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Secondary antiphospholipid syndrome in an adolescent girl with sjögren syndrome

1 Department of Pediatrics, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
2 Department of Pediatrics, Division of Pediatric Hemato-Oncology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
3 Department of Pathology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
4 Department of Rheumatology, Division of Pediatric Rheumatology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India

Date of Submission09-Sep-2020
Date of Acceptance28-Oct-2020

Correspondence Address:
Mahesh Janarthanan,
Department of Rheumatology, Division of Pediatric Rheumatology, Sri Ramachandra Institute of Higher Education and Research, Porur, Chennai - 600 116, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_255_20

How to cite this URL:
John A, Jayaraman D, Gunabooshanam B, Janarthanan M. Secondary antiphospholipid syndrome in an adolescent girl with sjögren syndrome. Indian J Rheumatol [Epub ahead of print] [cited 2021 Apr 15]. Available from:

Dear Editor

A 15-year-old adolescent girl was brought with blackish discoloration of her toes associated with pain and swelling for 15 days. She was diagnosed with SS at 9 years of age when she presented with recurrent parotitis for 6 months with low-grade fever and arthralgia. She did not have dry eyes or dry mouth. Her investigations, including her salivary gland biopsy, revealed features of chronic inflammation and were suggestive of Sjogren's syndrome (SS) [Figure 1]a. She was commenced on oral steroids and hydroxychloroquine. On admission to the hospital on this occasion, she was apyrexial, blood pressure was normal. There were no oral ulcers, arthritis, or rashes. Local examination revealed gangrene of the 2nd 3rd, and 5th toe of the left leg and of the 2nd and 5th toes of the right leg [Figure 1]b. Investigations revealed anemia with normal complete blood count, erythrocyte sedimentation rate (ESR)-100 mm at 1 h (10–20 mm), direct coombs test–positive, C3 C4-normal, hypergammaglobulinemia, antinuclear antibody (ANA)-nucleus speckled pattern, 3+ fluorescence, 1:320 titer, lupus anticoagulant positive, anticardiolipin antibody IgM, IgM and ß2 Glycoprotein 1 IgM positive, rheumatoid factor (RF)– 1250 (0–20 IU/ml), ENA-SS-A, SS-B, Ro-52 all 3+ positive and RNP/Sm 1+ positive, dsDNA and antineutrophil cytoplasmic antibodies–negative. An echocardiogram and Doppler of lower limbs were normal.
Figure 1: (a) Salivary gland tissue massively infiltrated by sheets of lymphoid cells with prominent germinal centres, enclosing salivary gland nests. (b) Gangrene of toes

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The patient was commenced on anticoagulation therapy with subcutaneous low-molecular-weight heparin injections (LMWH), methylprednisolone pulse therapy 30 mg/kg for 3 days followed by 2 m/kg of oral prednisolone and cyclophosphamide infusion. LMWH was switched over to warfarin at the time of discharge, and an international normalized ratio of 2–3 was targeted with a plan of anticoagulation at least for 6 months followed by reassessment. At the time of discharge, the pain and swelling had reduced. At 3 months follow-up, the gangrene of the toes was well demarcated with no further spread of gangrene. Anticardiolipin antibody and lupus anticoagulant remained positive.

SS is an autoimmune disease of lacrimal and salivary glands with the potential to involve other organs.[1] In contrast to adult disease, recurrent parotitis and systemic features are common.[2] Diagnosis is by demonstrating lymphocytic infiltration in the salivary gland and the presence of autoantibodies such as anti-Ro/SSA, anti-La/SSB, ANA, RF, elevated ESR, and hypergammaglobulinemia.[3]

On the other hand, antiphospholipid syndrome (APS) is characterized by episodes of thromboembolism or recurrent miscarriage and by the presence of elevated levels of anticardiolipin antibodies, β2-glycoprotein 1 antibodies and/or a positive lupus coagulant test.[4] In children, the clinical presentations may include stroke, chorea, pulmonary embolism, deep-vein thrombosis, or peripheral gangrene. SS/APS may occur in isolation as primary SS/APS and if they occur in the context of an underlying disorder such as systemic lupus erythematosus they are called secondary SS/APS, respectively.

In an international APS registry of 121 pediatric patients from 14 countries, there were equal numbers of primary and secondary APS patients. Of the children with secondary APS, 46 had lupus, 4 each with lupus-like illness and autoimmune thyroiditis, the remaining had other illnesses. There were no patients with SS in this pediatric cohort. Digital ischemia was the presenting manifestation in four patients.[5]

The presenting clinical features of primary SS and APS in children may vary when compared to adults, and these should be borne in mind when dealing with children with rheumatic diseases. To our knowledge, this is the first case report of secondary APS in a child with SS.

Declaration of patient consent

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

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Conflicts of interest

There are no conflicts of interest.

  References Top

Deprettere AJ, Van Acker KJ, De Clerck LS, Docx MK, Stevens WJ, Van Bever HP. Diagnosis of Sjögren's syndrome in children. Am J Dis Child 1988;142:1185-7.  Back to cited text no. 1
Virdee S, Greenan-Barrett J, Ciurtin C. A systematic review of primary Sjögren's syndrome in male and paediatric populations. Clin Rheumatol 2017;36:2225-36.  Back to cited text no. 2
Longhi BS, Appenzeller S, Centeville M, Gusmão RJ, Marini R. Primary Sjögren's syndrome in children: Is a family approach indicated? Clinics 2011;66:1991-3.  Back to cited text no. 3
Berkun Y, Kenet G. Pediatric antiphospholipid syndrome. Isr Med Assoc J 2008;10:45-7.  Back to cited text no. 4
Avcin T, Cimaz R, Silverman ED, Cervera R, Gattorno M, Garay S, et al. Pediatric antiphospholipid syndrome: Clinical and immunologic features of 121 patients in an international registry. Pediatrics 2008;122:e1100-7.  Back to cited text no. 5


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