Spondyloarthritis in SLE-Therapeutic Challenge!

Abstract

Systemic lupus erythematosus is a multisystem immune mediated disease and sometimes called as “disease of mimics”. It is the most common of the connective tissue diseases and most common presenting features are fever, rash, weight loss, lymphadenopathy and cytopenia. Inflammatory back pain and sacroileitis are uncommon in SLE and most commonly seen in HLA B27 positive males. We present this man with sacroileitis in SLE and the complexities in diagnosis and management.

Keywords: SLE; Spondyloarthritis; Adalimumab; Lupus nephritis

Introduction

Complex autoimmune diseases can manifest in different ways and SLE associated sacroileitis is rare. Systemic Lupus Erythematosus (SLE, lupus) is a highly complex and heterogeneous autoimmune disease that most often afflicts women in their child-bearing years. It is characterized by circulating self-reactive antibodies that deposit in tissues, including skin, kidneys, and brain, and the ensuing inflammatory response can lead to irreparable tissue damage. Inflammatory arthritis in ankylosing spondylitis causes pain and stiffness and progressively leads to new bone formation and ankylosis (fusion) of affected joints. SLE and Spondyloarthritis are two autoimmune rheumatologic diseases with different aetiopathogenesis as well as diverse clinical and genetic characteristics and are rarely seen together. To the best of our knowledge, there are only 9 reported cases of the coexistence of SLE and AS in the English literature [1]. I present a unique patient journey with challenging management decisions.

Case Presentation

A 24 yrs old male, PhD student, was referred with arthalgia, fever and cough. He had history of hair loss, mouth ulcers for a month. He had sacrolieitis diagnosed on CT Pelvis 2 yrs ago and had been continuing indomethacin for a year. His ANA and HLA B27 were negative.

At the time of hospitalization, he was febrile, tachypneic and in septic shock. He had features of pneumonia and ankle synovitis. His chest X ray showed pleural effusion with consolidation. He had hematoproteinuria and acute kidney injury.

Investigations

His blood test results showed neutrophilia, lymphopenia, ESR 110, platelets 410, CRP 48, Urea 74, creatinine 2.2, Urine PCR 5.2, ANCA, HLA B27, Rheumatoid Factor, ASO Titre, ACE levels all were negative, ANA 1 in 2560 positive and dsDNA high, Low c3, c4, Cardiolipin and antiphospolipid antibody normal, raised SGOT and SGPT with globulins too. ECHO was normal. CT chest showed consolidation with pleural effusion.

Management

He was diagnosed with sepsis due to Pneumonia and features of SLE. Following treatment for sepsis, he was given Methylpred and his renal functions improved. He was given Mycophenolate and steroids weaned. During his follow up, blood parameters, ANA, dsDNA normalized.

After 4 months he developed inflammatory back pain, His MRI showed asymmetric bilateral sacroileitis (Figure 1) and steroids was given with methotrexate instead of Mycophenolate. Although he improved with steroids he developed significant acne. His bloods showed raised CRP and no features of SLE.

    

    

    Figure 1:  MRI Pelvis shows active Sacroileitis bilaterally.