Israel Medical Association Journal

A 52-year-old man with a history of gout presented to the emergency department with painful purulent ulcers on the left index finger. Serum C-reactive protein was elevated to 112 mg/L. Hand radiographs [Figure 1A] demonstrated almost complete osteolysis of the two distal phalanges of the involved finger (asterisk), with multiple typical gouty erosions with sclerotic margins and overhanging edges in a marginal and juxta-articular distribution (white arrows), and soft tissue tophi (black arrows). Osteomyelitis of the index finger was suspected, and the finger was amputated.

Idiopathic inflammatory myopathies (IIM) are a group of rare, autoimmune, systemic diseases with a large spectrum of clinical phenotypes. The diagnosis and management of myositis demand an integrated evaluation of different clinical, laboratory, and pathological findings in various organs. Recent developments in IIM research, especially in the serological testing and pathology fields, has led to a new classification and better recognition of patients with early or extra-muscular disease, with improvement in clinical care and prognosis.

Background: Autologous hematological stem cell transplantation (HSCT) is a novel therapy for systemic sclerosis (SSc) that has been validated in three randomized controlled trials.

Objectives: To report the first Israeli experience with HSCT for progressive SSc and review the current literature.

Methods: Five SSc patients who were evaluated in our department and were treated by HSCT were included. Medical records were evaluated retrospectively. Demographic, clinical, and laboratory data were recorded. Continuous data are presented as the mean ± standard deviation. Categorical variables are presented as frequencies and percentages.

Results: Five SSc patients were treated with HSCT. Four patients were adults (mean age 53 ± 12 years) and one was a 12-year-old pediatric patient. All patients were female. HSCT was initiated 1.4 ± 0.8 years after diagnosis. Two patients were RNA POLIII positive, two were anti-topoisomerase 1 positive, and one only antinuclear antibodies positive. All patients had skin and lung involvement. The mean modified Rodnan Skin Score was 29 ± 4.7 before HSCT, which improved to 10.4 ± 9.6 after HSCT. The forced vital capacity improved from 68 ± 13% to 90 ± 28%. Diffusing capacity of the lungs for carbon monoxide increased by 6%. Among severe adverse events were cyclophosphamide-related congestive heart failure, antithymocyte globulin-related capillary leak syndrome, and scleroderma renal crisis. All symptoms completely resolved with treatment without sequela. No treatment related mortality was recorded.

Conclusions: HSCT is an important step in the treatment of progressive SSc in Israel. Careful patient selection reduces treatment related morbidity and mortality.

Background: Magnetic resonance imaging (MRI), which has recently become the leading imaging modality in the study of ankylosing spondylitis (AS), has not been evaluated in the assessment of disease-specific changes at the craniocervical junction (CCJ) in patients with AS.

Objectives: To describe the spectrum of active inflammatory lesions at the CCJ using MRI in a cohort of patients with AS and neck pain.

Methods: The study included 18 patients with AS presenting with neck pain and a control group of 9 fibromyalgia patients matched for age and levels of neck pain. All patients underwent a focused rheumatologic examination, X-ray of the cervical spine, and a 3T MRI study, which included STIR, CUBE T2, FSE and FSE FAT SAT sequences before and after administration of gadolinium.

Results: The median age of AS patients was 43 years with a median disease duration of 7 years. Fifteen of 18 patients were under biologic treatment. Seven of 18 AS patients had evidence of cervical syndesmophytes on X-ray films. Active inflammatory lesions of atlanto-occipital joints and apical and alar ligaments were detected in MRIs in 2 out of the 18 patients with AS and in none of the patients with fibromyalgia. Both AS patients with active inflammation of CCJ detected on MRI received treatment with biological agents prior to and during the study.

Conclusions: Active inflammation of both entheses and joints of the CCJ can be demonstrated by MRI in patients with AS.

The term non-radiographic axial spondyloarthritis (nrAxSpA) was coined for patients who have a clinical picture of ankylosing spondylitis (AS) but do not exhibit radiographic sacroiliitis. The ASAS classification criteria for nrAxSpA, ensuring the recruitment of homogenous study cohorts, were accepted in 2009, although the respective diagnostic criteria for daily clinical practice have not yet been developed. The clinical diagnosis should be based on the composite of clinical symptoms and signs of the disease, HLA B27 status, and magnetic resonance imaging (MRI) of sacroiliac joints. Notably, a negative MRI or HLA B27 does not exclude the diagnosis in patients with a high clinical suspicion for nrAxSpA. The prevalence of nrAxSpA is similar to that of AS, but the former has a higher female preponderance. The rate of progression of nrAxSpA to the radiographic stage of disease (AS) ranges from 10% to 20% over 2 years. Current treatment strategies for nrAxSpA are the same as for AS and include non-steroidal anti-inflammatory drugs and inhibitors of tumor necrosis factor-alpha. While this review summarizes the current achievements in the field of nrAxSpA, further understanding of the epidemiology and natural history of the disease and, particularly, mechanisms of inflammation and subsequent new bone formation is essential for the development of new treatment strategies for nrAxSpA patients.