Use of Quantitative Vertebral Bone Marrow Fat Fraction to Assess Disease Activity and Chronicity in Patients with Ankylosing Spondylitis

Objective@#We quantitatively measured the fat fraction (FF) in the vertebrae of patients with ankylosing spondylitis (AS) using magnetic resonance imaging (MRI) and investigated the role of FF as an indicator of both active inflammation and chronicity. @*Materials and Methods@#A total of 52 patients with AS who underwent spinal MRI were retrospectively evaluated. The FF values of the anterosuperior and anteroinferior corners of the bone marrow in the L1–S1 spine were assessed using the modified Dixon technique. AS activity was measured using the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Bath Ankylosing Spondylitis Functional Index (BASFI), AS Disease Activity Score (ASDAS), and serum inflammatory marker levels. AS disease chronicity was assessed by AS disease duration and the modified Stoke Ankylosing Spondylitis Spinal Score (mSASSS). Univariable and multivariable regression analyses were conducted to investigate the correlation between FF and other clinical characteristics. @*Results@#The mean FF ± standard deviation of the total lumbar spine was 43.0% ± 11.3%. At univariable analysis, spinal FF showed significant negative correlation with BASDAI (β = -0.474, p = 0.002) and ASDAS with C-reactive protein (ASDAS-CRP; β = -0.478, p = 0.002) and a significant positive correlation with AS disease duration (β = 0.440, p = 0.001). After adjusting for patient age, sex, and total mSASSS score, spinal FF remained significantly negatively correlated with BASDAI (β = -0.543, p 3.5) than in those with only high disease activity (2.1 ≤ ASDAS-CRP ≤ 3.5) (p = 0.010). @*Conclusion@#Spinal FF may help assess both AS disease activity and chronicity.

Use of Quantitative Vertebral Bone Marrow Fat Fraction to Assess Disease Activity and Chronicity in Patients with Ankylosing Spondylitis

Objective@#We quantitatively measured the fat fraction (FF) in the vertebrae of patients with ankylosing spondylitis (AS) using magnetic resonance imaging (MRI) and investigated the role of FF as an indicator of both active inflammation and chronicity. @*Materials and Methods@#A total of 52 patients with AS who underwent spinal MRI were retrospectively evaluated. The FF values of the anterosuperior and anteroinferior corners of the bone marrow in the L1–S1 spine were assessed using the modified Dixon technique. AS activity was measured using the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Bath Ankylosing Spondylitis Functional Index (BASFI), AS Disease Activity Score (ASDAS), and serum inflammatory marker levels. AS disease chronicity was assessed by AS disease duration and the modified Stoke Ankylosing Spondylitis Spinal Score (mSASSS). Univariable and multivariable regression analyses were conducted to investigate the correlation between FF and other clinical characteristics. @*Results@#The mean FF ± standard deviation of the total lumbar spine was 43.0% ± 11.3%. At univariable analysis, spinal FF showed significant negative correlation with BASDAI (β = -0.474, p = 0.002) and ASDAS with C-reactive protein (ASDAS-CRP; β = -0.478, p = 0.002) and a significant positive correlation with AS disease duration (β = 0.440, p = 0.001). After adjusting for patient age, sex, and total mSASSS score, spinal FF remained significantly negatively correlated with BASDAI (β = -0.543, p 3.5) than in those with only high disease activity (2.1 ≤ ASDAS-CRP ≤ 3.5) (p = 0.010). @*Conclusion@#Spinal FF may help assess both AS disease activity and chronicity.

Upper thoracic vertebral abnormalities in a patient with rheumatoid arthritis

No abstract available.

A Case of Lesch-Nyhan Syndrome Manifesting Only Chronic Gouty Arthritis without Neurologic Symptom

Deficiency of hypoxanthine-guanine phosphoribosyltransferase is a purine nucleotide disorder and is the most common genetic cause of uric acid overproduction. This disease has a wide range of spectrum with regard to neurological features depending on the extent of the enzymatic deficiency. Complete deficiency of hypoxanthine-guanine phosphoribosyltransferase, called Lesch-Nyhan syndrome, is presented with hyperuricemia and characteristic neurological manifestation and self-mutilation. Partial hypoxanthine-guanine phosphoribosyltransferase--deficient patients are presented with a various intensities of the aforementioned symptoms, from almost normal neurologic manifestation to a severe form along with hyperuricemia. We report a twenty-year-old man with complete hypoxanthine-guanine phosphoribosyltransferase mutation and Lesch-Nyhan sydrome, who manifested gouty arthritis without neurologic symptom.

Growing Heterotopic Calcification in the Prevertebral Space of a Cervical Spine as a Late Complication of Irradiation: Case Report

Heterotopic calcification following head and neck irradiation has rarely been reported. It usually develops as a late complication of radiotherapy in patients with malignancies, including breast cancer, lymphoma, and genitourinary malignancies. The occurrence of heterotopic calcification in the prevertebral space of the cervical spine has not been described as a late complication of irradiation. Here, we report a case of prevertebral heterotopic calcification in a patient with history of chemotherapy and radiotherapy for tonsil cancer 21 years ago.

A Case of Calcium Pyrophosphate Dihydrate Deposition Disease Associated with Primary Hyperparathyroidism

Calcium pyrophosphate dihydrate (CPPD) deposition disease is a heterogeneous group of diseases with CPPD crystal deposition. Aging is the most common risk factor for CPPD deposition, followed by osteoarthritis and previous injury. Occasionally, CPPD depositions are associated with familial predisposition and metabolic diseases, including hemochromatosis, primary hyperparathyroidism, hypophosphatasia, and hypomagnesemia. CPPD deposition diseases associated with primary hyperparathyroidism in Koreans have rarely been reported. Thus, we report a case of a relatively young female patient with CPPD deposition disease associated with primary hyperparathyroidism, which was diagnosed through a polarized microscopic examination of the synovial fluid and a subtotal parathyroidectomy.

A Case of Peripheral Bone Tuberculosis Mimicking Rheumatoid Arthritis / 대한내과학회지

A 30 year-old female visited our out-patient clinic with painful joint swelling in both hands and feet. Because she had tested positive for rheumatoid factor, and her inflammatory markers were elevated, the case was initially classified as rheumatoid arthritis (RA), according to the 2010 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria. However, radiographic examinations, including simple radiography and MRI, revealed that her peripheral bone lesions were compatible with bone tuberculosis. The patient also exhibited pulmonary tuberculosis (TB) on chest X-ray and CT examinations. She was treated with isoniazid (INH), rifampicin (RFP), ethambutol (EMB), and pyrazinamide (PZA), and exhibited a good response to these medications. The patient was diagnosed as having bone TB, and her peripheral bone lesions were resolved using anti-TB treatment. This was an uncommon case of bone TB mimicking RA.

An Unusual Cause of Wrist Pain; Kienbock's Disease

No abstract available.

Early-onset Childhood Sarcoidosis with Incidental Multiple Enchondromatosis

The triad of rash, arthritis, and uveitis seems to be characteristic for early-onset childhood sarcoidosis. We describe an interesting case of early-onset childhood sarcoidosis coexisting enchondromatosis, which clinically masquerade as Langerhans cell histiocytosis. A 33 months old girl presented with skin rash, subcutaneous nodules with polyarthritis, and revealed the involvement of lymph nodes as well as spleen during work-up. She also presented with multiple osteolytic lesions which pathologically proven enchondromatosis. Oral prednisone was prescribed at 2 mg/kg/day for 2 months until when subcutaneous nodules and joint swellings almost disappeared, and then slowly tapered over a period of 5 months. We report an unusual case of early-onset childhood sarcoidosis presented with osteolytic bone lesions which were irrelevant to sarcoidosis.

Acute Retropharyngeal Calcific Tendinitis in an Unusual Location: a Case Report in a Patient with Rheumatoid Arthritis and Atlantoaxial Subluxation

Retropharyngeal calcific tendinitis is defined as inflammation of the longus colli muscle and is caused by the deposition of calcium hydroxyapatite crystals, which usually involves the superior oblique fibers of the longus colli muscle from C1-3. Diagnosis is usually made by detecting amorphous calcification and prevertebral soft tissue swelling on radiograph, CT or MRI. In this report, we introduce a case of this disease which was misdiagnosed as a retropharyngeal tuberculous abscess, or a muscle strain of the ongus colli muscle. No calcifications were visible along the vertical fibers of the longus colli muscle. The lesion was located anterior to the C4-5 disc, in a rheumatoid arthritis patient with atlantoaxial subluxation. Calcific tendinitis of the longus colli muscle at this location in a rheumatoid arthritis patient has not been reported in the English literature.

A Case of the Lumbar Spine Involvement and Sacroiliitis in a Patient with Gout / 대한류마티스학회지

Although gout often initially affects the peripheral joints, gout may also involve the axial joints. The radiologic changes of axial gout are more common than are clinically recognized. According to a recent report, when the spine CT images of peripheral gout were reviewed for features of axial gout, there was about a 14% frequency of suspected axial gout. The vertebral level and the finding with the most common spinal gouty changes were L4 and lumbar facet joint erosions. We describe here the case of a 36-year-old gout patient with low back and right buttock pain and his lesions were unexpectedly diagnostic of lumbar facet joint arthritis and right sacroiliitis.

Chronic Finger Tip Pain / 대한류마티스학회지

No abstract available.

Camptodactyly, Arthropathy, Coxa vara, Pericarditis (CACP) Syndrome: A Case Report

The camptodactyly-arthropathy-coxa vara-pericarditis syndrome (CACP) is characterized by congenital or early-onset camptodactyly, childhood-onset noninflammatory arthropathy associated with synovial hyperplasia. Some patients have pro-gressive coxa vara deformity and/or noninflammatory pericardial effusion. CACP is inherited as an autosomal recessive mode and the disease gene is assigned to a 1.9-cM interval on human chromosome 1q25-31. We describe a 10-yr-old boy who has typical features of CACP without familial association.

The Clinical Significance of Bone Mineral Density Measurement in Patients with Ankylosing Spondylitis / 대한류마티스학회지

OBJECTIVE: Decrease in bone mineral density (BMD) occurs relatively early and is known to be correlated with disease activity in patients with ankylosing spondylitis (AS). We investigated BMD and its correlation factors in Korean patients with AS. METHODS: Fifty patients with AS fulfilling modified New York criteria and age-, sex-, and body mass index-matched 100 normal controls were selected. Medical records were reviewed retrospectively. Lumbar and femur BMD was measured using a dual energy X-ray absorptiometry. RESULTS: Both lumbar and femur BMD in patients with AS were significantly lower than BMD in control group. Although Bath ankylosing spondylitis disease activity index, C-reactive protein and erythrocyte sedimentation rate showed no correlation with BMD, lumbar BMD in patients with high disease activity in terms of inflammatory factors was significantly lower than that in patients with low disease activity. CONCLUSION: BMD in patients with AS was significantly lower than those in control group. Early detection and appropriate management will be necessary in patients with AS.

Pulmonary Manifestations of Systemic Lupus Erythematosus

Pulmonary involvement is more common in systemic lupus erythematosus (SLE) than in any other connective tissue disease, and more than half of patients with SLE suffer from respiratory dysfunction during the course of their illness. Although sepsis and renal disease are the most common causes of death in SLE, lung disease is the predominant manifestation and is an indicator of overall prognosis. Respiratory disease may be due to direct involvement of the lung or as a secondary consequence of the effect of the disease on other organ systems.

Pulmonary Complications in Renal Transplantation

PURPOSE: To evaluate the radiographic and CT findings of pulmonary complications other than pulmonary edema arising from renal transplantation. MATERIALS AND METHODS: Among 393 patients who had undergone renal transplantation at our hospital during a previous ten-year period, 23 with pulmonary complications other than pulmonary edema were included in this study. The complications involved were infection caused by CMV (n=6), bacteria (n=4), fungus (n=4), tuberculosis (n=2), varicella (n=1) or chlamydia (n=1), and malignancy involving lung cancer (n=4) or Kaposi's sarcoma (n=1). Two chest radiologists reviewed all images. RESULTS: The complications manifesting mainly as pulmonary nodules were lung cancer(4/4), tuberculosis (1/2), and Kaposi's sarcoma(1/1). Pulmonary consolidation was a main feature in bacterial infection(4/4), fungal infection(3/4), tuberculosis(1/2), chlamydial infection(1/1), and varicellar pneumonia(1/1). Ground-glass attenuation was a main CT feature in CMV pneumonia(4/6), and increased interstitial marking was a predominant radiographic feature in CMV pneumonia(2/6). CONCLUSION: The main radiologic features described above can be helpful for differential diagnosis of the pulmonary complications of renal transplantation.

CT Spectrum of Transient Peritumoral Hyperemia after Radiofrequency Thermal Ablation of Hepatic Tumors

PURPOSE: To determine the incidence and pattern of peritumoral hyperemia at CT after radiofrequency thermal ablation of hepatic tumors, as well as its correlation with local marginal recurrence. MATERIALS AND METHODS: Forty-five tumor nodules in 28 patients with hepatocellular carcinoma (n=34) or metastasis (n=11) were treated by RF thermal ablation. Serial follow-up contrast- enhanced CT scans were reviewed by three radiologists for 1) the presence, 2) the morphological characteristics [circumferential, THAD (transient hepatic attenuation difference), nodular form], and 3) the temporal course (acute, persistent, delayed onset pattern) of peritumoral hyperemia after RF thermal ablation. These findings were correlated with the frequency of recurrence at the margin of the treated tumors. RESULTS: The frequency of acute hyperemia observed on immediate follow-up CT scans after RF ablation was 71% (32/45). There was the local recurrence in nine of 32 tumors (28%) with hyperemia and in one of 13 (8%) without hyperemia (p>0.05). Among 32 tumors, the circumferential form was observed in 22 (69%); the THAD form in eight (25%); and the nodular form in two (6%). Marginal recurrence was noted in five of 22 tumors circumferential tumors (23%), in three of eight (38%) with the THAD form, and in one of two (50%) which were nodular. Among 32 nodules, an acute transient pattern was noted in 21 (66%), a persistent pattern in nine (28%), and a nodular pattern in two (6%). There was marginal recurrence in two (10%) of 21 tumors with acute transient hyperemia, in six (67%) of nine with persistent hyperemia, and in both tumors with delayed-onset hyperemia. There was significant correlation between the pattern of persistent/delayed-onset hyperemia and marginal tumor recurrence of (p=0.001). CONCLUSION: Although peritumoral hyperemia after RF thermal ablation therapy is a frequent transient finding at follow-up imaging studies, its temporal pattern may help detect early marginal tumor recurrence.

The Factors Related to Local Recurrence after Radiofrequency Thermal Ablation of Hepatic Malignancies : Assessment of Spiral CT Findings

PURPOSE: To determine the factors that are related to local recurrence after Radiofrequency thermal ablation (RFTA) of hepatic tumors. MATERIALS AND METHODS: We selected 30 patients with 51 hepatic nodules less than 5 cm in diameter (HCC, n=33; metastasis, n=18) who underwent RF thermal ablation between May 1999 and April 2000. Ablation was defined as 'complete'if immediately post-procedual CT showed that a nodule's margin was completely covered by ablation. Every three months, follow-up CT scans were examined for signs of local recurrence, and a nodule was assessed in terms of its size, the histologic diagnosis, adjacent vessels, perfect ablation (a safety margin of more than 5mm beyond the tumor margin), and whether hyperemia was observed after ablation. Finally, a group in which there was local recurrence (group A, n=15) and another showing no recurrence (group B, n=36) were compared. RESULTS: Mean nodule size in group A and group B was 3.26 and 2.24 cm, respectively. Local recurrence was noted in 7 of 33 HCC nodules (21.2%), and in 8 of 18 (44.4%) which were metastatic. There was recurrence in 9 of 31 nodules with adjacent vessels (29.0%), and in 6 of 20 (30%) without adjacent vessels. In two of 17 perfectly ablated nodules (11.8%) there was local recurrence, but this was observed in 13 of 34 imperfectly ablated nodules (38.2%). Finally, local recurrence was seen in 14 of 36 nodules showing hyperemia (38.9%) but in one of 15 (6.7%) without hyperemia. Using chi-square analysis, it was thus shown that with regard to local recurrence, tumor size, perfect ablation and peritumoral hyperemia were statistically significant factors (p<0.05). CONCLUSION: Local recurrence after RF thermal ablation of hepatic tumors clearly increases in nodules which are larger. The degree to which ablation is perfect, and the presence of peritumoral hyperemia, may be factors related to the local recurrence observed after RFTA.

Radiofrequency Thermal Ablation of Metastatic Liver Tumors: Usefulness of Combined Chemotherapy

PURPOSE: To assess the usefulness of radiofrequency (RF) thermal ablation with combined chemotherapy for the treatment of metastatic liver tumors. MATERIALS AND METHODS: A non-randomized, comparative study was performed in 21 patients with metastatic liver tumors. Inclusion criteria were that these should be less than five in number and less than 6 cm in diameter. Two groups were designed for comparison of the local and remote (new intrahepatic or extrahepatic) tumor control rate (Group A: RF alone, n=11; Group B: RF+combined chemotherapy, n=10). There was no significant difference in age, sex, and mass size between the two groups (p>0.05). All ablations were performed percutaneously with a 50W RF generator and 15G-needle electrode (RITA Medical System Inc.) under US guidance. In group B, six cycles of systemic chemotherapy were performed every month immediately after RF ablation. Follow-up CT scans were obtained within 24 hours of ablation and were compared with the findings of pre-ablation CT scanning. If an ablated lesion covered the mass without any residual enhancing foci, this was defined as complete ablation. Three and six months after ablation, local and remote tumor control rates were compared between the two groups (follow up: range 4 -17 (mean, 10.2) months. RESULTS: In group A, the local tumor control rate was 43.8% (7/16) and 31.2% (5/16) at 3 and 6 months follow-up, respectively, while in group B, the corresponding rates were both 75% (15/20). At three months, the difference in this rate between the two groups was not significantly different (p>0.05), but at 6 months there was significant difference (p<0.05). At 6 months follow-up, the remote tumor control rate for Group A and Group B was 27.3% (3/11) and 80.0% (8/10), respectively, reflecting a significant difference between the two groups (p<0.05). CONCLUSION: In patients with metastatic liver tumor, radiofrequency thermal ablation with combined chemotherapy may be superior to RF thermal ablation alone for both local and remote tumor control.

Imaging Findings of the Brain Abnormalities in Acute Lymphoblastic Leukemia of Children during and after Treatment

PURPOSE: We evaluated the imaging abnormalities of the brain observed during and after treatment of acute childhood lymphoblastic leukemia. MATERIALS AND METHODS: The study group consisted of 30patients (male: female= 19:11; mean age, 64months) with acute childhood lymphoblastic leukemia during the previous ten-year period who had undergone pro-phylaxis of the central nervous system. Irrespective of the CNS symptoms, baseline study of the brain involving CT and follow-up CT or MRI was undertaken more than once. We retrospectively evaluated the imaging findings, methods of treatment, associated CNS symptoms, and the interval between diagnosis and the time at which brain abnormalities were revealed by imaging studies. RESULTS: In 15 (50% ; male : female=9:6 ; mean age, 77months) of 30 patients, brain abnormalities that included brain atrophy (n=9), cerebral infarctions (n=4), intracranial hemorrhage (n=1), mineralizing microangiopathy (n=2), and periventricular leukomalacia (n=3) were seen on follow-up CT or MR images. In four of nine patients with brain atrophy, imaging abnormalities such as periventricular leukomalacia(n=2), infarction (n=1) and microangiopathy (n=1) were demonstrated. Fourteen of the 15 patients underwent similar treatment; the one excluded had leukemic cells in the CSF. Six patients had CNS symptoms. In the 15 patients with abnormal brain imaging findings, the interval between diagnosis and the demonstration of brain abnormalities was between one month and four years. After the cessation of treatment, imaging abnormalities remained in all patients except one with brain atrophy. CONCLUSION: Various imaging abnormalities of the brain may be seen during and after the treatment of acute childhood lymphoblastic leukemia and persist for a long time. In children with this condition, the assessment of brain abnormalities requires follow-up study of the brain.