Giacomi, Federica De

Objective To compare the effectiveness of rituximab (RTX) with cyclophosphamide (CYC) in patients who have central nervous system (CNS) involvement in antineutrophil cytoplasmic antibody–associated vasculitis (AAV). Methods A computer-assisted search was conducted to identify all adults who received a diagnosis of AAV with CNS involvement from January 1, 1997, through July 1, 2017, at our institution. Results Of the 17 patients identified, 11 had received RTX, and 6 had received CYC. Age at diagnosis of CNS involvement was similar in both groups. In the RTX group, 91% of the patients were women; in the CYC group, 33% were women (p = 0.03). At the time of CNS presentation, orbital involvement had occurred in 6 patients in the RTX group and in none of the patients in the CYC group. Initial remission of induction was achieved in all patients (100%) in the CYC group and in 10 patients (91%) in the RTX group. Two patients had no response to RTX: 1 patient when RTX was used for remission induction at the time of diagnosis and the second patient when RTX was used for remission induction after relapse. The median follow-up was 38 months (range, 9–127 months). Central nervous system relapse occurred in 4 patients in the RTX group and in 1 patient in the CYC group. Of the 4 patients in the RTX group with relapse, 3 had marked ocular involvement. Both nonresponder patients in the RTX group had ocular involvement. Conclusion Rituximab is as effective as CYC in remission induction in patients with CNS involvement in AAV.

BackgroundSpontaneous pneumomediastinum (SP) is an uncommon disorder but has been described in cases with connective tissue diseases (CTDs), most commonly dermatomyositis and polymyositis. We aimed to explore this relationship by analyzing the characteristics of CTD patients with SP.MethodsUsing a computer-assisted search, we identified 25 patients with CTD and SP evaluated between January 1997 and December 2016 at our institution. Clinicoradiologic characteristics, treatment, and outcomes were extracted and chest computed tomography studies analyzed.ResultsWe identified 25 patients with CTD and SP. Median (range) age at SP occurrence was 55 (18–82) years, and 60% of the patients were women. Smoking history was present in 37%. Spontaneous pneumomediastinum was symptomatic in 56% of patients. Eighteen patients (72%) had a known CTD diagnosis, and 20 patients (80%) manifested radiologic evidence of interstitial lung disease. Spontaneous pneumomediastinum diagnosis was achieved with chest radiography in 20% of cases and chest computed tomography in the other cases. Spontaneous pneumomediastinum was managed with expectant observation alone in 22 cases (88%). Four patients (16%) had concomitant pneumothorax, 1 of whom required chest tube drainage. There were no deaths attributable to SP during the median (range) follow-up of 13 (0–174) months. Cumulative survival was 52% at 1 year and 40% at 2 years.ConclusionsSpontaneous pneumomediastinum is an uncommon manifestation of CTD and usually occurs in the presence of interstitial lung disease. Although SP seems to be associated with a relatively benign short-term course, occurrence of SP in CTD patients may be a poor prognostic factor.

Purpose: Fibrotic interstitial lung diseases presenting with nonspecific and overlapping radiologic findings may be difficult to diagnose without surgical biopsy. We hypothesized that baseline quantifiable radiologic features and their short-term interval change may be predictive of underlying histologic diagnosis as well as long-term survival in idiopathic pulmonary fibrosis (IPF) presenting without honeycombing versus nonspecific interstitial pneumonia (NSIP). Materials and Methods: Forty biopsy-confirmed IPF and 20 biopsy-confirmed NSIP patients with available high-resolution chest computed tomography 4 to 24 months apart were studied. CALIPER software was used for the automated characterization and quantification of radiologic findings. Results: IPF subjects were older (66 vs. 48; P<0.0001) with lower diffusion capacity for carbon monoxide and higher volumes of baseline reticulation (193 vs. 83 mL; P<0.0001). Over the interval period, compared with NSIP, IPF patients experienced greater functional decline (forced vital capacity, −6.3% vs. −1.7%; P=0.02) and radiologic progression, as noted by greater increase in reticulation volume (24 vs. 1.74 mL; P=0.048), and decrease in normal (−220 vs. −37.7 mL; P=0.045) and total lung volumes (−198 vs. 58.1 mL; P=0.03). Older age, male gender, higher reticulation volumes at baseline, and greater interval decrease in normal lung volumes were predictive of IPF. Both baseline and short-term changes in quantitative radiologic findings were predictive of mortality. Conclusions: Baseline quantitative radiologic findings and assessment of short-term disease progression may help characterize underlying IPF versus NSIP in those with difficult to differentiate clinicoradiologic presentations. Our study supports the possible utility of assessing serial quantifiable high-resolution chest computed tomographic findings for disease differentiation in these 2 entities.

Abstract Acute eosinophilic pneumonia (AEP) is an uncommon acute respiratory illness of varying severity that includes presentation as acute respiratory distress syndrome with fatal outcome. AEP may be idiopathic, but identifiable causes include smoking and other inhalational exposures, medications, and infections. The pathogenesis of AEP is poorly understood but likely varies depending on the underlying cause. Airway epithelial injury, endothelial injury, and release of IL-33 are early events that subsequently promote eosinophil recruitment to the lung; eosinophilic infiltration and degranulation appear to mediate subsequent lung inflammation and associated clinical manifestations. Crucial for the diagnosis are the demonstration of pulmonary eosinophilia in the BAL fluid and the exclusion of other disease processes that can present with acute pulmonary infiltrates. Although peripheral blood eosinophilia at initial presentation may be a clue in suggesting the diagnosis of AEP, it may be absent or delayed, especially in smoking-related AEP. Optimal management of AEP depends on the recognition and elimination of the underlying cause when identifiable. The cessation of the exposure to the inciting agent (e.g., smoking), and glucocorticoids represent the mainstay of treating AEP of noninfectious origin. If AEP is recognized and treated in a timely manner, the prognosis is generally excellent, with prompt and complete clinical recovery, even in those patients manifesting acute respiratory failure.