Case Series of Changes in Greyscale and Power Doppler Signals on Salivary Gland Ultrasonography after Treatment in Patients with Sjögren's Syndrome.

The usefulness of greyscale (GS) in salivary gland ultrasonography for Sjögren's syndrome (SS) has been established; however, limited information is currently available on power Doppler signals (PDs), and changes after treatment remain unknown. PDs are considered to represent glandular inflammation, which indicates the worsening of GS in later years. We examined the changes in PDs in three immunosuppressant-treated SS patients. PDs decreased, along with GS and markers of disease activity, after treatment. PDs have the potential to provide insights into glandular inflammation in real time; however, large-scale studies on their clinical usefulness are needed.

Simultaneous occurrence of accelerated nodulosis in lungs, liver, and kidneys, and acute exacerbation of interstitial pneumonia in a patient with rheumatoid arthritis: an autopsy case report.

BACKGROUND: Accelerated nodulosis (ARN) is a rare variant of rheumatoid nodules (RNs) that is characterized by a rapid onset or the worsening of RNs. It generally develops at the fingers in patients with rheumatoid arthritis (RA) receiving methotrexate (MTX). Few case reports have described ARN at an extracutaneous location. CASE PRESENTATION: An elderly patient with long-standing RA was admitted to our hospital with acute respiratory failure. Computed tomography upon admission showed diffuse ground-glass opacities superimposed with subpleural reticular shadowing and honeycombing and multiple nodules in the lungs and liver. Despite the discontinuation of MTX and introduction of an immunosuppressive regimen with pulse methylprednisolone followed by a tapering dose of prednisolone and intravenous cyclophosphamide, the patient died due to the acute exacerbation (AE) of RA-related interstitial lung disease (ILD) following the parallel waxing and waning of a diffuse interstitial shadow and pulmonary and liver nodules. At autopsy, RNs were scattered throughout both lung fields in addition to extensive interstitial changes. RNs were also detected in the liver and kidneys. The foci of cryptococcosis were mainly identified in alveolar spaces. Based on the clinical and pathological findings, these nodules were most consistent with ARN because of acute increases in the size and number of previously detected pulmonary nodules. CONCLUSION: The present case is noteworthy because ARN was concurrently detected in multiple internal organs and may be associated with the AE of RA-related ILD.

Possible Neoangiogenesis in Achilles Tendon Xanthoma with Familial Hypercholesterolemia: A Novel Approach to Achilles Tendon Xanthoma.

Achilles tendon xanthoma (ATX) is one of the typical features of familial hypercholesterolemia (FH). The morphological evaluation of ATX by X-ray radiography is widely recognized; however, the utility of other imaging modalities remains unclear. We herein report two cases of FH in which Doppler ultrasound imaging demonstrated a microvascular flow in ATX that only rarely could be observed in normal Achilles tendons. Neoangiogenesis accompanies chronic inflammation and it may play an important role in the deposition of cholesterol crystals leading to ATX. In addition to the morphological evaluation of ATX, the assessment of neoangiogenesis may therefore be essential for the evaluation of ATX.

Paraneoplastic dermatomyositis appearing after nivolumab therapy for gastric cancer: a case report.

BACKGROUND: While dermatomyositis is often associated with malignancy, several autoimmune diseases like myositis can be caused by immune checkpoint inhibitors. Differentially diagnosing malignancy-associated dermatomyositis or myositis caused by immune checkpoint inhibitors is sometimes difficult, particularly when a patient with malignancy shows the symptoms of myositis after checkpoint inhibitor administration. We experienced such a case in which we had difficulties in diagnosing paraneoplastic dermatomyositis or drug-associated myositis. In this case, all of our team initially assumed that the diagnosis was myositis caused by immune checkpoint inhibitors. However, it turned out finally that the diagnosis was paraneoplastic dermatomyositis. Because the diagnosis was unexpected, we report here. CASE PRESENTATION: We report the case of a 71-year-old Japanese man who developed clinical symptoms of myositis, such as muscle aches and weakness, after initiation of nivolumab therapy for his gastric cancer. He was initially diagnosed with nivolumab-induced myositis, because the myositis symptoms appeared after nivolumab administration, and nivolumab is known to trigger various drug-associated autoimmune diseases. However, according to his characteristic skin lesions, the type of muscle weakness, his serum marker profiles, electromyography of his deltoid muscle, and magnetic resonance imaging, he was finally diagnosed as having paraneoplastic dermatomyositis. Accordingly, treatment with intravenously administered corticosteroid pulse treatment, immunoglobulin injection, and tacrolimus was applied; his symptoms subsequently improved. However, to our regret, at day 142 after administration, he died due to rapid worsening of his gastric cancer. CONCLUSION: Differentially diagnosing paraneoplastic dermatomyositis or drug-associated myositis caused by immune checkpoint inhibitors is difficult in some cases. The differential diagnosis is crucial because it influences the decision regarding the appropriateness of the use of immunosuppressive treatment against the autoimmune diseases as well as the decision regarding the appropriateness of the continuous use of immune checkpoint inhibitors against the primary cancers. Because subclinical autoimmune disease may become overt after administering immune checkpoint inhibitors, non-apparent autoimmune diseases, which have already existed, should also be considered to avoid the delay of appropriate treatment, when symptoms of autoimmune diseases are recognized.

Systemic lupus erythematosus as the concomitant manifestation of angioimmunoblastic T-cell lymphoma.

Herein we report a case of the simultaneous occurrence of angioimmunoblastic T-cell lymphoma (AITL) and systemic lupus erythematosus (SLE) in a 76-year-old woman. She presented with fever, night sweats, and general malaise. A laboratory examination revealed leukopenia, anemia, polyclonal hypergammaglobulinemia, hypocomplementemia, positive results for anti-nuclear antibodies and anti-double strand DNA (anti-dsDNA) antibodies, and mild proteinuria. A computed tomography scan of the abdominal cavity showed multiple swollen intra-abdominal and intra-pelvic lymph nodes. A biopsy specimen obtained from the peri-iliac lymph node confirmed the diagnosis of AITL, while renal biopsy results were consistent with lupus nephritis, International Society of Nephrology and Renal Pathology Society class V. These results indicated that our patient developed SLE concomitantly with AITL. These findings will lead to further understanding of the pathogenic mechanism of SLE.

Anti-neutrophil Cytoplasmic Antibody (ANCA)-associated Vasculitis Associated with Primary Biliary Cirrhosis: A Case Report and Literature Review.

We herein report a rare case of microscopic polyangiitis with primary biliary cirrhosis (PBC) and a literature review of six previously reported cases of PBC complicated by anti-neutrophil cytoplasmic antibody-associated vasculitis. Due to the scarcity of similar reports, it was not possible to establish a true overlap syndrome or casual association. When the biliary enzyme levels are elevated in patients with vasculitis, physicians should thus be mindful of the possible coexistence of these diseases.

Five-year administration of tocilizumab to a patient with rheumatoid arthritis complicated by severe chronic heart failure.

We herein described the long-term administration of tocilizumab (TCZ) to a patient with rheumatoid arthritis (RA) complicated by three vessel coronary artery disease and severe heart failure (HF). A 41-year-old male was admitted to our hospital with exacerbated RA and congestive HF. Cardiac ultrasonography revealed diffuse hypokinesis with a left ventricular ejection fraction (LVEF) of 16.8% and New York Heart Association (NYHA) class III/IV HF. Swelling and tenderness were noted in most of his joints. Methotrexate (MTX) was initiated during his hospitalization and TCZ was introduced 6 months later. Our patient has been treated with MTX and TCZ for five years without any adverse events, and RA and HF have remained stable. Although it may be anecdotal, we suggest that TCZ may be used as a treatment option in patients with RA complicated by severe HF.

Successful combination therapy with corticosteroids, biweekly intravenous pulse cyclophosphamide and cyclosporin A for acute interstitial pneumonia in patients with dermatomyositis : report of three cases.

We report three patients with dermatomyositis (DM) complicated with acute interstitial pneumonia (AIP). All of them complained of fever and acutely worsening dyspnea and were treated immediately by combination therapies with pulse therapy with methylprednisone (mPSL) followed by corticosteroids, biweekly intravenous pulse cyclophosphamide (IVCY) and cyclosporine A (CSA). They recovered rapidly soon after an initiation of this combination regimen. Early intervention with aggressive combination therapy is life-saving for the treatment of AIP in patients with DM.

Systemic lupus erythematosus complicated by Crohn's disease: a case report and literature review.

BACKGROUND: Although patients with systemic lupus erythematosus (SLE) may experience various gastrointestinal disorders, SLE and Crohn's disease (CD) rarely coexist. The diseases may have gastrointestinal (GI) manifestations, laboratory results, and radiographic findings that appear similar and consequently differentiating between GI involvement in CD and in SLE may be difficult. We present the case of a patient with SLE and CD who developed continuous GI bleeding and diarrhea that was initially treated as SLE-related colitis to little effect. CASE PRESENTATION: A 55-year-old Japanese woman with systemic lupus erythematosus (SLE) developed continuous gastrointestinal bleeding and diarrhea since the patient was aged 30 years that was initially treated as SLE-related colitis. Although a longitudinal ulcer and aphthous ulcers in the colon were observed every examination, biopsy showed only mild inflammation and revealed neither granuloma nor crypt abscess. The patient underwent surgery for anal fistulas twice at 50 and 54 years of age and her symptoms were atypical of lupus enteritis. Colonoscopy was performed again when the patient was 55 years of age because we suspected she had some type of inflammatory bowel disease (IBD). Cobblestone-like inflammatory polyps and many longitudinal ulcers were detected between the descending colon and the cecum. Macroscopic examination strongly suggested CD. Histopathological examination revealed non-caseating granuloma and no evidence of vasculitis, consistent with CD. Introduction of infliximab dramatically relieved the patient's melena and abdominal symptoms. CONCLUSION: Diagnostic criteria for CD and SLE overlap, making them difficult to diagnose correctly. It is important to consider CD for patients who have SLE with gastrointestinal manifestations. The pathology of lupus enteritis should be clarified through the accumulation of cases of SLE combined with CD.

Intravascular large B-cell lymphoma with diffuse FDG uptake in the lung by 18FDG-PET/CT without chest CT findings.

We report a rare case of intravascular large B-cell lymphoma (IVLBCL) with diffuse fluorodeoxyglucose (FDG) uptake in the lung by (18)FDG-positron emission tomography/computed tomography (PET/CT). CT showed nodular shadow, whereas diffuse FDG uptake in PET/CT suggested IVLBCL in the lung. A random skin biopsy provided histological evidence of IVLBCL. The patient responded well to combination chemotherapy. Only two cases of IVLBCL in which diffuse pulmonary FDG uptake was demonstrated have been reported previously. FDG-PET/CT plus random skin biopsy may be useful for the early diagnosis of IVLBCL with pulmonary involvement even without convincing radiological findings in the lung.

"Possible primary antiphospholipid syndrome" with concurrent diffuse alveolar hemorrhaging and Libman-Sacks endocarditis mimicking catastrophic antiphospholipid syndrome.

Although antiphospholipid antibody syndrome (APS) is an autoimmune condition that is primarily characterized by arterial or venous thrombosis or pregnancy morbidity and the presence of antiphospholipid antibodies (aPL), recent reviews have introduced non-thromboembolic manifestations. We describe the case of a 58-year-old woman with vegetation on the aortic valve, whose initial presentation of APS abruptly developed into diffuse pulmonary hemorrhage. Despite consecutive plasma exchange procedures and the administration of corticosteroids and high-dose intravenous immunoglobulin, multiple brain infarctions developed, and the patient died of pneumonia. Although anecdotal, this case might serve as a useful example of the non-standard complications of fulminant APS.

Time of initial appearance of renal symptoms in the course of systemic lupus erythematosus as a prognostic factor for lupus nephritis.

The prognosis of lupus nephritis (LN) was studied retrospectively in two LN categories, LN manifested initially at systemic lupus erythematosus (SLE) onset (I-LN) and LN of delayed manifestation after SLE onset (D-LN), based on a chart review (C) of 154 SLE (85 LN) patients with a mean observation of 20.8 +/- 9.3 years and a questionnaire study (Q) of 125 LN patients outside our hospital with mean observation of 17.6 +/- 9.2 years. In both study groups, half of I-LN patients were relapse-free by Kaplan-Meier analysis after initial therapy, and the relapsed I-LN patients responded to retherapy at higher 5-year relapse-free rates than those of patients receiving initial therapies for D-LN. At last observation, a higher frequency of prolonged remission was shown in I-LN compared with D-LN patients (C: 22/31, 71% versus 14/49, 29%, P < 0.01; Q: 65/89, 73% versus 11/33, 33% P < 0.01) and also a higher frequency of irreversible renal damage in D-LN compared with I-LN patients (C: 25/49, 51% versus 2/31, 6%, P < 0.001; Q: 14/33, 42% versus 6/89, 7%, P < 0.001), although class IV pathology was common in patients (C) in both LN categories. Onset time of lupus nephritis in the course of SLE may affect renal prognosis.

[A case report of segmental arterial mediolysis].

We report a case of 58-year-old woman with a ruptured dissecting aneurysm of the middle colic artery (MCA). Her initial manifestation was sudden and severe right-sided abdominal pain, followed by hemorrhagic shock and acute anemia. Abdominal CT showed a right retroperitoneal hemorrhage. Emergency catheter angiography and therapeutic coil embolization of the middle colic artery were performed and micro aneurysms were enhanced in the jejunal branch. Immunological tests showed nothing abnormal. Follow-up angiography after 3 months showed that the micro aneurysms had disappeared. The patient was diagnosed as having segmental arterial mediolysis (SAM), because no definitive evidence of atherosclerosis and polyarteritis nodosa were observed. SAM is a rare disease of unknown etiology. The arterial lesions developing in elderly patients are characterized by segmental lysis of the abdominal splanchnic arteries resulting in aneurysms, and acute bleeding in a skip pattern. Multiple aneurysms and abdominal pain due to the rupture of these lesions in SAM resemble the clinical findings in polyarteritis nodosa. Differential diagnosis of the two diseases is important because steroid therapy is not beneficial for SAM.