|CASE BASED REVIEW
|Year : 2022 | Volume
| Issue : 2 | Page : 174-179
Sacroiliitis as presenting manifestation in immune-mediated inflammatory disorders other than spondyloarthritis: Case series and review of literature
Kunal Chandwar, Kriti Kishor, PM Ankush, Mukesh Kumar Maurya, Puneet Kumar, Urmila Dhakad
Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, Uttar Pradesh, India
|Date of Submission||24-Oct-2021|
|Date of Acceptance||20-Apr-2022|
|Date of Web Publication||20-May-2022|
Dr. Urmila Dhakad
Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow - 226 003, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Sacroiliitis has long been thought to be a fiefdom of the spondyloarthritides. We present 5 different cases of Immune mediated inflammatory disorders (IMIDs) presenting with sacroilitis. Though sacroilitis on Imaging can be seen in postpartum women, Athletes and even normal people, erosions in sacroiliac joint and deep SI joint edema are likely to favour a pathological cause of sacroilitis seen on imaging. We also review the prevalence of sacroilitis in different IMIDs from the available literature.
Keywords: Immune-mediated inflammatory disorders, sacroiliitis, spondyloarthritis
|How to cite this article:|
Chandwar K, Kishor K, Ankush P M, Maurya MK, Kumar P, Dhakad U. Sacroiliitis as presenting manifestation in immune-mediated inflammatory disorders other than spondyloarthritis: Case series and review of literature. Indian J Rheumatol 2022;17:174-9
|How to cite this URL:|
Chandwar K, Kishor K, Ankush P M, Maurya MK, Kumar P, Dhakad U. Sacroiliitis as presenting manifestation in immune-mediated inflammatory disorders other than spondyloarthritis: Case series and review of literature. Indian J Rheumatol [serial online] 2022 [cited 2022 Jul 4];17:174-9. Available from: https://www.indianjrheumatol.com/text.asp?2022/17/2/174/345617
| Introduction|| |
Sacroiliitis has long been thought to be sacrosanct with a diagnosis of spondyloarthropathy (SpA). We present a case series of five cases with diverse immune-mediated inflammatory disorders (IMIDs) where sacroiliitis and inflammatory back pain were presenting manifestations.
| Case Reports|| |
A 32-year-old male patient was well until he was 18 when he started having inflammatory low-back pain (IBP), followed by additive asymmetric oligoarthritis (both ankle and right wrist joint). He has been taking alternative medications for the same with intermittent flares in between. About a year back, he started having complaints of giddiness and was diagnosed with hypertension and started on telmisartan-chlorthalidone. He started having asymmetric polyarthritis with both knees, right first and fifth metacarpophalangeal (MCP) and distal interphalangeal joints (DIP) and left ankle for the past 6 months. He also complained of significant weight loss of around 20 kg over the past year accompanied by myalgia and generalized weakness. There was no history of recurrent fever, skin lesions, orogenital ulcers, uveitis, thrombophlebitis, psoriasis, IBD, or any history suggestive of connective tissue disease. He presented with poorly controlled hypertension and polyarthritis. On examination, he had mild pallor with a pulse rate of 84/min and nonpalpable left-sided anterior tibial and dorsalis pedis arteries; blood pressure (BP) of 180/110 mmHg in the upper limbs and 150/100 mmHg in the lower limbs; and both knees, left ankle, and right first MCP and DIP and fifth MCP were swollen and tender. Blood investigations revealed mild anemia (10.7 g/dl), leukocytosis (15,600/mm3), thrombocytosis (4.45 lakh/mm3), elevated erythrocyte sedimentation rate (ESR) (54 mm/h), and C-reactive protein (CRP) (125 mg/l). Fundus examination showed bilateral Grade 2 hypertensive retinopathy, and a 2D-echocardiography suggested concentric left ventricular hypertrophy with Grade 1 diastolic dysfunction and preserved ejection fraction. Infective workup including cultures and serology for syphilis, tuberculosis, and HIV was negative. Autoimmune workup for antinuclear antibodies (ANA) by indirect immunofluorescence (IIF), rheumatoid factor, human leukocyte antigen-B27 (HLA-B27), pathergy test was negative. Computerized tomography (CT) of angiography showed wall thickening and narrowing of the lumen of the arch of the aorta, descending thoracic aorta [Figure 1], bilateral subclavian artery stenosis, nonvisualized left distal anterior tibial, peroneal, and dorsalis pedis arteries, suggestive of aortoarteritis and coronary angiography showing narrowing and thickening of the left anterior descending artery [Figure 1]. At the same time, arterial Doppler of the lower limbs showed an intimal thickening of the right common and superficial femoral artery, slow flow in the right popliteal artery and monophasic flow in all examined arteries. Magnetic resonance imaging (MRI) of the sacroiliac joint was suggestive of left-sided acute on chronic sacroiliitis and right chronic sacroiliitis [Figure 1].
|Figure 1: Patient 1: (a and b) Magnetic resonance imaging of the sacroiliac joint semicoronal cuts showing acute left-sided sacroiliitis on STIR (b) and bilateral chronic sacroiliitis on T1 (a) (orange arrows) with aortoarteritis (c), and coronary angiography showing narrowing and thickening of the left anterior descending artery (d)|
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Considering the diagnosis of Takayasu arteritis, he was initiated on 1 mg/kg prednisolone and methotrexate. Over time, his arthritis has improved, BP adequately controlled, weight regained, and laboratory parameters normalized.
An 18-year-old girl presented with 1-year history of asymmetric-onset oligoarthritis involving both knee joints and IBP. Complaints resolved within a few months of taking medications. The joint symptoms recurred about a month back with debilitating knee arthritis and IBP; within a few days, she developed acute descending paraparesis. There was no history of fever, weight loss, rash, and subcutaneous nodules. She consulted a local physician and was empirically started on antitubercular therapy for 2 months with no response. Her symptoms persisted, and she was found to have severe anemia (hemoglobin [Hb] – 4 g/dl), requiring multiple blood transfusions. At presentation, on examination, she had pallor, the joint examination was normal. On Nervous system examination power in both lower limbs was 1/5 at hip extensors, internal and external rotators, 3/5 at flexors, adductors and abductors of the hip, 3/5 at flexors and extensors of the knee with weak dorsiflexors and normal plantar flexors at the ankle, reflexes were absent and Joint position and the sensation was impaired till knees. On evaluation, her inflammatory markers were elevated (ESR – 76 mm/h and CRP – 56 mg/l), whereas liver function tests (LFTs) and renal function tests (RFTs) including electrolytes, creatine phosphokinase, and lactate dehydrogenase were within limits. ANA by IIF and HLA-B27 were negative; urine for porphobilinogen, serum and urine electrophoresis for paraproteinemia, and serum levels of Vitamin B12, folic acid, and iron were normal. Cerebrospinal fluid (CSF) examination was normal. Nerve conduction study suggested axonal polyneuropathy in both upper and lower limbs. A nerve biopsy was done, and in view of the above findings' possibility of mononeuritis multiplex secondary to polyarteritis nodosa was kept. She was started on 1 mg/kg steroids, on which weakness improved, and the nerve biopsy performed was reported as normal. MRI of the sacroiliac joint suggested right-sided acute on chronic and left-sided chronic sacroiliitis [Figure 2]. Her CT abdominal angiography did not show evidence of vasculitis. Considering unequivocal evidence of vasculitis can be as low as 20% for sural nerve biopsies, and a good response to steroids, a possible diagnosis of an undifferentiated vasculitis was kept, and pulse cyclophosphamide therapy was initiated. Her lower limb weakness improved on treatment over 6 months, and she was able to walk without support at the last follow-up with no active joints and normal Hb (13.7 g/dl).
|Figure 2: Patient 2: (a and b) Acute right sacroiliitis with effusion on STIR (a) and bilateral chronic sacroiliitis on T1 (b) (orange arrows). (c) Patient 3: Acute (Orange arrow) and significant left hip joint effusion, synovial thickening, and subarticular edema in the left femoral head and acetabulum cavity with associated protrusio acetabuli|
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A 38-year-old male presented with 1-year history of IBP, asymmetric large and small joint arthritis. It started with the left hip involvement and progressively involved both the knees, ankles, shoulder, and small joints of the hands. There was a family history of SpA, no history suggestive of psoriasis, reactive onset, or uveitis, but he had a history of hair loss, photosensitivity, and oral ulcers. At admission, there was painful restriction of hip movements, bilateral knee swelling, flexion decubitus at both elbows, painful restriction of shoulder movements (overhead abduction), and tenosynovitis of multiple flexors and extensor tendons of the hand. Investigations revealed anemia (Hb – 7.5 g/dl), elevated inflammatory markers (ESR – 128 mm/h, CRP – 69 mg/l), and ANA by IIF showing a 4+ homogenous pattern at 1:100 dilution. Line immunoassay depicted anti-SSA and anti-dsDNA positivity with anti-dsDNA levels of 80 (ref: 0–18). The C4 levels were low and C3 normal; RFTs, LFTs, and urine examination were normal. MRI of the hip and sacroiliac joint revealed significant left hip joint effusion and synovial thickening and subarticular edema in the left femoral head and acetabulum cavity with associated protrusio acetabuli; articular erosions were seen with mild effusion in the right sacroiliac joint. Colonoscopy and biopsy done to rule out IBD-associated arthritis were normal. A diagnosis of systemic lupus erythematosus (SLE) with sacroiliitis and bilateral hip arthritis was made, and the patient was started on nonsteroidal anti-inflammatory drugs, sulfasalazine, and methotrexate first along with intra-articular triamcinolone bilateral knee and left hip joint. The range of motion exercises for involved joints and sacroiliac joint mobilization exercises were advised. The flexion deformities and restricted shoulder and hip movement improved for 6 months.
A 35-year-old female who had a 10-year history of inflammatory back pain presented with Raynaud's phenomenon, gastroesophageal reflux, and inflammatory symmetrical polyarthritis predominantly involving the small joints of the hands and progressive breathlessness on exertion for the past 1 year. On examination, she had digital pits and basal end-inspiratory crepitations but no skin thickening or sclerodactyly. On evaluation, ANA by IIF was 4+ homogenous, anti-Scl-70, and anti-Ro positive online immunoassay. Pulmonary function tests were suggestive of restrictive pattern, and high-resolution CT of the thorax revealed areas of peripheral ground glass in both lower lobes, likely interstitial lung disease (ILD) (nonspecific interstitial pneumonia pattern). Complete blood count, inflammatory markers, LFTs, RFTs, and 2d-echocardiography were normal. Given chronic IBP, MRI of the sacroiliac joint revealed chronic sacroiliitis [Figure 3]. A working diagnosis of sine scleroderma with chronic sacroiliitis was made and treated with rituximab for ILD.
|Figure 3: Patient 4: (a) Chronic right-sided sacroiliitis (orange arrows) on T1 semiaxial cuts, (b) nonspecific interstitial pneumonia - interstitial lung disease on HRCT thorax. Patient 5: (c) Hilar lymphadenopathy on HRCT, and (d) MRI showing chronic sacroiliitis. HRCT: High-resolution computed tomography, MRI: Magnetic resonance imaging|
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A 40-year-old male with a family history of sarcoidosis (mother had sarcoidosis) presented with 2-week complaints of IBP, intermittent dry cough, and acute-onset bilateral ankle arthritis. On evaluation, he was HLA-B27 positive with raised ESR (64 mm/h), CRP (34 mg/l), and MRI of the sacroiliac joints showing right chronic sacroiliitis. Chest X-ray followed by subsequent high-resolution CT of the chest showed bilateral hilar lymphadenopathy with normal lung parenchyma. A transbronchial lymph node biopsy confirmed noncaseating granulomatous inflammation on histopathology. A diagnosis of sarcoidosis with right-sided sacroiliitis was made, and the patient started on 0.25 mg/kg prednisolone which was gradually tapered. At present, the patient remains asymptomatic, having stopped steroids a year back.
| Discussion|| |
Sacroiliitis has long been thought to be a fiefdom of the SpA group of disorders. This review looks into the prevalence of sacroiliitis in various IMIDs. All of our patients presented with IBP which is not a known presenting feature of many IMIDs. This emphasizes the need for detailed clinical examination and investigation in patients with IBP who have additional atypical features not commonly seen in SpA. Further, most of these patients had peripheral arthritis; hence, it can be presumed that sacroiliitis may be a part of the spectrum of the polyarticular involvement in IMIDs, although most prevalence studies [Table 1] have not shown common concurrence of axial and peripheral arthritis in IMIDs.
Another point of interest would be deciding the therapy for axial arthritis in IMIDs. Methotrexate is the therapy of peripheral arthritis for most IMIDs, but data regarding its effectiveness in axial arthritis is still missing.
Biologics used for SpA management, especially antitumor necrosis factor agents, are not commonly used in SLE or scleroderma and hence making a therapeutic choice can be difficult. Therapies used in IMIDs such as cyclophosphamide or rituximab have not been effective in the SpA spectrum of disorders.
The other question is whether a mere presence of sacroiliitis would warrant labeling the IMID having SpA overlap and would the semantics matter in clinical practice.
The prevalence of LBP in the general population is 4%–6%.
Sacroiliitis and tendinopathy have not been commonly reported in SLE patients. In a study done by Yilmaz et al. in a cohort of 285 SLE patients, 46 (16.3%) patients had a history of IBP of which 22 (7.8%) were found to have MRI evidence of sacroiliitis, one patient being HLA-B27 positive with history of uveitis. Eighteen patients had active sacroiliitis, whereas two had chronic features.
Kayacan Erdogan et al. found that in a cohort of 63 SLE patients, 13 (28.2%) had a history of classical IBP, 46 patients (73%) fulfilled the Assessment of SpondyloArthritis international Society (ASAS) criteria for sacroiliitis on MRI, and 3 were HLA-B27 positive. A total of 30 patients had a history of peripheral arthritis, whereas enthesitis was found in 9, dactylitis in 4, and family history of psoriasis in 1. Acute sacroiliitis was evident in 21 (33.3%) and chronic in 4 (12.9%).
Alekberova et al. carried out a trial to study joint pathology in patients with Behcet's disease and reported sacroiliitis in 11 patients, nine patients with bilateral pathology and 9.7% with HLA-B27 positivity. According to a study by Gur et al. in 100 Behcet's disease patients, there was a 38.1% incidence of sacroiliitis.
In a study by Osman et al., out of 100 patients with RA, 52% of patients had a history of inflammatory back pain, whereas total sacroiliitis incidence was 22% on MRI. Of the cohort of patients with sacroiliitis, 10 patients (19.2%) had unilateral sacroiliitis, 29 (55.8%) had bilateral involvement on MRI, and enthesitis coexisted in 41.9%. Three patients were HLA-B27 positive.
According to Can et al., among 167 patients with RA, 28 (16.8%) had IBP, 11 (31%) had sacroiliitis, 21 (53.8%) with acute, and 18 (9.7%) with chronic sacroiliitis.
The prevalence of sacroiliitis in a cohort of 30 systemic sclerosis patients was reported to be 31% by Arslan Tas et al., with only 2/11 being HLA-B27 positive and 8/11 patients showing both active and chronic changes on MRI.
In a recent study on 34 Takayasu arteritis patients, 11.7% had sacroiliitis and 3 had concomitant IBD. Kwon et al. reported a 7.1% incidence of sacroiliitis in 268 Takayasu patients on CT out of which 1 was HLA-B27 positive with a history of anterior uveitis, 7 had concomitant IBD, and 16 had peripheral arthritis.
Sacroiliitis in sarcoidosis has been reported mainly across case series., Kobak et al. reported an incidence of 14.2% in a study of 42 patients of sarcoidosis; 6 had accompanying enthesitis and none were HLA-B27 positive. In a similar study conducted on 61 patients by Erb et al., 4 (6.6%) had sacroiliitis, whereas IBP was present in 49/61 patients. Only one was HLA-B27 positive with no patient with enthesitis, IBD, or family history of SpA.
The incidence of sacroiliitis in gout was reported as 16.8% by Alarcón-Segovia et al., where 23/142 (16.2%) patients had radiographic evidence of chronic sacroiliitis and 15 (10.56%) with a bilateral presentation.
Yıldırım et al. reported seven cases of familial Mediterranean fever More Details (FMF) with sacroiliitis, all of whom were HLA-B27 negative, and five with bilateral and two with unilateral involvement on MRI by ASAS criteria. Aydin et al. investigated the prevalence of sacroiliitis in FMF patients and found a similar incidence (2.6%) among a 650 patient cohort. Ten patients had isolated sacroiliitis, and seven had associated diseases (five enthesitis-related arthritis, one psoriatic arthritis, and one ulcerative colitis). Sacroiliac joint tenderness was present in 77%. In a study by Langevitz et al., 11/160 patients had IBP and sacroiliitis, all with MRI evidence per ASAS criteria and 7/11 with enthesitis. None of the cases were HLA-B27 positive.
Sacroiliitis in Sjogren's syndrome was reported by Eren et al. in a cohort of 97 patients in which only two had sacroiliitis with HLA-B27 positivity through IBP was present in 21 (24.7%), whereas Jarrot et al. reported a prevalence of 20% in their cohort.
Rarely, (Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides) patients can have sacroiliitis, as mentioned by Noritake et al. in a case series of 50 Granulomatosis with polyangiitis (GPA) patients, among whom three had sacroiliitis with peripheral arthritis.
The occurrence of acute sacroiliitis with HLA-B27 positivity in inflammatory myositis was reported by Prajapati recently.
| Conclusion|| |
There is a variable reported prevalence of sacroiliitis in non-SpA arthritis and IMIDs in the literature. It should be carefully searched for in patients presenting with back pain even in the absence of classical manifestations defining SpA.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given their consent for 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.
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[Figure 1], [Figure 2], [Figure 3]