Clinical experience in 115 patients with arthritis and/or enthesitis who met the classification criteria for psoriatic arthritis (CASPAR) within the last two years-Possible association with malignant disorders.

Among about 400 patients with active arthritis and/or enthesitis who were referred to our department within the last two years, 140 of them were strongly suspected as having psoriatic arthritis by a comprehensive diagnostic procedure and after consulting specialists from dermatology, orthopedics, and radiodiagnostics at our institution and other institutions. Among them, 115 patients strictly met the classification criteria for psoriatic arthritis (CASPAR). Among the 115 patients, 19 patients (9 males and 10 females) had current psoriasis and 96 patients (22 males and 74 females) did not have current psoriasis. Nineteen (16.5%) of the 115 patients had developed malignant tumor before the onset of arthritis, and 4 (3.5%) developed malignant tumor after the onset of arthritis. Twenty-two of the 23 patients who developed malignancy were female and 10 patients developed breast cancer. Differential diagnoses in these 23 patients may include paraneoplastic syndrome. We consider that it is important to take into account the possibility of paraneoplastic syndrome in patients with arthritis and/or enthesitis who apparently meet the CASPAR criteria, and detailed screening and monitoring of malignant disease may be beneficial to the patients.

Safety and efficacy of upfront graded administration of trimethoprim-sulfamethoxazole in systemic lupus erythematosus: A retrospective cohort study.

OBJECTIVES: Trimethoprim-sulfamethoxazole (TMP/SMX) is effective as prophylaxis against many infections in immunocompromised patients. However, it is not commonly prescribed for patients with systemic lupus erythematous (SLE) due to the risk of adverse reactions (ADRs). An upfront graded administration protocol for TMP/SMX was adopted, and its safety and efficacy were assessed. METHODS: Data from 59 patients with SLE patients who received prophylactic TMP/SMX were retrospectively analyzed. The incidence and risk factors for ADRs in patients who received TMP/SMX before and after the introduction of graded administration were assessed. RESULTS: The incidence of ADRs was 41.9% in the non-graded administration group, vs. 10.7% in the graded administration group (p = 0.009). The rate of high fever, liver function test (LFT) abnormality, shortness of breath, and hospitalization were reduced in upfront graded administration group. In addition, a higher rate of anti-Ro/SS-A positivity was found in patients experienced ADRs (46.2% in reactors vs. 5.6% in non-reactors; p = 0.012) in the non-graded administration group. CONCLUSIONS: Upfront graded administration of TMP/SMX reduces the incidence and severity of ADRs in SLE patients. The high incidence of TMP/SMX ADRs in SLE patients was also confirmed, especially when anti-Ro/SS-A antibody is present.

MRI findings of the shoulder and hip joint in patients with polymyalgia rheumatica.

OBJECTIVES: The purpose of this study is to evaluate magnetic resonance imaging (MRI) findings of the shoulder and hip joint in patients with polymyalgia rheumatica (PMR). METHODS: MR images of a total of 25 PMR patients (23 shoulders and 6 hips), 43 rheumatoid arthritis (RA) patients (22 shoulders and 22 hips), and 50 control patients (25 shoulders and 25 hips) were examined. The following MRI findings were evaluated: In the shoulder, thickness and abnormalities of the supraspinatus tendon, effusion around the glenohumeral joint, subacromial-subdeltoid bursa, and the biceps tendon; In the hip, effusion around the acetabulofemoral joint, iliopsoas bursa, and trochanteric bursa. Periarticular soft-tissue edema and bone findings were also analyzed. RESULTS: The supraspinatus tendon was significantly thicker in PMR patients than in RA patients and control patients (p < 0.05). Severe rotator cuff tendinopathy was frequently observed in PMR patients (p = 0.002). The scores for the amount of effusions (joint, bursa, and tendon sheath in the shoulder and bursa in the hip) were much higher in PMR patients (p < 0.05). Periarticular soft tissue edema was detected more frequently in PMR patients than in RA patients and control patients (p < 0.05). CONCLUSIONS: Thick supraspinatus tendon, severe rotator cuff tendinopathy, effusion around the joints, and periarticular soft tissue edema can be good indicators for the diagnosis of PMR.

Safety and efficacy of mizoribine in patients with connective tissue diseases other than rheumatoid arthritis.

The objective of this study was to examine the safety and efficacy of mizoribine (MZR), an inhibitor of inosine monophosphate dehydrogenase, in patients with connective tissue diseases (CTDs) other than rheumatoid arthritis. We identified all patients who had ever been treated with MZR for CTDs at our institution during the period from January 2001 to May 2011. A retrospective review of medical records was performed to evaluate safety and efficacy of MZR. A total of 63 patients (13 induction and 50 maintenance therapy with MZR) were included. During 70.2 patient-years of follow-up, only one patient required discontinuation of MZR due to an adverse event. Doses of PSL were significantly decreased at last follow-up in both the induction (45.2 ± 15.6 vs. 8.4 ± 5.7 mg/day, p < 0.01) and the maintenance group (12.4 ± 7.6 vs. 9.3 ± 6.4 mg/day, p < 0.01). MZR appears to be a safe and well-tolerated steroid-sparing agent in patients with CTDs.

Diagnostic Dilemma of Syncope: Esophageal Hiatal Hernia and High-risk Bundle-branch Block.

We herein report a complicated case of recurrent syncope accompanying bundle branch block and hiatal hernia of the esophagus. An 83-year-old woman presented with syncope. Echocardiography visualized the left atrium compressed by an esophageal hiatal hernia, which had potential to decrease the cardiac output. Although she underwent esophageal repair surgery, two months after the surgery, she presented to the emergency department again with complaints of syncope. At the return visit, her face was pale and her pulse rate was 30 beats per minute. Electrocardiography showed complete atrioventricular block. On reviewing the patient's previous electrocardiography findings, we found a record of trifascicular block. This case illustrates the importance of predicting atrioventricular blocks in patients with high-risk bundle-branch blocks. Keeping in mind high-risk bundle-branch blocks will help clinicians avoid anchoring bias due to a striking image masquerading as the true diagnosis.

A case of eosinophilic granulomatosis with polyangiitis preceded by allergic bullous lesions.

A 62-year-old man with a history of diabetes mellitus was hospitalised with numbness of lower limbs, bullous lesions of the whole body, kidney dysfunction, presence of eosinophils, and elevated antineutrophil cytoplasmic antibodies to myeloperoxidase and anti-bullous pemphigoid 180 antibodies and was diagnosed with mononeuritis multiplex. Kidney and muscle biopsies showed vasculitis with fibrinoid necrosis, whereas skin biopsies showed only blister formation between the epidermis and dermis; a high eosinophilic infiltrate was present in all three tissues. These findings led to a diagnosis of eosinophilic granulomatosis with polyangiitis combined with allergic bullous lesions. Immunohistological examination indicated cytolytic eosinophils and extracellular traps, suggesting the presence of eosinophil extracellular trap cell death (eosinophil ETosis) in diseased tissue.

Laryngeal edema in drug reaction with eosinophilia and systemic symptoms syndrome due to sulfasalazine.

We present a case of a 47-year-old man with drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome. The patient had been diagnosed with rheumatoid arthritis and sulfasalazine was prescribed 4 weeks before admission. Initial symptoms with fever and rash worsened even after a discontinuation of the medication, and concomitant symptoms developed including typical manifestations of facial rash and edema sparing the periorbital area, as well as atypical laryngeal edema. Rheumatologists should be aware that sulfasalazine is derived from sulfonamide and can possibly induce DRESS syndrome, one of the life-threatening drug eruptions.

A 33-Year-Old Man With Hemoptysis and Renal Dysfunction.

CASE PRESENTATION: A 33-year-old man presented with a 10-day history of fever, dry cough, and dyspnea. He reported small amounts of frank hemoptysis that occurred several times a day for the past 3 days and a reduction in urine volume. There was no joint pain, skin rash, muscle weakness, or bleeding symptoms, except for the hemoptysis. He had a medical history of childhood asthma and untreated hypertension for the past 2 years. He had no history of smoking, recent travel, medication use, or occupational inhalation.