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Table of Contents
CASE REPORT
Year : 2021  |  Volume : 4  |  Issue : 2  |  Page : 82-84

Unilateral orbital cellulitis as the presenting manifestation in a child with lupus nephritis


Division of Pediatric Nephrology, Sardar Vallav Bhai Patel Post Graduate Institute of Pediatrics, SCB Medical College, Cuttack, Orissa, India

Date of Submission26-Jan-2021
Date of Decision30-May-2021
Date of Acceptance06-Sep-2021
Date of Web Publication28-Dec-2021

Correspondence Address:
Subal Kumar Pradhan
Sardar Vallav Bhai Patel Post Graduate Institute of Pediatrics, SCB Medical College, Cuttack - 753 002, Orissa
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ajpn.ajpn_3_21

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  Abstract 


Pediatric systemic lupus erythematous (SLE) is a multisystem disease chiefly affecting skin, kidneys, joints, and hematologic system. However, no organ of the body is immune to this condition. Ocular manifestations are seen in one third of adult patients. The most common ocular manifestation is keratoconjunctivitis sicca, whereas the most severe forms are optic neuritis and retinal vasculitis. An isolated unilateral ocular manifestation is a rare initial presentation of lupus. Here, we described a 9-year-old girl who presented to us with orbital preseptal cellulitis and was later diagnosed as diffuse proliferative lupus nephritis, which responded well to immunosuppression.

Keywords: Orbital cellulitis, pediatric, systemic lupus erythematous


How to cite this article:
Nayak S, Pradhan SK. Unilateral orbital cellulitis as the presenting manifestation in a child with lupus nephritis. Asian J Pediatr Nephrol 2021;4:82-4

How to cite this URL:
Nayak S, Pradhan SK. Unilateral orbital cellulitis as the presenting manifestation in a child with lupus nephritis. Asian J Pediatr Nephrol [serial online] 2021 [cited 2022 Jan 17];4:82-4. Available from: https://www.ajpn-online.org/text.asp?2021/4/2/82/334034




  Introduction Top


Pediatric systemic lupus erythematous (SLE) is a multisystem autoimmune disease chieftly involving skin, kidneys, joints, and hematologic system. However, no organ of the body is immune to this condition. Around one third of adult systemic lupus erythematous (SLE) patients have ocular manifestations.[1] Approximately 50%–70% adults and 37%–82% children with SLE develop lupus nephritis.[2] An isolated unilateral ocular manifestation is a rare initial presentation of SLE with lupus nephritis. Here, we described a 9-year-old girl who first presented to us as orbital preseptal cellulitis and was later diagnosed as diffuse proliferative lupus nephritis, which responded well to immunosuppressant therapy.


  Case Report Top


A 9-year-old girl was admitted to our hospital with chief complaints of fever for 7 days with pain, redness of left eye along with periorbital puffiness. She had no other complaints. She was doing well before this episode. There was no history of trauma to the eye. She had stable vitals with normal anthropometric parameters. General examination was within normal limits except mild pallor and redness of left bulbar and palpebral conjunctiva with associated chemosis. Systemic examination revealed no abnormalities. Hence, orbital cellulitis was kept as provisional diagnosis and treatment was initiated with injection ceftriaxone and cloxacillin. Routine laboratory investigations revealed normocytic normochromic anemia (hemoglobin 9.1 g/dL), leukopenia (total leukocyte count 3670/mm3), and thrombocytopenia (platelet count 1,40,000/mm3). erythrocyte sedimentation rate was 30 mm in first hour; liver and kidney function tests were within normal limits. Orbital ultrasonography revealed preseptal cellulitis. Computed tomography scan of brain was normal. Intravenous antibiotics were continued while awaiting blood culture report. However, redness of the eye and tenderness persisted. After 7 days, she developed bilateral pitting pedal edema with oliguria and stage I hypertension (blood pressure >95th percentile for her sex, height and age). Repeat investigations suggested nonnephrotic proteinuria (urine spot protein and creatinine ratio of 1.6 mg/mg) and microscopic hematuria with elevated blood urea: urea (52.8 mg/dL) and serum creatinine (1.68 mg/dL). Serum albumin was 2.31 g/dL with total protein 5 g/dL. Serum cholesterol was 173 mg/dL. The provisional diagnosis was kept as acute glomerulonephritis with preseptal cellulitis. The antistreptolysin O (ASO) titer was not elevated. Serum complement C3 (14 mg/dl; normal range 80–160 mg/dL) and C4 (<1 mg/dL normal range 20–40 mg/dL) were low. Antinuclear antibody (ANA) by enzyme immunoassay (142.9 units/mL; normal value <20 units/mL) and antidouble stranded DNA antibody (82 IU/ mL; normal value <30 IU/mL) were positive, while direct Coombs test (DCT) was negative. Blood cultures report revealed no growth. Contrast-enhanced computed tomography of orbit revealed preseptal soft tissue swelling with adjacent fat stranding; extraocular muscles were normal.

With these laboratory investigations and rapid worsening of kidney function during hospital stay, SLE with lupus nephritis was considered as the most probable diagnosis associated with preseptal cellulitis. Hence, kidney biopsy was planned. Renal histopathology in light microscopy revealed diffuse mild to moderate increase in mesangial cellularity and matrix in all 21 glomeruli and segmental or global endocapillary cell proliferation with variable neutrophil infiltration. Two glomeruli had segmental fibrinoid tuft necrosis. [Figure 1]. These findings suggested Class IV (ISN/RPS 2018) lupus nephritis with disease activity index of 14/24 and chronicity index of 0/12. Immunofluorescence was consistent with 'full-house' pattern (IgA 2+, IgG 3+, IgM 2+, C3 2+, C1q 2+, kappa light chain 3+, and lambda light chain 3+) [Figure 2] With a diagnosis of active lupus nephritis stage IV, pulse methylprednisolone, at a dose of 30 mg/kg/day was administered for 3 consecutive days followed by oral prednisolone. Injection cyclophosphamide (750 mg/m2) pulse therapy was started for induction of remission and planned to continue monthly for 6 doses. Oral hydroxychloroquine was also added. Gradually, orbital swelling improved, along with nephritis. Repeat slit lamp and fundus examination revealed no other abnormalities. She was discharged from hospital with advice regarding sun protection, oral hydroxychloroquine, prednisolone and amlodipine, and planned monthly pulse dose of cyclophosphamide injections.
Figure 1: Histopathology of renal biopsy-Light microscopy showing segmental endocapillary proliferation and mesangial hypercellularity (Hematoxylin and Eosin x200)

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Figure 2: Immunofluoresence staining for IgG showing global, peripheral and mesangial deposits (magnification x 200)

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  Discussion Top


pSLE is a multisystemic disease with predominant involvement of skin, kidneys, joints, and blood-forming cells.[3] Other major organ systems involved are central nervous system in form of seizures, psychosis, headache, and cognitive impairment. Cardiovascular and pulmonary manifestations are not uncommon. Common presentations are prolonged fever with generalized fatigue, myalgia, acute nephritis, photosensitivity, and arthritis. Clinical features may appear sequentially and not simultaneously, so cases with high index of suspicion need to be followed up meticulously. Our patient was confirmed to have pSLE as per SLICC criteria, and she progressed to biopsy-confirmed nephritis with high ANA titer positivity.

Scleritis, episcleritis, and keratitis are the usual ocular manifestations of SLE in adults. Keratoconjunctivitis sicca is the most common ocular manifestation.[1] Orbital involvements described in adults include periorbital edema, ischemia or infarction, anterior uveitis, retinopathy, and optic neuropathy.[4] There are case reports in adults with SLE presenting for the first time with ocular myositis, isolated scleritis, and episcleritis.[5],[6] As such, ocular manifestations as the first presentation of SLE are very rare. In pSLE, the most common ocular finding is cotton-wool spots indicating retinal vasculitis.[7],[8] Other findings in children are episcleritis, scleritis, keratoconjunctivitis sicca, and occlusion of central retinal vein. However, orbital cellulitis as the first presentation of pSLE is very rare. In our patient, there were no features of uveitis in slit-lamp examination, and fundoscopy done after resolution of orbital cellulitis was within normal limits. Orbital cellulitis was her initial presentation which was poorly responding to antibiotics alone and the disease progressed with nephropathy. Her orbital manifestation and nephritis improved significantly with aggressive immunosuppressive therapy. Hence, we hypothesize that the orbital preseptal cellulitis was the first autoimmune manifestation of pSLE, and in this case, it was not associated with anterior or posterior uveitis.

Patient consent for publication

Signed informed consent document was obtained from the patient for the publication of this case report.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Palejwala NV, Walia HS, Yeh S. Ocular manifestations of systemic lupus erythematosus: A review of the literature. Autoimmune Dis 2012;2012:1-9.  Back to cited text no. 1
    
2.
Szymanik-Grzelak H, Kuźma-Mroczkowska E, Małdyk J, Pańczyk-Tomaszewska M. Lupus nephritis in children - 10 years' experience. Cent Eur J Immunol 2016;41:248-54.  Back to cited text no. 2
    
3.
Cassidy. Systemic lupus erythematosus. In: Textbook of Pediatric Rheumatology. Ch. 21., 6th ed. Philadelphia: Elsevier; 2011. p. 317.  Back to cited text no. 3
    
4.
Dammacco R. Systemic lupus erythematosus and ocular involvement: An overview. Clin Exp Med 2018;18:135-49.  Back to cited text no. 4
    
5.
Szymanik-Grzelak H, Kuźma-Mroczkowska E, Małdyk J, Pańczyk-Tomaszewska M. Lupus nephritis in children - 10 years' experience. Cent Eur J Immunol 2016;41:248-54.  Back to cited text no. 5
    
6.
Lin WV, Saumur M, Al-Mohtaseb Z. Scleritis, keratitis, and orbital cellulitis: Isolated ocular manifestation of systemic lupus erythematosus. Lupus 2018;27:1985-8.  Back to cited text no. 6
    
7.
Chan AJ, Rai AS, Lake S. Orbital myositis in systemic lupus erythematosus: A case report and literature review. Eur J Rheumatol 2020;7:135-7.  Back to cited text no. 7
    
8.
Sivaraj RR, Durrani OM, Denniston AK, Murray PI, Gordon C. Ocular manifestations of systemic lupus erythematosus. Rheumatology (Oxford) 2007;46:1757-62.  Back to cited text no. 8
    


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