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Arthritis
Published in Harry Griffiths, Musculoskeletal Radiology, 2008
However, erosive osteoarthritis spares the metacarpophalangeal joints and wrists, whereas, obviously, rheumatoid arthritis involves these areas primarily (Fig. 58). In black patients, rheumatoid arthritis may present as very localized erosive changes, resembling an acute inflammatory arthritis, such as infection or even gout. This form of rheumatoid arthritis has a very old name—palindromic rheumatism (Fig. 47).
Late-onset familial Mediterranean fever in Japan
Published in Modern Rheumatology, 2020
Dai Kishida, Masahide Yazaki, Akinori Nakamura, Ayako Tsuchiya-Suzuki, Yasuhiro Shimojima, Yoshiki Sekijima
Recently another cohort study examining the age of disease onset in Japanese FMF patients has been reported [11]. In this study, late-onset FMF patients presented similarly to our results, with a lower frequency of serositis, family history of FMF, and similar percentage of exon 10 mutation. On the other hand, the frequency of musculoskeletal manifestations was reported to be higher for late-onset patients and differed from our analysis which showed no significant differences between the groups. Several previous reports found that musculoskeletal manifestations were more frequent in young onset patients compared to late onset patients [8,12,13]. Additionally, arthritis and myalgia are common symptoms of various rheumatic diseases. From a Spanish cohort of palindromic rheumatism patients, 12.5% of the patients carried some MEFV mutations with higher prevalence than would be expected [14]. Various diseases including infections, inflammatory or immunologic disorders, and malignancies were revealed as different underlying diagnoses for FMF [15]. Although further investigation will be required for evaluating musculoskeletal manifestations in Japanese FMF patients, careful differential diagnosis and observation is needed for late-onset patients that mainly have arthritis or myalgia rather than serositis. The effectiveness of colchicine and features of attacks, such as recurrence, length, and self-limited nature, will be helpful in better establishing the diagnosis.
Progression of palindromic rheumatism to juvenile idiopathic arthritis in a Japanese girl carrying heterozygous L110P-E148Q substitutions of MEFV gene
Published in Modern Rheumatology, 2018
Ichiro Kobayashi, Yasuhiro Yamazaki, Yusuke Tozawa, Masahiro Ueki, Shunichiro Takezaki, Masafumi Yamada, Tadashi Ariga
Palindromic rheumatism (PR) is characterized by recurrent arthritis or periarthritis that is separated by asymptomatic interval without residual joint destruction [1,2]. PR often progresses to chronic arthritis and is finally diagnosed as rheumatoid arthritis (RA) in adult cases [3–8]. However, PR is quite rare in children [9]. Although PR, juvenile idiopathic arthritis (JIA), and RA are considered as polygenic disorders, several reports have suggested association with mutations of MEFV gene, a causative gene of Familial Mediterranean Fever (FMF) [10–12]. We, herewith, report a 3-year-old girl with heterozygous substitution in MEFV gene who showed gradual evolution of PR to RF-negative polyarticular JIA by 9 years after the onset.
Spontaneous necrosis of a single digit: watershed necrosis
Published in Case Reports in Plastic Surgery and Hand Surgery, 2021
Alain J. Azzi, Gabriel Bouhadana, Fanyi Meng, Peter G. Davison
On examination, necrosis was noted, demarcated at the mid-middle phalanx of the third digit (Figure 1). No other skin lesions were noted and there were no signs of infection. The digit demonstrated decreased range of motion and absent sensory findings. The patient denied any symptoms of claudication, a history of prolonged cold exposure, or sensitivity to cold. Laboratory tests revealed normal liver and renal function and were not suggestive of systemic etiologies for the necrosis. The patient had no signs of an underlying inflammatory process nor any anemia, thrombocytosis or elevated C-Reactive Protein count. Albeit that the patient was positive for Rheumatoid Factor and anti-cyclic citrullinated peptide (anti-CCP) antibodies, this comes to no surprise as her rheumatism was a longstanding disease, with a remote history of flare ups that were well-controlled with trials of prednisone. Although a progression of the patients’ palindromic rheumatism into a rheumatoid vasculitis is possible, it is highly unlikely due to absence of clinical evidence of rheumatological decompensation other than digital necrosis and absence of any laboratory findings (the patient was negative for any antinuclear, anti-Smith or anti-RNP antibodies). An x-ray of the hand was unremarkable. A left upper extremity arteriogram was ordered which showed several significant findings: 80% stenosis of the radial artery at the level of the wrist, suggestive of an atheromatous plaque, absence of the DPA, an incomplete SPA at the level of the fourth and fifth digits (collaterals were seen bridging the incomplete superficial arch) and no sign of vascularity distal to the base of the middle phalanx of the third digit (Figure 2 and Supplemental Online Material 1).