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Abstract Number: 1056

Descriptive Data Analysis of Patients with Anti Jo1 Syndrome (AJS) and Lung Involvement

Mohamed Alalwani1, Bassam Alhaddad2, Basem Zraik3, Christopher O'Rourke3, Ruchi Yadav3, Ali Askari1, Charles Malemud1 and Soumya Chatterjee4, 1Case Western Reserve University / University Hospitals Cleveland Medical Center, Cleveland, OH, 2MetroHealth Medical Center, Westlake, OH, 3Cleveland Clinic, Cleveland, OH, 4Cleveland Clinic, Richmond Heights, OH

Meeting: ACR Convergence 2020

Keywords: interstitial lung disease, Myositis

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Session Information

Date: Sunday, November 8, 2020

Title: Muscle Biology, Myositis & Myopathies Poster

Session Type: Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: To evaluate the patterns, severity and, prognostic parameters of interstitial lung disease (ILD) in Anti Jo1 Syndrome (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: Of 51 patients; 20 were male and 15 had a diagnosis of dermatomyositis (DM). The median age at onset was 47 years, mean follow-up duration was 6.6 years; 3/15 (20%) presented with ILD prior to AJS diagnosis. Arthritis, mechanic’s hands, Raynaud phenomenon and fever were reported in 88%, 37%, 31%, and 23% of DM patients, respectively. Four patients had cancer: lung (1), prostate and gallbladder (1) and lymphoma (2). At diagnosis 3 patients had upper respiratory pneumonia, 13 (25%) had organizing pneumonia (3 with non-specific interstitial pneumonia (NSIP), with 5 developing 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. The 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. A lower DLCO at diagnosis of DM was a predictor of disease progression [50 vs. 65% (p=0.0290)]. All upper respiratory pneumonia 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 a DLCO of 25% in those with early diagnosis. Sjögren’s syndrome antibody (SSA) positivity was detected in 53% of patients; 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; 6/7 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 trended 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)].

Conclusion: Lower DLCO at diagnosis was associated with progression of ILD. The lower DLCO observed in late onset ILD may suggest a silent disease and reemphasizes the role of screening for ILD in AJS. In contrast to previous studies; SSA co-positivity was not associated with an adverse outcome. Esophageal disease seemed to be prevalent in anti-Jo-1 patients with ILD and may predict worsening fibrosis. However, the association of malignancy with anti-Jo1 syndrome (AJS) was not significant.

ILD patterns and Mechanic’s hands in AJS

Table: Comparisons by Esophageal Disorder

Patterns of ILD in AJS at diagnosis


Disclosure: M. Alalwani, None; B. Alhaddad, None; B. Zraik, None; C. O'Rourke, None; R. Yadav, None; A. Askari, None; C. Malemud, None; S. Chatterjee, None.

To cite this abstract in AMA style:

Alalwani M, Alhaddad B, Zraik B, O'Rourke C, Yadav R, Askari A, Malemud C, Chatterjee S. Descriptive Data Analysis of Patients with Anti Jo1 Syndrome (AJS) and Lung Involvement [abstract]. Arthritis Rheumatol. 2020; 72 (suppl 10). https://acrabstracts.org/abstract/descriptive-data-analysis-of-patients-with-anti-jo1-syndrome-ajs-and-lung-involvement/. Accessed .
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