The extension of the ankylosing spondylitis "dagger sign" into the sacrum.
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Publications and source records attributed to A Padula.
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A patient suffering from Behçet's syndrome and undifferentiated spondyloarthritis developed dactylitis and psoriasis over the next three years.
OBJECTIVE: To determine the clinical characteristics of patients with "pure" remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) syndrome, and to investigate its relation with polymyalgia rheumatica (PMR). Magnetic resonance imaging (MRI) was used to describe the anatomical structures affected by inflammation in pure RS3PE syndrome. METHODS: A prospective follow up study of 23 consecutive patients with pure RS3PE syndrome and 177 consecutive patients with PMR diagnosed over a five year period in two Italian secondary referral centres of rheumatology. Hands or feet MRI, or both, was performed at diagnosis in 7 of 23 patients. RESULTS: At inspection evidence of hand and/or foot tenosynovitis was present in all the 23 patients with pure RS3PE syndrome. Twenty one (12%) patients with PMR associated distal extremity swelling with pitting oedema. No significant differences in the sex, age at onset of disease, acute phase reactant values at diagnosis, frequency of peripheral synovitis and carpal tunnel syndrome and frequency of HLA-B7 antigen were present between patients with pure RS3PE and PMR. In both conditions no patient under 50 was observed, the disease frequency increased significantly with age and the highest frequency was present in the age group 70-79 years. Clinical symptoms for both conditions responded promptly to corticosteroids and no patient developed rheumatoid arthritis during the follow up. However, the patients with pure RS3PE syndrome were characterised by shorter duration of treatment, lower cumulative corticosteroid dose and lower frequency of systemic signs/symptoms and relapse/recurrence. Hands and feet MRI showed evidence of tenosynovitis in five patients and joint synovitis in three patients. CONCLUSION: The similarities of demographic, clinical, and MRI findings between RS3PE syndrome and PMR and the concurrence of the two syndromes suggest that these conditions may be part of the same disease and that the diagnostic labels of PMR and RS3PE syndrome may not indicate a real difference. The presence of distal oedema seems to indicate a better prognosis.
Distal extremity swelling with pitting edema due to altered lymphatic drainage has been reported in some patients with psoriatic arthritis (PsA). The edema usually affected the upper limbs in an asymmetric pattern and was resistant to therapy. We describe 2 additional cases. The distal swelling and pitting edema responded promptly and completely to corticosteroids in the first patient but persisted in the second. Lymphoscintigraphy and magnetic resonance imaging (MRI) revealed a predominant tenosynovitis in the hand without lymphedema in the first patient, and impaired lymphatic drainage without tenosynovial sheath involvement in the second. We conclude that 2 different mechanisms, characterized by a different response to therapy, may be associated with the same clinical picture of distal swelling with pitting edema in patients with psoriatic arthritis. Lymphoscintigraphy and MRI are useful in defining the structures involved and in predicting the prognosis.
OBJECTIVE: To determine the frequency and the characteristics of distal musculoskeletal manifestations in polymyalgia rheumatica (PMR). METHODS: Prospective followup study of 177 consecutive patients meeting clinical criteria for PMR, diagnosed over a 5-year period in 2 rheumatology secondary referral centers in Italy. RESULTS: Seventy-nine of the 177 patients (45%) had distal musculoskeletal manifestations. Peripheral arthritis occurred in 45 patients (25%), carpal tunnel syndrome in 24 (14%), distal extremity swelling with pitting edema in 21 (12%), and distal tenosynovitis in 5 (3%). These manifestations were usually associated with PMR proximal symptoms (69%); however, 31% of the episodes represented isolated relapse/recurrence at distal sites. Distal symptoms responded promptly to corticosteroids. No evidence of joint deformities, erosions, or development of rheumatoid arthritis was observed during the followup. The group of patients with peripheral arthritis included a higher proportion of females, had a longer duration of therapy, and had more relapses/ recurrences. Patients who had distal extremity swelling with pitting edema had a higher age at disease onset, a shorter duration of therapy, and lower initial and cumulative prednisone doses. CONCLUSION: Inflammatory involvement of distal articular and/or tenosynovial structures occurs in approximately half of the cases of PMR. Peripheral arthritis is associated with more severe disease, while distal extremity swelling with pitting edema appears to identify a more benign disease subset.
In this study we aimed at evaluating the modifications in the pharmacokinetic profile of cyclosporin A (CyA) after conversion from standard formulation (CyA-ST) to a new formulation (CyA-NF, Sandimmun Neoral) in patients with rheumatoid arthritis (RA). It was an open, crossover study that involved 15 RA patients who were on stabilized treatment with CyA-ST. The patient continued receiving CyA-ST (mean dose of 3.0 +/- 0.7 mg/kg per day) for 3 weeks and then converted 1:1 to CyA-NF for a further 3 weeks. CyA pharmacokinetics were established on day 1 (CyA-ST evaluation) and +21 (CyA-NF evaluation). The results showed that the bioavailability of CyA-NF was greater than that of CyA-ST (AUC tau, bss: 3335 +/- 1300 vs 2667 +/- 1155 ng.h/ml, P = 0.0073; AUC tau, bss ratio 1.26 +/- 0.40 vs 1.0 as reference, P < 0.05), with higher and earlier peak blood concentrations (Cmax: 677 +/- 256 vs 475 +/- 213 ng/ml, P = 0.0329; tmax: 1.5 +/- 0.7 vs 2.6 +/- 1.6 h, P = 0.0720). The pharmacokinetic profile of CyA-NF showed greater between-patient reproducibility (lower CV% for all of the considered parameters). In conclusion, when using CyA-NF instead of CyA-ST, greater and more constant exposure to CyA should be expected.
Seronegative spondylarthropathies include ankylosing spondylitis, Reiter's syndrome and reactive arthritis, psoriatic arthritis, arthritis associated with ulcerative colitis and Crohn's disease, plus other forms which do not meet the criteria for definite categories and are called undifferentiated. Recently two sets of classification criteria have been proposed for the entire group including undifferentiated forms: the European Spondylarthropathy Study Group and the Amor criteria. The prevalence of spondylarthropathies is directly correlated with the prevalence of the HLA-B27 antigen in the population. The highest prevalence of ankylosing spondylitis (4.5%) has been found in Canadian Haida Indians, where 50% of the population is B27 positive. Among Europeans the frequency of the B27 antigen in the general population ranges from 3 to 13% and the prevalence of ankylosing spondylitis is estimated to be 0.1-0.23%. Seronegative spondylarthropathies have common clinical and radiologic manifestations: inflammatory spinal pain, sacroiliitis, chest wall pain, peripheral arthritis, peripheral enthesitis, dactylitis, lesions of the lung apices, conjunctivitis, uveitis and aortic incompetence together with conduction disturbances. All of these may also occur in isolation.
Sacroiliitis is an indispensable condition for the diagnosis of ankylosing spondylitis according to the present criteria and is usually diagnosed on standard anteroposterior radiographs of the pelvis. In cases with suspicious abnormalities (grade 1 of the New York criteria) CT permits the diagnosis since it shows a higher degree of sacroiliitis. MRI is superior to CT in the early diagnosis as it can detect the cartilage abnormalities which precede bony changes. 'Romanus lesions' with 'shiny corners', 'squaring' of the vertebral bodies, syndesmophytes, spondylodiscitis and osteoporosis are the radiological findings of ankylosing spondylitis. The nonmarginal, asymmetric, coarse and broad syndesmophytes of psoriatic spondylitis and spondylitis associated with Reiter's syndrome resemble the flowing bone outgrowths of diffuse idiopathic skeletal hyperostosis (DISH). The ossification of the posterior longitudinal ligament and of the flavum ligament are rare manifestations of ankylosing spondylitis. Peripheral extra-articular enthesitis is a clinical hallmark of seronegative spondylarthropathies. Plain film radiography shows erosions and spurs but only in advanced phases. US shows the swelling of the entheses and the peritendinous soft tissues and the distension of adjacent bursae by fluid collection. MRI shows the inflammation of the bone adjacent to the insertion as well as the soft tissue changes. Dactylitis is another typical manifestation of seronegative spondylarthropathies. In the past it was thought to be due to concomitant tenosynovitis and arthritis. Recently, however, we have demonstrated with US and MRI that the 'sausage-like' appearance is due to the flexor tenosynovitis and that joint capsule distension is not indispensable.
Enthesitis is a distinctive pathological feature of spondyloarthropathy and may involve synovial joints, cartilaginous joints, syndesmoses and extra-articular entheses. This review focuses on peripheral extra-articular enthesitis which is a clinical hallmark of spondyloarthropathy. The entheses of the lower limbs are more frequently involved than those of the upper limbs, and heel enthesitis is the most frequent. Entheseal pain may be mild or moderate as well as severe and disabling. Peripheral enthesitis may be observed in all forms of spondyloarthropathy, including the undifferentiated ones, and may for a long time be the only long-standing clinical manifestation of the B27-associated disease process. Various imaging methods have been suggested for studying peripheral enthesitis. Ultrasonography and magnetic resonance imaging are the most useful because they may show alterations of the structures involved. Therapy of peripheral enthesitis consists of NSAIDs, orthoses and physical therapy. Steroid injections, second line drugs such as sulphasalazine and radiotherapy are reserved for more severe cases.
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The association between the onset of eosinophilic fasciitis and exposure to a drug or a toxin has occasionally been reported. We describe 3 patients who developed eosinophilic fasciitis a few months after they received subcutaneous calcium heparin. In 2 patients, clinical manifestations and eosinophilia improved after interruption of the therapy. Although spontaneous occurrence of eosinophilic fasciitis cannot be excluded in our patients, the temporal relationship with the beginning of subcutaneous heparin therapy raises the possibility that the syndrome might be precipitated by the drug.
OBJECTIVE: To establish by magnetic resonance imaging (MRI) and ultrasonography (US) the frequency of retrocalcaneal bursa involvement in Achilles enthesitis of spondyloarthropathy (SpA) and to compare the results of the 2 examinations. METHODS: Nineteen Achilles tendons with severe enthesitis and 9 normal tendons of 14 patients meeting the Amor criteria for SpA were examined by MRI and US. RESULTS: Both MRI and US showed a significant increase in the mean Achilles tendon thickness in the pathologic legs compared to the normal legs both at the superior calcaneal surface and 3 cm above. MRI showed retrocalcaneal bursitis in 14 (73.7%) of 19 pathologic legs and superficial bursitis in 2 (10.5%). US showed fluid collection only in 7 of 14 retrocalcaneal bursae positive on MRI, and failed to show fluid in the 2 superficial bursae involved. Using MRI as the gold standard, US showed 50% sensitivity and 100% specificity for retrocalcaneal bursa involvement and lacked sensitivity for superficial bursitis. CONCLUSION: Achilles tendon involvement in SpA is not only a disease of the enthesis but also of the adjacent bursae.
A variety of cutaneous lesions may occur in Behçet's disease (BD) both at presentation and over the course of the disease. Skin involvement of the hands and feet has been infrequently observed. We describe a patient with BD with recurrent, multiple, papulonodular cutaneous lesions affecting the palm and fingers of both hands, occurring simultaneously with aphthous stomatitis. The lesions consisted of roundish, erythematous, painful, bluish-red nodules, 0.5-1 cm in diameter, with a "pernio-like" aspect. Histologic examination revealed perivascular neutrophilic infiltrates. We suggest that cutaneous lesions with a pernio-like aspect as observed in our patient may be included in the spectrum of the cutaneous manifestations of Behçet's disease.
BACKGROUND: The cause of musculoskeletal symptoms in the proximal extremities of patients who have polymyalgia rheumatica is not completely understood. The diffuse and severe discomfort can only be partially explained by the mild joint synovitis that is observed in these patients. OBJECTIVE: To determine the involvement of the synovial structures of the shoulder girdle of patients who have active symptoms of polymyalgia rheumatica. DESIGN: Case-control study. SETTING: 2 secondary referral centers of rheumatology. PATIENTS: 13 case-patients who had active symptoms of polymyalgia rheumatica seen during a 6-month period, 9 control-patients who had early symptoms of elderly-onset rheumatoid arthritis, and 10 age-matched healthy controls. MEASUREMENTS: Magnetic resonance imaging of the shoulder was done on the 13 case-patients, 9 control-patients, and 10 healthy controls. RESULTS: The frequency of subacromial and subdeltoid bursitis was significantly higher in the case-patients (who had polymyalgia rheumatica) than in the control-patients (who had elderly-onset rheumatoid arthritis). The frequencies of synovitis of the joints and tenosynovitis of the biceps did not significantly differ between the 13 case-patients and the 9 control-patients. None of the healthy controls showed evidence of fluid accumulation in the joints, bursae, or sheaths of the long head of the biceps. CONCLUSIONS: Inflammation of subacromial and subdeltoid bursae in association with synovitis of the glenohumeral joints and tenosynovitis of the biceps may contribute to the diffuse discomfort in the shoulder girdle observed in patients with polymyalgia rheumatica.
The case of a man suffering from undifferentiated seronegative spondyloarthropathy (uSpA) without spine involvement and with iliolumbar ligament ossification is reported. Unlike a similar previously published case showing ossification only of the middle part of the left ligament, our patient had ossification of the whole course of both ligaments.
OBJECTIVE: To evaluate the efficacy and toxicity of cyclosporin A (CsA) in the treatment of patients with psoriatic arthritis (PsA). METHODS: We reviewed the literature dealing with CsA treatment of PsA. RESULTS: In the 1980s, some studies evaluating CsA in severe cases of psoriasis documented an improvement in the associated arthritis. Subsequently, open prospective studies included patients with active peripheral arthritis. Using initial CsA dose of 3 to 6 mg/kg/day, improvement in the clinical parameters was noted. A controlled trial showed that CsA and methotrexate (MTX) are equally effective treatment for PsA. CsA and MTX combination was effective in PsA patients resistant to previous second-line therapy. No studies have evaluated the efficacy of CsA on axial disease and on the progression of radiological damage. The most important side effect was nephrotoxicity. However, of 170 CsA-treated patients in 16 studies, only 10 (6%) discontinued the drug because of renal side effects. CONCLUSIONS: CsA seems to be an effective and safe therapy for PsA. However, controlled studies on large number of patients are necessary.
OBJECTIVE: To investigate using magnetic resonance imaging (MRI) the part played by flexor and extensor tenosynovitis and synovitis of the metatarsophalangeal (MTP), proximal interphalangeal (PIP) and distal interphalangeal (DIP) joints in producing the "sausage-like" aspect of spondyloarthropathy (SpA) toe dactylitis. METHODS: Twelve sausage-like toes and corresponding contralateral toes of 7 consecutive patients meeting Amor criteria for SpA were studied by MRI. RESULTS: All dactylitic toes showed fluid collections in the flexor synovial sheaths on MRI. Due to the sheath distension the plantar bone to skin distance was significantly increased (p < 0.05) in the dactylitic toes compared to normal contralateral toes. Peritendinous soft tissues were not involved since these were significantly thicker (p < 0.05) in normal toes. Extensor synovial sheaths were involved in only 4 dactylitic toes. Of the 36 joints of the 12 dactylitic toes only 2 MTP joints showed capsule distension. Considering MRI as the "gold standard", examination showed 100% sensitivity and specificity for flexor sheath involvement but lacked sensitivity for extensor synovial sheaths and showed a low specificity for joint capsule distension. CONCLUSION: Like finger dactylitis, toe dactylitis may also be due to flexor tenosynovitis and synovitis of MTP, PIP, and DIP joints may not be a required condition for sausage-shaped appearance. Extensor tenosynovitis may be present in addition to flexor tenosynovitis. Physical examination is a sufficient method for diagnosing toe dactylitis.
OBJECTIVE: To identify isolated peripheral enthesitis and/or dactylitis as a subset of psoriatic arthritis (PsA) and to define the clinical characteristics of these patients. METHODS: We examined 401 unselected patients with PsA seen in 3 Italian rheumatological centers over a 6 month period. The diagnosis of PsA was based upon the clinical experience of a rheumatologist. The clinical features of patients with PsA were assessed by clinical examination and review of the patients' charts, evaluating the presence of peripheral arthritis, spinal involvement, dactylitis, and enthesitis. A series of 483 rheumatological patients without psoriasis and spondyloarthropathy (European Spondylarthropathy Study Group criteria) seen consecutively in a one month period constituted the control group. RESULTS: 14 patients (3.5%) presented isolated episodes of peripheral enthesitis and/or dactylitis. No patient developed peripheral arthritis and/or axial involvement during the followup period (median 30 mo; range 3-72 mo). 10/14 patients (71%) presented at least one episode of finger and/or toe dactylitis. 5 of these 10 patients (50%) had additional episodes of peripheral enthesitis (Achilles tendinitis, plantar fasciitis, and posterior tibial tendinitis). Episodes of Achilles tendinitis and/or plantar fasciitis were present in 8/14 patients (57%). 3 of these 8 patients (37%) had associated peripheral enthesitis in other sites as well: lateral epicondyle, insertion of the patella tendon into the inferior pole of the patella, femoral quadriceps, and posterior tibial tendons. An additional case had posterior tibial tendinitis and 2 episodes of toe dactylitis. None of these 14 cases presented radiological evidence of sacroiliitis and only one of the 13 typed was HLA-B27 positive. 12 patients (2.4%) of the control group had episodes of peripheral enthesitis (11 plantar fasciitis and one Achilles tendinitis). No patient had episodes of dactylitis. The frequency of isolated Achilles tendinitis and/or dactylitis was significantly higher in patients with PsA compared to controls (3.5 vs 0.2%; p = 0.001). CONCLUSION: In some patients PsA can occur only with peripheral enthesitis, particularly Achilles tendinitis, and/or dactylitis. These patients may represent a subset of PsA, not defined by Moll and Wright and spondyloarthritis classification criteria, and poorly recognized in the studies on PsA.