Howard Haw-Chang Lan

Sonographic Appearance of Subcutaneous Sarcoidosis

Sarcoidosis is a granulomatous multisystem disorder that rarely involves subcutaneous tissue. To our knowledge, the characteristic sonographic appearance of subcutaneous sarcoidosis has not been reported. Here we report the sonographic appearance of a case diagnosed as sarcoidosis involving skin and subcutaneous tissue over both knees.

Association of ultrasonographic findings of synovitis with anti-cyclic citrullinated Peptide antibodies and rheumatoid factor in patients with palindromic rheumatism during active episodes.

Objective. The purpose of this study was to investigate whether the presence of ultrasonographic findings of synovitis is associated with the presence of anti–cyclic citrullinated peptide (CCP) antibodies and rheumatoid factor (RF) in patients with palindromic rheumatism (PR) during active episodes. Methods. Clinically involved regions of 84 patients with PR during active episodes were examined with high‐resolution ultrasonography. Serum levels of anti‐CCP antibodies were determined by an enzyme‐linked immunosorbent assay, and RF levels were measured by nephelometry. Results. Thirty patients (36%) had ultrasonographic findings of synovitis during active episodes. Significantly higher positive rates of anti‐CCP antibodies and RF were observed in patients with PR who had ultrasonographic findings of synovitis compared with those who had no ultrasonographic findings of synovitis (26.7% versus 5.6%; odds ratio, 6.18; P < .05; and 30.0% versus 5.6%; odds ratio, 7.29; P < .01, respectively). The intraobserver and interobserver agreement for the detection of synovitis and Doppler signals by ultrasonographic assessment was excellent. Conclusions. Ultrasonography is a reliable method for assessing the presence of synovitis in patients with PR during active episodes. The ultrasonographic findings of synovitis are associated with the presence of anti‐CCP antibodies and RF in patients with PR.

Combination of transverse myelitis and arachnoiditis in cauda equina syndrome of long-standing ankylosing spondylitis: MRI features and its role in clinical management

The cauda equina syndrome (CES) is a rare neurological complication of ankylosing spondylitis (AS). Imaging diagnosis of CES in long-standing AS patients (CES-AS) using myelography, computed tomography (CT), and magnetic resonance imaging (MRI) were reported in the literature. They, however, demonstrate only the chronic abnormalities of CES-AS, i.e., dural ectasia, dorsal dural diverticula, and selective bone erosion at the posterior elements of the vertebrae. To our knowledge, imaging features of acute intradural inflammation in CES-AS were not described. We report a patient of CES-AS in whom MRI disclosed acute transverse myelitis and arachnoiditis along the lower spinal cord, and discuss the pathogenesis of CES-AS and the role of MRI in clinical management.

Crico-thyroid perichondritis leading to sore throat in patients with active adult-onset Still’s disease

A review of 341 adult-onset Still’s disease (AOSD) patients noted that 69% of all reported cases1 and 84% (69/82) of our series2 displayed sore throat early in the disease course. Despite the presence of severe sore throat, physical examinations showed normal findings or only mild pharyngeal infection, and imaging studies (including computed tomography (CT) scans) of the neck were negative.1–4 The lesions responsible for sore throat in active AOSD patients have not yet been explored. We performed magnetic resonance imaging (MRI) of the larynx5 in 6 active AOSD patients (3 females and 3 males; mean age 33.5 years; table 1) presenting with sore throat and fulfilling the Yamaguchi criteria.6 Our aim was to identify the lesions responsible for sore throat in AOSD patients. Throat swabs for bacterial cultures were negative and serological tests for …

Predicting the Progression of Palindromic Rheumatism to Rheumatoid Arthritis: The Role of Ultrasonography and Anti-cyclic Citrullinated Peptide Antibodies

We investigated whether sonography and anti-cyclic citrullinated peptide (anti-CCP) antibodies in Chinese patients with palindromic rheumatism (PR) during active episodes are of predictive value for development of rheumatoid arthritis (RA). Clinically involved regions of 84 PR patients during active episodes were examined with ultrasonography using a 6–13 MHz linear transducer. Serum levels of anti-CCP antibodies were determined by enzyme-linked immunosorbent assay. All patients were followed up monthly for 3 years after investigation. Thirteen (15%) of the PR patients had progressed to RA after a mean of 1.4 years (range, 0.4–3.0 years). Of these 13 patients, 11 patients (85%) had sonographic features of synovitis and 8 patients (62%) had a positive anti-CCP antibody test. The absence of sonographic features of synovitis during active episodes provided a very high 3-year predictive value in excluding the possibility of progression to RA for PR patients with a negative anti-CCP antibody test. Both the sonographic findings of synovitis and a positive anti-CCP antibody test were significant predictors for progression of PR to RA within 3 years by backward stepwise logistic regression analysis. Sonographic examination together with an anti-CCP antibody test during active episodes is useful for predicting the progression of PR to RA.

Soft Tissue Hemangiomas: High-resolution Grayscale and Color Doppler Ultrasonographic Features in 43 Patients

Background High-resolution ultrasonography (HRUS) is the first-line imaging modality for the assessment of soft tissue tumors. The reported HRUS features of soft tissue hemangiomas vary greatly. We reviewed 43 histologically proven soft tissue hemangiomas and describe the HRUS and color Doppler ultrasound (CDUS) features, and propose a convenient CDUS sign for diagnosis. Materials and Methods HRUS data of 43 proven soft tissue hemangiomas were included in this study. Data assessment included tumor margin, tumor echogenicity and echotexture, presence of phleboliths, fluid–fluid level in the masses, and CDUS features. In masses with weak or no color Doppler signal, color Doppler enhancing maneuver (CDEM) was applied, which was carried out using light compression on the masses or on the soft tissues adjacent to the masses. CDEM was considered positive when the color Doppler signal in a mass was more prominent and/or extensive. Results Twenty-three hemangiomas were ill-defined and 20 were well-defined. Twenty-six hemangiomas were hypoechoic and 17 were hyperechoic. Thirty-seven masses were heterogeneous in echotexture. Phleboliths were found in only nine hemangiomas. Thirty-seven hemangiomas had multiple cystic spaces and fluid–fluid levels were noted in 12. Color Doppler signals were detected in 37 hemangiomas, i.e. 33 masses had weak signals and four masses had strong signals. CDEM was positive in all 33 hemangiomas with weak color Doppler signals and was positive in four of six hemangiomas with no detectable color Doppler signal. Conclusion Typical HRUS features of a soft tissue hemangioma were an ill-defined or well-defined hypoechoic mass of heterogeneous echotexture with multiple cystic spaces. On CDUS, there may be no detectable or only weak color Doppler signals. CDEM was helpful in the diagnosis of soft tissue hemangiomas, especially in masses with no detectable color Doppler signal.

Sonographic Features of Nodular-type Muscular Sarcoidosis

Sarcoidosis is a granulomatous multisystem disorder that rarely involves muscle. Although characteristic ultrasound features of nodular-type muscular sarcoidosis have been reported, observations for such lesions at different disease stages are limited. We report ultrasonographic findings for nodular-type muscular sarcoidosis at 3 months and 1 year after the onset of disease in the left medial gastrocnemius muscle of a 35-year-old woman. A hypoechoic mass was initially detected in the involved muscle, and central echogenicity appeared later. It is, therefore, considered that ultrasonography can be a valuable tool in detecting and following up nodular-type muscular sarcoidosis lesions.

A Mucolipidosis III Patient Presenting Characteristic Sonographic and Magnetic Resonance Imaging Findings of Claw Hand Deformity

Mucolipidosis III (ML-III), or pseudo-Hurler polydystrophy, is an autosomal recessive Hurler-like disorder without mucopolysacchariduria. The diagnosis is challenging for rheumatologists since the musculoskeletal presentation is similar to some rheumatic diseases. We report a case of ML-III in a 16-year-old Taiwanese boy. The characteristic findings of sonography and magnetic resonance imaging (MRI) of claw hand deformity are described. A 16-year-old boy was referred to our rheumatologic clinic because of progressive claw hand deformity, multiple joint stiffness and tightness of the skin over the fingers at the age of 6 years. Sonography and MRI examination disclosed tendon sheath thickening over extensor tendons of both wrists and fingers without features of active inflammation over tendons or joints nor thickening of skin. Urinary glycosaminoglycans were normal. The diagnosis of ML-III was confirmed by the presence of elevated activities of beta-glucuronidase (2141.99 nmol/mg protein/hour), arylsulfatase A (1237.7 nmol/mg protein/hour) and alpha-fucosidase (52.95 nmol/mg protein/hour) in his plasma and decreased activity of these lysosomal enzymes in cultured skin fibroblasts. Sonography and MRI screening for claw hand deformity may offer important clues enabling early diagnosis of ML-III.

Ossified Intramuscular Hemangioma

Although hemangioma is a common benign soft-tissue tumor, the formation of intratumoral ossification is rare. Ossified intramuscular hemangioma, an infrequent benign tumor, is characterized by a progressively enlarging painless mass in the lower extremity of young female. Herein, we report a case of 27-year-old female suffered from a progressively enlarging painless mass in her left thigh. The mass showed a ”Swiss cheese”-like calcification on plain radiographs. Magnetic resonance images revealed multiple cystic spaces with marked contrast enhancement suggesting vascular nature and intratumoral non-enhanced foci of very low signal intensity. The image finding may prompt the diagnosis of ossified intramuscular hemangioma, which was confirmed by the histologic examination. Our report emphasized that the characteristic imaging features of ossified intramuscular hemangioma might be helpful for the correct diagnosis preoperatively.