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AB0557 (2023)
PERIPARTAL-ONSET OF LUPUS VASCULITIS WITH COMPLICATIONS OF ANTI-PHOSPHOLIPID SYNDROME-A CASE-BASED REVIEW
S. Onwualu Onosenigbuan1, O. Ochiagha2,3, O. Adelowo4, H. Olaosebikan4
1University of Benin Teaching Hospital, Benin City Nigeria, Rheumatology-Internal Medicine, Benin, Nigeria
2Usman Danfodio University Teaching Hospital, Sokoto Nigeria, Rheumatology-Internal Medicine, Sokoto, Nigeria
3Usman Danfodio University Teaching Hospital, Sokoto Nigeria, Rheumatology-Internal Medicine, sokoto, Nigeria
4Lagos State University Teaching Hospital, Rheumatology-Internal Medicine, Lagos, Nigeria

 

Background Lupus vasculitis (LV) [1] is one of the secondary vasculitides occurring in the setting of systemic lupus erythematosus most commonly affecting small vessels or medium vessels, rarely large vessels. LV is commonly associated with anti-phospholipid syndrome [2]

Objectives Case Report 42year old female, 10weeks peripartal with twin-gestation presenting with progressive difficulty in walking with associated numbness and paraesthesia of the lower limbs [3], difficulty in breathing, Fever, non-erosive polyarthralgia and polyarthritis. Her symptoms started after delivery of her set of twins after 10years of secondary infertility.

On examination; generalised cutaneous vasculitic rashes, left foot drop, bilateral lower limb swelling, coarse crepitations at the lung bases, reduced power at both lower limbs2/5, reduced tone and reflexes. Reduced sensation to light and deep touch at the left foot.

Methods To access practicable treatment modalities in resource poor settings-Nigeria.

Results Electromyelography-Mononeuritis multiplex, HRCT-pulmonary embolism in bilateral pulmonary artery extending into the segmental artery, Doppler USS of both lower limbs-Acute deep vein thrombosis of the left internal jugular vein, ENA panel-positive dsDNA, anti-smith, anti-Ro/Anti-La, Antiphospholipid antibody panel [4]- Positive aCL, anti-B2GP1, LAC, thrombocytopenia, anaemia. Renal parameters marginally deranged. Elevated inflammatory markers.

Management High dose corticosteroids, Rescue therapy low dose Rituximab @days 0,15 patient had minimal response, maintained on low dose prednisolone, azathioprine and physiotherapy. Trial of EUROLUPUS regimen (6 courses) of low dose cyclophosphamide [5]. Rivaroxaban for PE & DVT. Inferior venae cavae filter for DVT.

Outcome Regained full power, tone and reflexes of her lower limbs and walked without support after 6 courses of cyclophosphamide. however, she has residual left foot drop undergoing physiotherapy.

Renal parameters marginally resolved. Pulmonary embolism and deep vein thrombosis resolved.

Conclusion Lupus Vasculitis can affect any organ systems. Modalities for treatment are according to the presentation and severity of the disease.

References

  1. Lupus Vasculitis: An Overview [Internet]. [cited 2023 Jan 15]. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8615745/
  2. Alarcón-Segovia D, Drenkard C. Vasculitis and the antiphospholipid syndrome [2]. Rheumatology. 2000;39(8):922–3.
  3. Gorson KC. Vasculitic neuropathies: An update. Neurologist. 2007 Jan;13(1):12–9.
  4. Gómez-Pacheco L, Villa AR, Drenkard C, Cabiedes J, Cabral AR, Alarcón-Segovia D. Serum anti-β2-glycoprotein-I and anticardiolipin antibodies during thrombosis in systemic lupus erythematosus patients. Am J Med. 1999 Apr;106(4):417–23.
  5. Barile-Fabris L, Ariza-Andraca R, Olguín-Ortega L, Jara LJ, Fraga-Mouret A, Miranda-Limón JM, et al. Controlled clinical trial of IV cyclophosphamide versus IV methylprednisolone in severe neurological manifestations in systemic lupus erythematosus. Ann Rheum Dis. 2005 Apr;64(4):620–5.

Acknowledgements to my prolific trainers- Prof.Adelowo an Dr Hakeem Olaosebikan.

Disclosure of Interests Shalom ONWUALU ONOSENIGBUAN: None declared, Olisa Ochiagha: None declared, Olufemi Adelowo Grant/research support from: pfizer-Knowledge of rheumatoid arthritis amongst general practitioners in Nigeria, Hakeem Olaosebikan: None declared.

Keywords: Disease-modifying drugs (DMARDs), Anti-phospholipid syndrome, Autoantibodies

DOI: 10.1136/annrheumdis-2023-eular.6169


Citation: , volume 82, supplement 1, year 2023, page 1475
Session: SLE, Sjön’s and APS - treatment (Publication only)