Objectives ANCA-associated vasculitis (AAV) can affect all age groups. We aimed to show that differences in disease presentation and 6 month outcome between younger- and older-onset patients are still incompletely understood. Methods We included patients enrolled in the Diagnostic and Classification Criteria for Primary Systemic Vasculitis (DCVAS) study between October 2010 and January 2017 with a diagnosis of AAV. We divided the population according to age at diagnosis: <65 years or ≥65 years. We adjusted associations for the type of AAV and the type of ANCA (anti-MPO, anti-PR3 or negative). Results A total of 1338 patients with AAV were included: 66% had disease onset at <65 years of age [female 50%; mean age 48.4 years (s.d. 12.6)] and 34% had disease onset at ≥65 years [female 54%; mean age 73.6 years (s.d. 6)]. ANCA (MPO) positivity was more frequent in the older group (48% vs 27%; P = 0.001). Younger patients had higher rates of musculoskeletal, cutaneous and ENT manifestations compared with older patients. Systemic, neurologic,cardiovascular involvement and worsening renal function were more frequent in the older-onset group. Damage accrual, measured with the Vasculitis Damage Index (VDI), was significantly higher in older patients, 12% of whom had a 6 month VDI ≥5, compared with 7% of younger patients (P = 0.01). Older age was an independent risk factor for early death within 6 months from diagnosis [hazard ratio 2.06 (95% CI 1.07, 3.97); P = 0.03]. Conclusion Within 6 months of diagnosis of AAV, patients >65 years of age display a different pattern of organ involvement and an increased risk of significant damage and mortality compared with younger patients.

We performed a retrospective chart review of three patients with hypomyopathic dermatomyositis and rapidly progressive interstitial lung disease. The patients were Japanese women of 71, 69, and 65 years of age. Two patients were anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive and 1 was anti-aminoacyl-tRNA synthetase (anti-ARS) antibody-positive. Their respiratory statuses deteriorated despite the administration of glucocorticoid, calcineurin inhibitors, and intravenous cyclophosphamide therapy. We subsequently administered rituximab. The anti-ARS antibody-positive patient survived, while 2 anti-MDA5 antibody-positive patients died.

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Dermatomyositis-related interstitial lung disease mimicking COVID-19 pneumoniaA 51-year-old man with no signifi cant medical history presented to the emergency department with fever and dyspnea lasting for 10 days. On presentation, his body temperature was 36.6°C (97.9°F), respiratory rate 15 breaths per minute, and oxygen saturation 90% on 1 L per minute of oxygen.On physical examination, fi ne crackles were heard bilaterally at the base of the lungs. No evidence of muscle weakness was observed. Skin examination (Figure 1) revealed Gottron papules at the knees, elbows, and metacarpophalangeal joints of the hands, a skin ulcer at the knee, hyperkeratosis of the lateral aspect of the index fi nger of both hands, peri-ungual erythema, and palmar papules.Results of initial laboratory testing were as follows:• Leukocyte count 10.7 × 10 9 /L (reference range 3.3-8.6 × 10 9 /L) • Creatine kinase 257 IU/L (reference range 59-248 IU/L) • Ferritin 841.8 ng/mL (reference range 39-340 ng/mL) • C-reactive protein 5.5 mg/dL (reference range 0-0.14 mg/dL). Chest computed tomography (CT) (Figure 2) revealed bilateral peripheral subpleural ground-glass opacities in the upper and lower lobes.In the emergency department, interstitial THE CLINICAL PICTURE

Background Parvovirus B19 infection in adults has been associated with a variety of rheumatic manifestations and laboratory findings mimicking rheumatic disease. Objectives To explore the clinical features of recent parvovirus B19 infection in adults and investigate the number of patients who fulfill the classification criteria for rheumatoid arthritis (RA) or systemic lupus erythematosus (SLE). Methods We performed a retrospective chart review of adult patients with newly-diagnosed parvovirus B19 infection at a community-based hospital in Japan from April 2006 to December 2013. Diagnosis was made with positive serum anti-parvovirus B19 IgM antibody measured by enzyme-linked immunosorbent assay. Results Sixty patients were included (female, 77%; mean age, 39 years) with a mean follow-up of 118 weeks (range, 0-356). Mean disease duration was 34 days (range, 5-316) and mean duration of arthritis was 29 days (range, 7-140). The mean interval between development of the initial manifestation and the onset of arthritis was 4 days (range, 0-19). Arthritis of more than 6 weeks was seen in only one patient. A half of the patients (53%) had a recent history of exposure to people who had clinical manifestations of viral infection. In our study population, predominant signs and symptoms were: arthritis/arthralgia (87%), skin rash (65%), edema (58%), fever (55%), and lymphadenopathy (32%). Regarding arthritis/arthralgia, 18 patients (30%) had physician-diagnosed arthritis, while 34 (57%) had arthralgia alone. Commonly involved joints were in the knee (68.4%), followed by wrist (58%), hand (55%), ankle (55%), and elbow (45%). The most frequent pattern of arthritis/arthralgia was polyarticular (83%). Ten patients (17%) had rash on the cheeks, and 5 others (8%) had characteristic lacy rash on the extremities. The most frequent type of rash was pruritic erythema on the upper and lower limbs. Laboratory findings included anemia (12/60, 20%), lymphocytopenia (41/57, 72%), elevated lactate dehydrogenase (32/55, 58%), positive anti-nuclear antibody (ANA) (30/33, 91%), and hypocomplementemia (13/29, 45%). Autoantibody to DNA and anti-phospholipid antibody were positive in 32% (6/19) and 54% (7/14) of the patients, respectively. Rheumatoid factor (RF) was not detected (0/38), while anti-CCP antibody (ACPA) was detected in 11% (2/18). Persistent infection was diagnosed in two patients. There is no significant difference in baseline characteristics between arthritis group and non-arthritis group. Of all 60 patients, 12 patients fulfilled the 2012 SLICC SLE criteria (six patients met the 1997 revised ACR SLE criteria). Of 18 patients with arthritis, five patients fulfilled the 2010 ACR/EULAR RA criteria (one patient met the 1987 ACR RA criteria). Conclusions Parvovirus B19 infection in adults often presents with acute polyarthritis and skin rash accompanied with lymphocytopenia, hypocomplementemia, and positive ANA. Our study demonstrated that 20% of adult patients with recent parvovirus B19 infection fulfill the SLE criteria and...