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Abstract Number: 395

Systemic Lupus Erythematosus (SLE) with Inflammatory Myositis

Nicole Bitencourt, Elizabeth (Blair) Solow and Bonnie L. Bermas, Division of Rheumatic Diseases, UT Southwestern Medical Center, Dallas, TX

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: inflammatory myositis and myositis, Lupus, SLE

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Session Information

Date: Sunday, October 21, 2018

Title: Muscle Biology, Myositis and Myopathies Poster I: Clinical Features and Disease Course

Session Type: ACR Poster Session A

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

Background/Purpose:

The goal of this study was to identify specific clinical features of patients with SLE who have inflammatory myositis.

Methods:

A retrospective chart review was performed of patients seen in the rheumatology clinic at a single center safety net hospital over a period of 4 months and who had an encounter diagnosis of SLE by ICD-10 code [M32.x]. Diagnosis of SLE was confirmed by one author (NB) using 2015 ACR/SLICC criteria. Cases of inflammatory myositis were defined by either muscle biopsy or with CK elevation/muscle weakness with or without confirming muscle MRI consistent with myositis. Data on patient demographics, clinical and laboratory features were extracted. Chi-square was used to analyze clinical features.

Results:

Of 354 patients with an encounter diagnosis of SLE, 30 (8.5%) were diagnosed with myositis. Most (93%) were women; 56% were African American and 43% Hispanic. Half were diagnosed with SLE and myositis simultaneously, 30% developed myositis after SLE diagnosis (mean 5.5 years later) and 20% were diagnosed with myositis prior to SLE (mean 10.9 years earlier). Patients fell into two clinical categories, those with features of systemic sclerosis (60%) and those without (40%). Among those with features of systemic sclerosis, 9 of 18 met the 2013 ACR/EULAR classification criteria for systemic sclerosis, while 9 of 18 did not but scored 5 or more criteria points.

SLE patients with co-existing features of systemic sclerosis were more likely to have Raynaud’s phenomenon and interstitial lung disease  (p<0.05) (Table 1 and 2). Muscle biopsy was performed in 16 patients, with 12 biopsies among 11 patients available for review (one patient underwent two biopsies over 3 years). Non-specific inflammatory changes were the most commonly observed muscle biopsy feature, followed by tubuloreticular inclusions by electron microscopy (Table 3). Autoimmune necrotizing myopathy was described in 4 of 12 muscle biopsies, three of whom lacked features of systemic sclerosis.

 

Conclusion:

In our cohort of SLE patients at a large safety net hospital, the diagnosis of co-existing myositis was seen in 8.5% of patients, most of whom had overlap features of systemic sclerosis.

 

Table 1. Clinical features of patients with SLE and inflammatory myositis

Clinical Features, n (%)

SLE without Scleroderma Features

(n=12)

SLE with Scleroderma Features

(n=18)

All Patients

(n=30)

Arthralgias

10 (83%)

17 (94%)

27 (90%)

Inflammatory arthritis

10 (83%)

16 (89%)

26 (87%)

Leukopenia or lymphopenia

11 (92%)

15 (83%)

26 (87%)

Myalgia

9 (75%)

16 (89%)

25 (83%)

Raynaud’s

6 (50%)

18 (100%)*

24 (80%)

Thrombotic event or significant pregnancy morbidity

8 (67%)

10 (56%)

18 (60%)

Interstitial lung disease

2 (17%)

15 (83%)*

17 (57%)

Serositis

9 (75%)

8 (44%)

17 (57%)

Alopecia

7 (58%)

10 (56%)

17 (57%)

Oral/nasal ulcers

7 (58%)

8 (44%)

15 (50%)

Gottron’s or heliotrope rash

6 (50%)

4 (22%)

10 (33%)

Nephritis

4 (33%)

6 (33%)

10 (33%)

Discoid lupus

3 (25%)

5 (28%)

8 (27%)

Myocarditis

1 (8%)

6 (33%)

7 (23%)

* p<0.05 by chi-square

 

Table 2. Serological features of patients with SLE and inflammatory myositis

Serologic Marker, n (%)

SLE without Scleroderma Features

SLE with Scleroderma Features

All Patients

RNP

7/12 (58%)

16/18 (89%)

23/30 (77%)

Low C3 or C4

7/12 (58%)

14/18 (78%)

21/30 (70%)

Any APLS Ab

5/10 (50%)

10/14 (71%)

15/24 (63%)

Smith

6/12 (50%)

12/17 (71%)

18/29 (62%)

RF or CCP

5/10 (50%)

12/16 (75%)

17/26 (65%)

SSA

5/12 (42%)

11/18 (61%)

16/30 (53%)

dsDNA

5/12 (42%)

10/18 (56%)

15/30 (50%)

p-ANCA

2/8 (25%)

4/9 (44%)

6/17 (35%)

U2snRNP

0/6 (0%)

5/10 (50%)

5/16 (31%)

Ku

0/6 (0%)

2/10 (20%)

2/16 (13%)

Jo 1

0/6 (0%)

2/14 (14%)

2/20 (10%)

Mi2

1/6 (17%)

1/12 (8%)

2/18 (11%)

SRP

1/6 (17%)

0/10 (0%)

1/16 (6%)

* p<0.05 by chi-square.

APLS: antiphospholipid antibody, RF: rheumatoid factor, CCP: anti-citrulinnated peptie

Table 3. Muscle biopsy features of patients with SLE and inflammatory myositis

Muscle biopsy features

Patients without scleroderma features

Patients with scleroderma features

 

Pt 1 Pt 2 Pt 3 Pt 4 Pt 5 # 1 Pt 5 #2 Pt 6 Pt 7 Pt 8 Pt 9 Pt 10 Pt 11

 

Autoimmune Necrotizing Myositis   Necrotic fibers with sparse mononuclear cell infiltrate X X X X

 

Myophagocytosis X X X X X X

 

Polymyositis- related Changes   Endomysium inflammatory cells X X X X X X

 

Increase in endomyseal connective tissue X X X   X

 

Nonspecific Inflammatory Changes Variable fiber size X X X X X X X X X

 

Necrotic/regenerating muscle fibers X X X X X X X X X X X

 

MHC class 1 sarcolemmal upregulation (if stained) X X X X

 

Dermatomyositis-related Changes   Perivascular inflammatory cells X X X X X X

 

Perifascicular atrophy X X X   X X

 

Perimyseal inflammation   X X X X   X X

 

Tubuloreticular inclusion by electron microscopy X X X X X X X X

 

Neuropathic Changes Denervation atrophy X X X X

 

Note: patient 5 had two biopsies performed. “X” denotes presence of biopsy finding.    

 

 


Disclosure: N. Bitencourt, None; E. Solow, None; B. L. Bermas, UptoDate, 7.

To cite this abstract in AMA style:

Bitencourt N, Solow E, Bermas BL. Systemic Lupus Erythematosus (SLE) with Inflammatory Myositis [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/systemic-lupus-erythematosus-sle-with-inflammatory-myositis/. Accessed .
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