Utility of a simplified ultrasonography scoring system among patients with rheumatoid arthritis: A multicenter cohort study

We aimed to evaluate the utility of a simplified ultrasonography (US) scoring system, which is desired in daily clinical practice, among patients with rheumatoid arthritis (RA) receiving biological/targeted synthetic disease-modifying antirheumatic drugs (DMARDs).A total of 289 Japanese patients with RA who were started on tumor necrosis factor inhibitors, abatacept, tocilizumab, or Janus kinase inhibitors between June 2013 and April 2019 at one of the 15 participating rheumatology centers were reviewed. We performed US assessment of articular synovia over 22 joints among bilateral wrist and finger joints, and the 22-joint (22j)-GS and 22-joint (22j)-PD scores were evaluated as an indicator of US activity using the sum of the GS and PD scores, respectively.The top 6 most affected joints included the bilateral wrist and second/third metacarpophalangeal joints. Therefore, 6-joint (6j)-GS and -PD scores were defined as the sum of the GS and PD scores from the 6 synovial sites over the aforementioned 6 joints, respectively. Although the 22j- or 6j-US scores were significantly correlated with DAS28-ESR or -CRP scores, the correlations were weak. Conversely, 6j-US scores were significantly and strongly correlated with 22j-US scores not only at baseline but also after therapy initiation.Using a multicenter cohort data, our results indicated that a simplified US scoring system could be adequately tolerated during any disease course among patients with RA receiving biological/targeted synthetic DMARDs.     

A case of synchronous primary lung cancer with hamartoma]

We present a case of a synchronous primary lung cancer adjacent to a hamartoma. In a 48-year-old man, a nodular shadow was found in the right middle lung field in 1990, and had grown slowly for 10 years. Another mass shadow was detected in the right upper lung field in 2000. The patient was admitted to our hospital for further examination of these abnormal shadows. Bronchoscopic examination revealed, in the right upper lobe, a poorly differentiated adenocarcinoma of clinical stage IIIB. Neoadjuvant chemotherapy followed by pneumonectomy was performed. The microscopic findings for the tumor resected from the right S2 showed poorly differentiated adenocarcinoma and those for the other, in the right S3, showed chondromatous hamartoma. Some 50 reports of lung cancer in parents with a chondromatous hamartoma have appeared in the literature. Most of these cases have some common features as follows: 1) men past middle age, 2) adenocarcinoma, and 3) lung cancer and chondromatous hamartoma present in the same lobe. The present case had all of these features, and may assist in the understanding of the process of development of lung cancer adjacent to a hamartoma.     

Positron emission tomography-positive squalene-induced lipoid pneumonia confirmed by gas chromatography-mass spectrometry of bronchoalveolar lavage fluid

Squalene is a type of oil obtained from shark liver. We describe a 76-year-old man diagnosed with chronic exogenous lipoid pneumonia due to squalene. A chest CT scan revealed pulmonary consolidation with ground-glass opacities in the right upper lobe. Positron emission tomography (PET) revealed significant uptake of 2-deoxy-2-F-fluoro-d-glucose (FDG) and 3'-deoxy-3'-F-fluorothymidine (FLT). Bronchoalveolar lavage (BAL) fluid contained many lipid-laden macrophages, and a transbronchial lung biopsy specimen showed clusters of foamy macrophages in alveolar spaces and granulomatous lesions. In addition, the presence of squalene in the BAL fluid was confirmed by gas chromatography-mass spectrometry, leading to a diagnosis of squalene-induced lipoid pneumonia. To the best of our knowledge, this is the first report of squalene-induced lipoid pneumonia in which squalene itself was successfully detected. This case also suggests the possibility that lipoid pneumonia shows significant uptake in FDG-PET and FLT-PET.     

Aplastic Anemia in a Patient with Cronkhite-Canada Syndrome

Cronkhite-Canada syndrome (CCS) is a rare polyposis disorder accompanied by alopecia and onychodystrophy. A 63-year-old man with a history of CCS and repeated embolism developed progressive thrombocytopenia and mild anemia. Laboratory testing, a bone marrow examination, and magnetic resonance imaging of the spine resulted in a diagnosis of concurrent aplastic anemia (AA). Paroxysmal nocturnal hemoglobinuria (PNH)-type cells were detected in a peripheral blood specimen. In addition, human leukocyte antigen (HLA) included DRB1*15:01 and DRB1*15:02. Mesalazine was discontinued in consideration of possible drug-induced pancytopenia. Immunosuppressive therapy ameliorated both the gastrointestinal symptoms of CCS and pancytopenia. A common autoimmune abnormality might underlie both CCS and AA.     

Three cases of non-specific interstitial pneumonia associated with primary lung cancer

NSIP associated with primary lung cancer has been rarely reported. In the present report, three cases of histologically proven non-specific interstitial pneumonia (NSIP) associated with primary lung cancer are described. Importantly, in our 3 cases, interstitial pneumonia which is histologically proven to be NSIP was observed diffusely in both lungs. NSIP in these 3 cases responded to steroid therapy. However, 2 patients died from primary lung cancer and 1 patient died from progression of the interstitial pneumonia. Although the association between lung cancer and NSIP has been rarely documented, this combination was considered to be one of the paraneoplastic phenomena. The possible association between primary lung cancer and NSIP is discussed.     

Hepatic venous outflow obstruction after right lateral sector living-donor liver transplantation,treated by insertion of an expandable metallic stent

Hepatic venous outflow obstruction is a relatively uncommon but important and devastating complication occurring after liver transplantation. Recently, right lateral sector liver grafts have sometimes been used in living-donor liver transplantation (LDLT), but, to our knowledge, early hepatic venous outflow obstruction has never been reported in right lateral sector LDLT. A 58-year-old woman was diagnosed with liver cirrhosis and hepatocellular carcinoma and underwent right lateral sector LDLT. Postoperatively, she developed liver dysfunction. Doppler ultrasound examination revealed flat waveforms and low-flow velocity in the right hepatic vein (RHV). A computed tomography (CT) scan revealed a ventrally distorted RHV due to hypertrophy of the liver graft. Hepatic venous obstruction was suspected and a hepatic venogram was performed. The venogram revealed stenosis of the RHV due to the distortion of the vein. We performed percutaneous transfemoral balloon dilatation, but this was not effective. We then inserted an expandable metallic stent (EMS) into the RHV. After the EMS placement, the condition of the patient improved. Venogram and CT data suggested that the obstruction of the RHV developed because of distortion of the RHV to the ventral side during liver regeneration.     

Implications of carotid arteriomegaly in patients with aortic aneurysm

The pathophysiology of aortic aneurysm is complex and it has remained unclear how frequently arteriomegaly, a diffuse dilatation of the artery, is associated with aneurysm. Therefore, ultrasonic study of the carotid artery was conducted to clarify this issue in a large number of subjects. Carotid ultrasonography was performed in 1,108 Japanese men aged 50 or older, and the results of 379 patients with arteriosclerotic aortic aneurysm (AA) were compared with those of 211 patients with peripheral arterial disease, 65 patients with aortic dissection, 232 hypertensive subjects, and 221 normotensive subjects. The carotid diameter was measured bilaterally at two points on the common carotid artery, and we defined carotid arteriomegaly as an arterial diameter in the 95th percentile or above that in the normotensive control group according to the relevant age subgroups. The incidence of carotid arteriomegaly in the AA group (25.9%) was significantly higher than in the other groups (p < 0.01) even when adjusted for body height and blood pressure. In the arteriomegaly subgroup, hypertension and cigarette smoking was significantly more frequent than in the non-dilated artery subgroup. This study demonstrates that one fourth of patients with aortic aneurysm have arteriomegaly as a generalized systemic abnormality in the arterial wall.     

Concurrent occurrence of allergic granulomatous angiitis and temporal arteritis: a case report and review of the literature

Allergic granulomatous angiitis (AGA) is a disease entity that was first distinguished from classical polyarteritis nodosa by Churg and Strauss in 1951, and is characterized by the clinical features of allergic rhinitis or bronchial asthma, eosinophilia, and vasculitis. Allergic granulomatous angiitis has been described to mainly involve small vessels. We herein describe a case of Churg–Strauss syndrome which demonstrated the clinical and laboratory findings supporting a diagnosis of AGA and was also associated with the clinical and pathological findings for temporal arteritis, which was characterized by eosinophil infiltration and granuloma formation of the temporal artery (middle-sized vessel).