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CASE REPORT
Ahead of print publication  

Spondyloarthritis associated with inflammatory myositis: An unusual case report


 Baroda Arthritis and Rheumatism Care Centre, Vadodara, Gujarat, India

Correspondence Address:
Pradip Kumar Prajapati,
Baroda Arthritis and Rheumatism Care Centre, Kothi, Vadodara - 390 001, Gujarat
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_63_19

  Abstract 


Spondyloarthritis is an inflammatory disorder of the axial skeleton, while inflammatory myositis is an inflammatory disease affecting the striated muscles. There is no usual association of spondyloarthritis and inflammatory myositis. A 35-year-old female patient presented with proximal muscle weakness and lower back pain with a history of knee synovitis and was diagnosed to have axial spondyloarthritis with inflammatory myositis. Her radiological studies showed sacroiliitis with inflammatory edema in the thigh muscle, with laboratory investigations showing elevated levels of creatine phosphokinase and inflammatory markers and positive human leukocyte antigen B27. She responded well to treatment (prednisolone, methotrexate, analgesic, and supportive medicines) with normalization of laboratory parameters and improved clinically.

Keywords: Ankylosing spondylitis, axial spondyloarthritis, back pain



How to cite this URL:
Prajapati PK. Spondyloarthritis associated with inflammatory myositis: An unusual case report. Indian J Rheumatol [Epub ahead of print] [cited 2019 Sep 22]. Available from: http://www.indianjrheumatol.com/preprintarticle.asp?id=264875




  Introduction Top


Spondyloarthritis is an inflammatory disorder of the axial skeleton, while inflammatory myositis is an inflammatory disease affecting the striated muscles.[1],[2],[3] There is no usual association of spondyloarthritis and inflammatory myositis. Here, we present a case of a 35-year-old female with spondyloarthritis with inflammatory myositis. Rarity of this co-occurrence prompted us to report this case.


  Case Report Top


A 35-year-old female presented with muscle weakness associated with difficulty in raising both arms and difficulty in standing from the sitting position, which gradually developed over 3 months. The patient reported having a history of back pain for the past 6 months with difficulty in bending forward. Her back pain was associated with bilateral gluteal region pain with sleep disturbance due to back pain. In addition, 3 months before appearance of these symptoms, the patient had bilateral knee pain with knee synovitis, which improved after treatment with deflazacort and analgesics.

Her musculoskeletal (MSK) examination showed proximal muscle weakness and tenderness over the thigh and bilateral shoulder regions, including upper arms. The results of spinal mobility test showed reduced lumbar flexion with mild sacroiliac joint tenderness. Laboratory examination revealed mild anemia (hemoglobin, 9.5 g/dL), elevated inflammatory markers (erythrocyte sedimentation rate [ESR], 103 mm/h), abnormal liver function test (aspartate transaminase, 117 U/L; alanine transaminase, 99 U/L), elevated creatine phosphokinase (CPK) levels (up to 5052 U/L), and elevated lactate dehydrogenase (LDH) levels (949 U/L). Thyroid profile and urine routine examination results were within normal limits. Magnetic resonance imaging (MRI) of the lumbosacral spine showed irregular widening with subarticular erosions and mild fluid in both sacroiliac joints with diffuse altered signals in adjacent sacrum and ilium [Figure 1]. L3 vertebral body showing corner sign and facet joint arthropathy was seen bilaterally in the lumbar spine [Figure 2]. T2-weighted short-tau inversion recovery imaging showed diffuse hyperintense signals extensively involving iliopsoas, glutei, obturator internus, and external piriformis and visualized muscle of root of thigh representing edema [Figure 3]. Immunofluorescence assay was positive for anti-nuclear antibody with homogenous-nuclear pattern in 1:100 dilution (3 + positive). Anti-extractable nuclear antibodies (ENA) was negative (with negative of anti-Jo-1 antibody, nucleosome, and ds-DNA).
Figure 1: (a and b) Sacroiliac joints showed irregular widening with subarticular erosions and mild fluid

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Figure 2: Magnetic resonance imaging L3 vertebral body showing corner sign and facet joint arthropathy

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Figure 3: (a and b) T2-weighted-short-tau inversion recovery imaging: Diffuse hyperintense signals involving iliopsoas, glutei, obturator internus, and external piriformis and edema of muscles of the root of the thigh

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The patient was treated with prednisolone, methotrexate, analgesic, and supportive medicines. On the next follow-up visit (after 1 month), her symptoms improved and laboratory parameters also improved with reduction in CPK level (1453 U/L), LDH (624 U/L), and ESR (41 mm/h). The polymerase chain reaction for human leukocyte antigen B27 (HLA-B27) was found to be positive. After 3 months of treatment, a significant improvement in proximal muscle weakness, back pain, and near-normal CPK levels (156 U/L) was observed.


  Discussion Top


Axial spondyloarthritis is a chronic systemic inflammatory rheumatic disorder, with a predilection for axial skeletal involvement and inflammation at sites of bony insertions of tendons and ligaments (enthesitis). Sacroiliitis is the hallmark of the disease. There is a strong genetic predisposition associated with HLA-B27. The precise etiopathogenesis of axial spondyloarthritis is still unknown. The role of genes is undisputable; however, axial spondyloarthritis has been long known to be highly familial. HLA-B27 accociation is significantly higher in patients with axial spondyloarthritis compared to general population. Clinical manifestations include chronic inflammatory low back pain with stiffness, reduced spinal mobility and chest expansion. Uveitis or other less common extra-articular manifestations occur in some. Radiographic findings are characteristics of the disease (X-ray, MRI, and MSK ultrasonography for enthesitis and synovitis). Generally, patients respond well to nonsteroidal anti-inflammatory drugs.

Myositis (inflammation of striated muscles) is associated with characteristic muscle weakness, cutaneous features, and a variety of systemic complications. It is classified into three types – polymyositis, dermatomyositis, and inclusion body myositis. Polymyositis and dermatomyositis are referred collectively as myositis or idiopathic inflammatory myositis. It can occur at any age, but the occurrence is higher in females. Immunologic features include serum autoantibodies in association with myositis as a major manifestation of the syndrome. There is a typical presentation of painless proximal muscle weakness with or without rash. Muscle biopsy demonstrates inflammatory infiltrates. Among other organ systems, lung, heart, gastrointestinal tract, and joints can also be involved.

In the present case, the patient was diagnosed to have idiopathic inflammatory myositis based on clinical features, elevated muscle enzymes, and raised inflammatory markers and axial spondyloarthritis based on inflammatory back pain with radiographic evidence of sacroiliitis, and HLA-B27 positivity, which was later observed during follow-up. She was treated with prednisolone, methotrexate, analgesic, and supportive medicines and responded well to the treatment. On follow-up visits, her symptoms improved and laboratory parameters also improved with reduction in CPK to normal level. A significant improvement was observed in proximal muscle weakness and low back pain.

There are very few cases of spondyloarthritis associated with inflammatory myositis.[4],[5] In a previous case by Waragai and Shinotoh, a 21-year-old man reported a history of stiffness in his lower back when bending to pick up an object from the ground. The patient later developed dull pain in his buttocks that extended to the back and had a muscle wasting of right thigh, followed by weakness in legs and lower back pain.[4] In another case, a 35-year-old female patient had pain and tenderness in the muscles, loss of weight, and erythematous skin lesions of recent onset over the knuckles and forehead. This patient had insulin-dependent diabetes mellitus for 2 years, rheumatoid arthritis, and then subsequently ankylosing spondylitis and dermatomyositis.[5]


  Conclusion Top


A case of spondyloarthritis with inflammatory myositis is an unusual association.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Hopkins GO, McDougall J, Mills KR, Isenberg DA, Ebringer A. Muscle changes in ankylosing spondylitis. Br J Rheumatol 1983;22:151-7.  Back to cited text no. 1
    
2.
Faus-Riera S, Martínez-Pardo S, Blanch-Rubió J, Benito-Ruiz P, Duró-Pujol JC, Corominas-Torres JM. Muscle pathology in ankylosing spondylitis: Clinical, enzymatic, electromyographic and histologic correlation. J Rheumatol 1991;18:1368-71.  Back to cited text no. 2
    
3.
Calin A. Raised serum creatine phosphokinase activity in ankylosing spondylitis. Ann Rheum Dis 1975;34:244-8.  Back to cited text no. 3
    
4.
Waragai M, Shinotoh H. Ankylosing spondylitis associated with myositis. J Neurol Neurosurg Psychiatry 1994;57:661-2.  Back to cited text no. 4
    
5.
Sattar MA, Al-Sughyer AA, Siboo R. Coexistence of rheumatoid arthritis, ankylosing spondylitis and dermatomyositis in a patient with diabetes mellitus and the associated linked HLA antigens. Br J Rheumatol 1988;27:146-9.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

 
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