High Prevalence of the Lung Ultrasound Interstitial Syndrome in Systemic Sclerosis Patients with Normal HRCT and Lung Function-A Pilot Study.

Objective: High-resolution computed tomography (HRCT) may lack sensitivity for the early detection of interstitial lung disease associated with systemic sclerosis (SSc-ILD). Lung ultrasound is an emerging technique for the diagnosis of SSc-ILD. This cross-sectional study aimed to describe the prevalence of ultrasound interstitial syndrome in SSc patients with normal HRCT and pulmonary function tests (PFT). Methods: Thirty SSc patients with normal HRCT, FVC > 80% predicted and DLCO > 70% predicted were included. Echocardiography and PFT including impulse oscillometry and cardiopulmonary exercise testing were performed. Lung ultrasound was analyzed by two blinded operators. Patients were classified into two groups, according to the presence or absence of ultrasound interstitial syndrome, defined as the sum of B-lines in all thoracic areas ≥10 and/or pleural line thickness >3 mm on at least one thoracic area and/or a pleural line irregularity score >16%. Results: Ultrasound interstitial syndrome was present in 12 patients (40%). Inter-reader agreement for the diagnosis of ultrasound interstitial syndrome defined by the Kappa coefficient was 0.93 (95%CI 0.79-1.00). Patients with ultrasound interstitial syndrome were younger (37 years vs. 53 years, p = 0.009), more often had pitting scars (n = 7/12 vs. 3/18, p = 0.045) and had lower FVC (102 vs. 110% pred, p = 0.009), TLC (114 vs. 122% pred, p = 0.042) and low-frequency respiratory system reactance (Xrs5 Z-score 0.16 vs. 1.02, p = 0.018), while pulmonary gas exchange was similar. Conclusions: Ultrasound interstitial syndrome was detected in 12/30 SSc patients with normal HRCT and PFT. Patients with ultrasound interstitial syndrome had differences in lung function consistent with reduced respiratory compliance, suggesting minimal and/or early suspected SSc-ILD.

Immunoglobulin A Vasculitis Following COVID-19: A French Multicenter Case Series.

OBJECTIVE: Immunoglobulin A vasculitis (IgAV) usually occurs following viral respiratory tract infection. In the context of the global coronavirus disease 2019 (COVID-19) pandemic, we describe a case series of patients who developed IgAV following SARS-CoV-2 infection. METHODS: This national multicenter retrospective study included patients with IgAV following SARS-CoV-2 infection from January 1, 2020, to January 1, 2022. Patients had histologically proven IgAV and reverse transcription PCR (RT-PCR)-proven SARS-CoV-2 infection. The interval between infection and vasculitis onset had to be < 4 weeks. RESULTS: We included 5 patients, 4 of whom were women with a mean age of 45 years. Four patients had paucisymptomatic infections and 1 required a 48-hour low-flow oxygen treatment. All 5 patients had purpuric skin involvement. Arthritis was observed in 2 patients, 3 had IgA glomerulonephritis, and 2 had digestive involvement. Three renal biopsies were performed and showed mesangial IgA deposits without any extracapillary proliferation. Median C-reactive protein was 180 (range 15.1-225) mg/L, median serum creatinine level was 65 (range 41-169) µmol/L, and 2 patients had a glomerular filtration rate < 60 mL/min. Four patients received first-line treatment with glucocorticoids. All patients had a favorable progression and 2 patients experienced minor skin relapses, one after COVID-19 vaccination. CONCLUSION: This series describes the emergence of IgAV closely following COVID-19; we were not able to eliminate an incidental link between these events. Their disease outcomes were favorable. In most of our patients, the SARS-CoV-2 infection was paucisymptomatic, and we recommend RT-PCR tests to look for COVID-19 in patients without any evident triggers for IgAV.

IgA vasculitis with underlying monoclonal IgA gammopathy: innovative therapeutic approach targeting plasma cells. A case series.

OBJECTIVE: There is currently no evidence of the possible benefit of plasma cell-targeting therapies (PCTT) in immunoglobulin A (IgA) monoclonal gammopathy (MG) associated with IgA vasculitis (IgAV). We report the outcome of different PCTT regimens in a cohort of MG-IgAV. METHODS: We used a French network to retrospectively describe the outcome of MG-IgAV patients treated with PCTT. RESULTS: Five patients were included (mean age 65 years). All patients had severe baseline presentation including extensive necrotic purpura (n = 5), gastrointestinal involvement (n = 2), peripheral neuropathies (n = 2), and glomerulonephritis (n = 1). Two patients had IgA indolent multiple myeloma and three had IgA "MG of undetermined significance." Monotypic IgA deposition in the skin vessels wall was highlighted using an immunofluorescence assay. Cases of vasculitis in three patients (n = 3) were refractory to multiple line therapies, including cyclophosphamide (n = 3) or rituximab. Finally, PCTT including bortezomib plus cyclophosphamide and dexamethasone, bortezomib plus melphalan and prednisone, or bortezomib plus lenalidomide and dexamethasone were proposed, allowing complete remission in 4/5 patients without major adverse drug events. CONCLUSION: This study suggests that the MG-IgAV phenotype might be distinctive of usual IgAV (severe and refractory to conventional immunosuppressive regimens) and supports the benefit of PCTT. This study sheds new light on the overall biology of IgAV, strengthening the pathogenic role of the monoclonal IgA component in IgAV.

Organizing pneumonia and occupational and environmental risk factors: a case-control study.

OBJECTIVES: A single-center case-control study was carried out to investigate the relationship between occupational and environmental exposure and organizing pneumonia (OP). METHODS: Thirty-seven cases of OP, including 25 cases of cryptogenic OP, and 111 controls were included. Occupational exposure was assessed retrospectively by an industrial hygienist and an occupational physician, through semi-quantitative estimates of exposure. An exposure score was calculated for each subject, based on probability, intensity, daily frequency, and duration of exposure for each period of employment. The final cumulative exposure score was obtained by summing exposure scores for all periods of employment. RESULTS: Significant associations with all-cause OP were observed for exposure to tetrachloroethylene (OR 13.33, CI 95% 1.44-123.5) and silica (OR 6.61, CI 95% 1.16-37.71). A significant association with cryptogenic OP was observed only for tetrachloroethylene (OR 31.6, CI 95% 1.64-610.8). No associations were found for environmental exposure. CONCLUSION: Despite its low statistical power, this work suggests that occupational risk factors could be involved in OP.

Quality of life of patients treated for giant cell arteritis: a case-control study.

The objective of the study was to assess the quality of life (QOL) of patients with giant cell arteritis (GCA), following high dose of corticosteroids (CS). Thirty patients with GCA who had stopped CS or who were under long-term low dose of CS were included and matched to 60 controls. QOL was measured by the SF-36 score and a specific questionnaire. GCA patients had no impairment of QOL compared to controls according to SF-36. Most of them (57%) estimated that their general condition was improved following treatment. Patients with GCA complications or CS therapy side effects had no significant impairment of their QOL compared with patients without complications or adverse effects. Only the patients who had gained weight had a lower score on the domain "Vitality" (VT; p = 0.013). Walking difficulties were the most frequent complaints. They were associated with impaired scores on the physical summary score (p = 0.0340) and on the "General Health" (GH; p = 0.005) and "Physical Functioning" (PF, p = 0.0298) domains. Falls among GCA patients were associated with altered scores on the domain VT (p = 0.0058) and on the mental summary score if they had fallen at least three times (p = 0.0460). GCA patients following high dose of CS or under long-term low doses of CS have no significant impairment of their QOL compared to controls. GCA complications, including visual impairment, do not seem to have any major impact on QOL.