In patients with cutaneous LCV, IgA is the most common immunoglobulin found by DIF. IgA deposition, but not IgM or IgG, is predictive of associated renal and gastrointestinal organ involvement by vasculitis. No association between the type of immunoglobulin and preexisting infection or malignancy was found. DIF results add information that is clinically relevant to the diagnosis and management of LCV.
Objective The aim was to gain a better understanding of the prevalence, organ involvement, clinical characteristics and long-term outcomes of medical and surgical treatments of IgG4-related disease (IgG4-RD). Methods Query of the Cleveland Clinic pathology database for IgG4 plasma cell staining between 2007 and 2017 yielded 1481 results, of which 57 cases were identified as highly likely ( n = 28; 49%) or probable ( n = 29; 51%) IgG4-RD by histopathological criteria and IgG4 serum concentrations. Patient demographics, type of treatment and outcomes were retrieved from medical records. Patients were designated as being in remission if indicated in the chart and/or symptom- and objective finding-free for >6 months, relapsed if symptoms/findings recurred after remission, active if no remission was achieved during follow-up, and as unable to determine if the duration of follow-up was <60 days or they were lost to follow-up. Results Of all patients who met the IgG4 staining criteria ( n = 119), half ( n = 57) satisfied the IgG4-RD histopathological criteria; 63% were males, age 57.9 ± 14.8 years. The average follow-up was 2.7 ± 2.2 years. The pancreas was the most affected organ in our cohort (26.4%). Almost half of the patients (45.6%; n = 26) were managed surgically, 21.1% ( n = 12) medically, and 24.6% ( n = 14) received both types of treatment. Medical treatment included prednisone (45.6%), MTX (5.3%), AZA (7%) and rituximab (8.8%). Remission was achieved by 77% of patients receiving surgical, 67% receiving medical and 72% receiving both treatments. Conclusion A histological diagnosis of IgG4-RD could be made in half of the patients who had IgG4 + plasma cells ≥10/high-power field or IgG4 + /IgG + ratio >40%. In our cohort, surgical treatment compared with medical treatment had a higher proportion of remission according to our outcome classification.
Background: Hypertrophic obstructive cardiomyopathy (HOCM) is known to be associated with supraventricular and ventricular arrhythmias. The burden of such arrhythmias and its effect on in-hospital outcomes in patients hospitalized with HOCM is not clear. Objectives: Our aim was to identify the burden of arrhythmia and its effect on in-hospital mortality in patients hospitalized with HOCM in recent years (2008 -2014) using National Inpatient Sample (NIS). Methods: We identified patients who were hospitalized with primary diagnosis of HOCM using International Classification of Diseases codes -9th edition (ICD-9) code 425.11. We identified presence of arrhythmias in this group using appropriate ICD-9 codes. We used multivariate binary logistic regression and multivariate linear regression to identify predictors associated with in-hospital mortality, length of stay (LOS), and total hospital charges respectively. Results: We identified 8534 patients discharged with diagnosis of HOCM. A total of 2880 (33.7%) patients had concomitant diagnosis of any arrhythmia. Among patients with HOCM, 235 (2.8%) patients had ventricular fibrillation (VF), 2025 (23.7%) had atrial fibrillation (AF), 324 (3.8%) had atrial flutter (AFL), 347 (4.1%) had atrioventricular nodal blocks, and 268 (3.1%) had premature beats. The in-hospital mortality in arrhythmia group was 2.6% compared to 1.5% in non-arrhythmia group. We found that only VF had significant effect on in-hospital mortality with these patients having adjusted odds ratio (AOR) of 19.41 (95% CI = 10.39 -36.25, P < .001) when compared to patients without VF. Median length of stay in arrhythmia group was 5 days compared to 3 days in non-arrhythmia group. Arrhythmias predicting increased LOS included VF (average of 3.63 days longer), AF (average of 0.61 days longer), and AFL (average of 6.95 days longer). Mean total charges of arrhythmia group were $122,503 compared to $76,435 in non-arrhythmia group. Arrhythmias predicting increased total hospitalization charges included VF (increased by an average of $70,572), and AFL (increased by an average of $131,193). Conclusion: About 1/3rd of patients admitted with HOCM had a concomitant diagnosis of arrhythmia. Atrial fibrillation was the most common arrhythmia. Presence of VF was a significant predictor of in-hospital mortality. Presence of VF, AF, AFL increased the LOS, while VF and AFL were associated with increased total hospital charges in patients hospitalized with HOCM.
Objectives To evaluate the patterns, severity and prognostic parameters of ILD in AJS. Methods We identified 51 anti-Jo-1 patients with ILD between 2003 and 2012. Clinical and laboratory data were obtained along with PFTs and pattern/scoring of thoracic HRCT scans based on Ooi scoring system (inflammation and fibrosis indices) (1). Progression of ILD was defined as follows: a decrease in DLCO and/or FVC (first to last reported) by >15%, an increase in fibrosis index or total HRCT score of ≥2, and death from respiratory failure. Results Out of 51 patients; 20 were males and 15 had dermatomyositis. Median age at onset was 47 years, mean follow up duration was 6.6 years; 20% had ILD prior to AJS diagnosis. Arthritis, mechanic's hands, Raynaud phenomenon and fever were reported in 88%, 37%, 31%, and 23% respectively. Four patients had cancer: lung (1), prostate and gallbladder (1) and lymphoma (2). At diagnosis 3 patients had UIP, 13 (25%) had organizing pneumonia (3 with NSIP, 5 developed NSIP later); 36 (70%) had NSIP: 22 fibrotic, 2 mixed and 12 cellular; 75% of cellular NSIP became fibrotic on subsequent imaging. Mean DLCO and FVC at diagnosis were 58.9% and 67% respectively. Median initial inflammation and fibrosis indices at diagnosis were 5.6, 1.16 and at last follow up 4.8, 2.1 respectively. Among 45 patients with available follow up data; 17 had worsening ILD (39%) and 5 (11%) died of respiratory failure. Lower DLCO at diagnosis was a predictor of disease progression [50 vs. 65% (p=0.0290)]. All UIP patients progressed during follow up. Only 7 patients had a late diagnosis of ILD (≥3 years after onset of AJS). Of those; 5 (70%) had a DLCO ≤50% compared to 25% in those with early diagnosis. SSA positivity was detected in 53%; lower initial (53% vs. 62%) and last (56% vs. 67%) DLCO as well as higher fibrosis score at last follow up [3.5 vs. 0.9 (p=0.089)] were observed in SSA negative group. These differences did not reach statistical significance. Interestingly; 6 of 7 patients who died were SSA negative. Nine patients who developed pulmonary hypertension had higher median disease duration (10 years) and CT score (12.5) and all of them had DLCO ≤50%. Esophageal disease other than dysphagia was detected in 43% [GERD (33%), abnormal manometry or gastric emptying (15%) and dilatation on chest CT (10%)]. Esophageal disease had a trend towards lower DLCO at diagnosis [53 vs. 62% (p=0.084)], higher fibrosis score [2.7 vs. 1.6 (p=0.089)] and higher %change of total CT score [1.1 vs. -0.7 (p=0.007)]. Conclusions Lower DLCO at diagnosis was associated with progression of ILD. The lower DLCO observed in late onset ILD may suggest silent disease and re-emphasize the rule of screening for ILD in AJS. In contrast with previous studies; SSA co-positivity was not associated with adverse outcome. Esophageal disease seemed to be prevalent in anti-Jo-1 patients with ILD and may predict worse fibrosis. The association of malignancy in AJS was not significant. References Ooi et al; Interstitial lung disease in systemi...
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