparse regarding JLE in India, the mean age of onset was reported to be 9.0 ± 2.6 years. Malaviya et al reported a point prevalence of JLE to be 3.2 per 100,000 in a population study from North India.[1]Mondal et al from Eastern India repor- ted the incidence of JLE to be 3.9% of all children presenting to a tertiary care children hospital.[2]

JLE is reported to have aggressive renal manifestations but the frequency of organ in- volvement can variable.[3]In a study by Hoffman et al, renal neurological and hematological involvement were found to be more common, and in a study by Carreño et al, dermato- logical, renal, and neurological involvement were more frequently observed. In India, renal involvement was reported to be common.[4],[5],[6]Myalgias are one of the common presenting symptoms in lupus, but myositis is rare. The incidence of lupus myositis was reported to be between 5% and 11% in the adult population. In JLE, the incidence of myositis is not known. In a study by Record et al, 17 of 55 children from Alabama (31%) had myositis and myositis was the presenting clinical pro- blem in 8/17 (47%). In this study, high levels of anti-U1RNP antibodies, anti-Smith anti- bodies, low levels of anti-dsDNA antibodies, and low complement levels were observed in the myositis group (17/55) compared to non myositis presenting (38/55) group.[7]However, Hoffman et al reported that antiribosomal P, anti-dsDNA, and antihistone antibodies are common in JLE.[6]

The clinical feature of myositis includes pro- ximal muscle weakness, myalgia, and muscle atrophy. The laboratory abnormalities are high levels of CPK, unlike mild to moderate eleva- tion seen in overlap syndromes, an abnormal magnetic resonance imaging of proximal muscles, lymphocytic vasculitis on muscle biopsy and high levels of anti-U1RNP anti- bodies.

Renal involvement was not reported in the children with lupus myositis.[6]Endo et al re- ported myositis and lupus nephritis in a 42- year-old woman, who presented with myalgia, proteinuria, and hematuria.[8]Jaybhaye et al reported myositis as presenting manifestation of lupus nephritis was reported in a 7-year-old girl.[9]

In the above two cases, there was simul- taneous renal and muscle involvement. How- ever, in our case, the muscle involvement pre- ceded lupus nephritis by more than a year. The possibility of development of lupus nephritis and the aggravation of cardiac features may be attributed to the inadequate immunosuppres- sion given during the first visit wherein patient refused to take cytotoxic therapy despite se- vere organ involvement in the form of peri- cardial effusion and interstitial lung disease. This emphasizes the need for timely diagnosis and the need of the hour to explain the benefits of immunosuppression outweighing the risks involved. It should be made understood that continuation on conservative measures alone would lead to unrelenting progression of the disease with cumulative organ involvement over a period of time.

Regarding the cardiopulmonary involvement, the incidence in JLE was reported to be 31%.[10]Combined cardiopulmonary involvement is more common than isolated cardiac or isolated pulmonary involvement. Cardiac manifesta- tions include pericardial effusion, cardiac tam- ponade, and conduction defects. Pulmonary involvement may be in the form of pleural effusion, interstitial lung disease, and pulmo- nary hypertension.[4]More often, the cardio- pulmonary involvement is found during the course of illness rather than at the time of diagnosis itself.

The points of interest are 2-fold: (1) An unusual presentation of lupus with myositis and asymp- tomatic cardiopulmonary involvement, (2) myositis preceding the occurrence of lupus nephritis, and (3) aggressive treatment with adequate immunosuppression as and when needed.

Conflict of interest: None.

   References Top

Malaviya AN, Singh RR, Singh YN, Kapoor SK, Kumar A. Prevalence of systemic lupus erythematosus in India. Lupus 1993;2:115-8.   Back to cited text no. 1
Mondal R, Nandi M, Ganguli S, Ghosh A, Hazra A. Childhood lupus: Experience from Eastern India. Indian J Pediatr 2010;77:889-91.   Back to cited text no. 2
Mina R, Brunner HI. Pediatric lupus - Are there differences in presentation, genetics, response to therapy, and damage accrual compared with adult lupus? Rheum Dis Clin North Am 2010;36:53-80, vii-viii.  Back to cited text no. 3
Habibi S, Saleem MA, Ramanan AV. Juvenile systemic lupus erythematosus: Review of clinical features and management. Indian Pediatr 2011;48:879-87.  Back to cited text no. 4
Carreño L, López-Longo FJ, Monteagudo I, et al. Immunological and clinical differences between juvenile and adult onset of systemic lupus erythematosus. Lupus 1999;8:287-92.  Back to cited text no. 5
Hoffman IE, Lauwerys BR, De Keyser F, et al. Juvenile-onset systemic lupus erythematosus: Different clinical and serological pattern than adult-onset systemic lupus erythematosus. Ann Rheum Dis 2009;68:412-5.  Back to cited text no. 6
Record JL, Beukelman T, Cron RQ. High prevalence of myositis in a Southeastern United States pediatric systemic lupus erythematosus cohort. Pediatr Rheumatol Online J 2011;9:20.  Back to cited text no. 7
Endo Y, Ikawa M, Takahashi N, Nishino I, Suzuki S, Yoneda M. A case of systemic lupus erythematosus predominantly presenting with myositis at onset. Rinsho Shinkeigaku 2013; 53:634-7.  Back to cited text no. 8
Jaybhaye AP, Sutay NR, Chate SV, Rathod TN. Juvenile systemic lupus erythematosus: A diagnostic dilemma. J Nat Sci Biol Med 2011; 2:229-31.  Back to cited text no. 9
Beresford MW, Cleary AG, Sills JA, Couriel J, Davidson JE. Cardio-pulmonary involvement in juvenile systemic lupus erythematosus. Lupus 2005;14:152-8.  Back to cited text no. 10

Correspondence Address:
Manjusha Yadla
Department of Nephrology, Gandhi Medical College, Hyderabad - 500 081, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1319-2442.182441

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