Gadolinium-enhanced magnetic resonance imaging in shoulders contributes accurate diagnosis and predicting recurrence to patients with polymyalgia rheumatica.

OBJECTIVES: We aimed to evaluate whether gadolinium-enhanced magnetic resonance imaging (MRI) in shoulders can contribute to more accurate diagnosis and prediction of recurrence in patients with polymyalgia rheumatica (PMR). METHODS: Gadolinium-enhanced MRI and ultrasonography (US) in shoulders were performed in the patients who had bilateral shoulders pain and fulfilled the Bird's Classification Criteria between June 2012 and June 2018. PMR was clinically diagnosed by at least two rheumatologists. MRI and US findings assessed by independent radiologists were compared between the PMR or non-PMR patients. PMR patients were treated with 20 mg/day of prednisolone and were followed-up until June 2019 to determine any recurrences of the disease. RESULTS: PMR was diagnosed in 58 of 137 patients received gadolinium-enhanced MRI and US examinations. Enhancement of joint capsule, enhancement of rotator cuff tendon and focal bone oedema in humerus heads were frequently found in the PMR patients. If the three findings were used in combination to diagnose PMR, MRI had 76% sensitivity and 85% specificity, higher compared to US findings, which had 50% sensitivity and 72% specificity. During follow-up, PMR recurred in 24 patients. Patients with recurrent PMR were younger in age, had less enhancement of rotator cuff tendon and more synovial hypertrophy findings on their MRI. CONCLUSIONS: Gadolinium-enhanced MRI could display capsulitis, rotator cuff tendonitis and focal bone oedema in humerus heads that was sensitive and specific to patients with PMR, improving diagnostic accuracy in PMR. Rotator cuff tendonitis and synovial hypertrophy on MRI could help predict recurrence in PMR.

Anti-MDA5 Antibody-positive Clinically Amyopathic Dermatomyositis with Rapidly Progressing Interstitial Lung Disease Successfully Treated by Initiation of Combined Immunosuppressive Therapy Plus Plasma Exchange and Subsequently Switching Tacrolimus to Tofacitinib.

A 36-year-old man with inverse Gottron's sign was admitted for clinically amyopathic dermatomyositis (CADM) with rapidly progressive interstitial lung disease (RP-ILD). Early addition of plasma exchange (PE) to triple therapy improved severe respiratory failure and transiently decreased serum ferritin levels and anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) titers. Furthermore, switching from tacrolimus to tofacitinib resulted in disease remission. Recognition of the inverse Gottron's sign may allow for the earlier diagnosis of anti-MDA5 Ab-positive dermatomyositis, and early addition of PE to triple therapy and administration of tofacitinib in refractory cases may be effective for anti-MDA5 Ab-positive CADM with RP-ILD under life-threatening conditions.

Subcutaneous Panniculitis-like T-cell Lymphoma Lacking Subcutaneous Tumor Mimicking Adult-onset Still's Disease.

We herein report a case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) resembling adult-onset Still's disease (AOSD). A 40-year-old woman presented with a fever, erythema, and painful subcutaneous nodules on the trunk. Laboratory data and a bone marrow analysis showed hemophagocytic syndrome. Although AOSD was suspected, based on a histopathological evaluation of the erythema, she was diagnosed with SPTCL. She was refractory to combination chemotherapy but achieved durable remission with cyclosporine monotherapy. Genetic testing revealed a homozygous HAVCR2 c.245A>G variant (rs184868814) that had caused NLRP3 inflammasome activation. SPTCL and AOSD share a pathogenesis in terms of NLRP3 inflammasome activation, so the clinical phenotype of SPTCL reasonably mimics AOSD.