Dilemma in management of hemorrhagic myositis in dermatomyositis

Chandler, Julia M.; Kim, Yoo Jung; Bauer, Justin L; Wapnir, Irene

doi:10.1007/s00296-019-04501-7

Cited by 10 publications

(12 citation statements)

References 17 publications

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“…According to previous reports, the use of anticoagulation treatment has also been speculated as a probable provoking factor of hemorrhagic myositis in patients with DM ( 9 , 11 , 18 ). Of note, more than half of patients were receiving anti-thrombotic therapy when hemorrhagic events occurred, probably meant to prevent deep venous thrombosis.…”

Section: Discussionmentioning

confidence: 99%

Spontaneous Intramuscular Hemorrhage in Anti-MDA5 Positive Dermatomyositis: A Case Series and Literature Review

et al. 2022

Front. Med.

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ObjectiveSpontaneous intramuscular hemorrhage (SIH) is a rare but life-threatening complication associated with dermatomyositis (DM). This study reported a case series of SIH associated with DM. In addition, the characteristics and prognostic effects for this complication were analyzed based on literature review.MethodsWe reported seven cases of anti-melanoma differentiation-associated gene five positive dermatomyositis (MDA5+DM) complicated by SIH in our single-center cohort, and a comprehensive literature review was performed. Clinical characteristics, treatment, and outcome data of all eligible reported cases were summarized. Potential prognostic effects were identified by comparisons between the deceased and survivors.ResultsAmong cumulatively reported patients with DM patients and SIH, the overall mortality was 60.9% (14/23) (including our cases). Fourteen out of nineteen (73.7%) hemorrhagic events occurred within 6 months of disease onset. Anti-MDA5 antibody predominated in those myositis-specific antibodies available cases (8/10), although patients with positive anti-NXP2 and anti-Mi2 have also been documented. Iliopsoas (52.2%, 12/23) was the most frequently involved bleeding location. Bleeding in deep muscles was identified to be associated with poorer prognosis. The mortality of patients with DM and deep muscular hematoma (non-palpable) (80%, 12/15) was significantly higher than that of patients with only superficial muscular hematoma (palpable) (25%, 2/8) (p =0.023).ConclusionSpontaneous hematoma in non-palpable deep muscles probably leads to excess mortality in dermatomyositis, particularly for those with anti-MDA5 antibody, which often occurs within 6 months of disease onset. Clinicians should be vigilant to this rare but potentially fatal complication and carefully balance the risks and benefits of prophylactic anti-thrombotic treatment.

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Section: Discussionmentioning

confidence: 99%

Spontaneous Intramuscular Hemorrhage in Anti-MDA5 Positive Dermatomyositis: A Case Series and Literature Review

et al. 2022

Front. Med.

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“…This supports the distinction between non-DM SIH and DM-associated hematomas, as the latter result from diffuse bleeding from multiple small vessels, whereas the former originates from a single artery [ 3 ]. Despite normal anticoagulation studies, prophylactic anticoagulation and the use of antiplatelet therapy have been associated with an increased risk of bleeding in DM [ 2 ], which may have contributed to or worsened the rapid evolution of pectoral hematoma in our case, given his exposure to prophylactic anticoagulation during his previous admission.…”

Section: Discussionmentioning

confidence: 97%

“…However, in our review, 10 out of the 18 patients did not have their antibody results reported or mentioned in their case, including our patient, leaving only 44% (n=4) of patients with reported antibodies. Detected antibodies included: anti-Mi 2 [ 2 ], anti-KL-6 [ 1 ], anti-MDA5 [ 9 , 13 ], anti-RO52 plus MDA5 [ 3 ], and anti-RO52 plus NXP-2 [ 16 ]. These dual-positive anti-MDA5 and anti-Ro52 antibodies have also been correlated with worse prognosis and poor pulmonary outcomes, especially in the older population [ 16 ], and interestingly, patients with anti-KL6, anti-MDA5, and anti-RO52 positivity did not survive.…”

Section: Discussionmentioning

confidence: 99%

“…Spontaneous intramuscular hemorrhage (SIH) is an exceedingly rare but feared complication of DM, with most reported cases being fatal. While the mechanism and risk factors of this entity have not been elucidated, it is generally associated with acute-phase presentation and exposure to prophylactic anticoagulation, although idiopathic cases have also been reported [ 2 , 3 ]. Here, we present a case of SIH in a patient recently diagnosed with DM who was on deep vein thrombosis (DVT) prophylaxis.…”

Section: Introductionmentioning

confidence: 99%

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A Case of Hemorrhagic Myositis Associated With Prophylactic Heparin Use in Dermatomyositis

Bartl¹,

Casanovas²,

Loftis³

et al. 2023

Cureus

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Dermatomyositis (DM) is a rare systemic autoimmune disease that is associated with inflammation of the skin and muscles. It typically presents with weakness of the proximal muscles along with characteristic skin lesions such as Gottron's papules and heliotrope rash. One of the most feared complications of this disease is the appearance of spontaneous hemorrhagic myositis, as most reported cases are fatal. The mechanism or risk factors of this condition have not been elucidated; however, prophylactic anticoagulation has been correlated with it in previous case reports, although idiopathic hemorrhagic myositis may also be present. We present a case of spontaneous intramuscular hemorrhage (SIH) in a recently diagnosed DM patient. A 59-year-old Hispanic male with a medical history of recently diagnosed prostate cancer and DM presented to the emergency department (ED) due to worsening anemia. His previous hemoglobin (Hgb) was 9 g/dl, but repeated laboratory tests revealed a level of 6.5 g/dl and later 5.5 g/dl at the ED. On admission, the patient was afebrile, tachycardic, and normotensive, with no overt sign of gastrointestinal bleeding. The physical exam revealed an ecchymosis on the right medial aspect of the thigh, and a digital rectal exam was negative. Computer tomography (CT) of the abdomen and pelvis without contrast was ordered due to suspicion of a retroperitoneal hematoma, revealing an interval development of a right groin complex fluid collection of up to 6 cm, concerning a possible hematoma. The patient did not have any previous vascular procedures in the area but was exposed to deep vein thrombosis (DVT) prophylaxis during the previous admission. Vascular surgery was consulted, and the recommendation was made to proceed with conservative management. On the third day, the patient developed new-onset, left-sided pleuritic chest pain. Upon examination, significant swelling and tenderness were noted in his left pectoral region, which was not present on admission. A CT chest without contrast was ordered due to concerns of underlying hematomas, revealing bilateral thickening of the pectoralis muscles, more on the right side, with a fluid collection of 2.5 cm × 1.3 cm. In addition, there was thickening of the right lateral chest wall muscles in the posterior right trapezius or supraspinatus muscles, most likely from intramuscular hemorrhage. The patient was transferred to the step-down unit for close monitoring. Conservative management was continued with as-needed transfusions for three days until hemoglobin stabilized at 9.8 mg/dL. Once stable, the patient was resumed on steroids and immunosuppressive therapy with posterior resolution of the SIH. SIH has been reported in DM, particularly more prominent in those with anti-MDA-5 antibodies. A case series and literature review showed 60.9% mortality within six months in those presenting with SIH, with a poorer prognosis (80% mortality) in those with deep muscle bleeding versus superficial (25%). There is currently no consensus on the treatment approach, and...

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“…Pneumomediastinum is a rare but still known complication in the course of DM, while DM-related spontaneous intramuscular bleeding (SIH) is very rarely defined and is not very well known among rheumatologists. So far only case reports have been identified in the literature [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18]. The frequency and risk factors of DM-related SIH are unknown.…”

Section: Introductionmentioning

confidence: 99%

Evaluation of the frequency and risk factors of spontaneous intramuscular hemorrhage associated with dermatomyositis

Cansu,

Yildirim,

Uludoğan

et al. 2024

Rheumatol Int

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Dermatomyositis (DM) is an idiopathic inflammatory myositis (IIM) characterized by skin manifestations and muscle involvement. Spontaneous intramuscular hemorrhage (SIH) is a fatal complication that is very rare in the course of DM, but not well known to rheumatologists. Our aim was to determine the frequency and possible risk factors of DM-related SIH. A retrospective analysis was conducted on a cohort of DM patients who were observed in the rheumatology department of the university hospital between 1998 and January 2024. The clinical, laboratory, radiological data of the patients and the treatments they received during the follow-up were analyzed. To determine possible risk factors for the development of SIH in the course of DM, our patients with DM were analyzed together with other rare SIH cases in the literature. The study included 42 of our DM patients. 32 of the patients (76.2%) were female. The median age of the patients was 53 (24–82) years, the median age of DM diagnosis of the patients was 47 (18–75) years, and the median duration of DM of the patients was 36 (2-276) months. 7.1% of patients had dysphagia, and 16.7% had intertitial lung disease (ILD). 5 (11.9%) patients were diagnosed with malignancy. The incidence rate of SIH development in our DM cohort was 0.238/100 patient years (95% CI 0.006–1.256). We tried to identify independent risk factors for SIH development by comparing our 41 DM patients without SIH with the data of patients with 23 DM-related SIH collected from the literature by adding our 1 patient (24 pts). Male sex (OR 4.97, 95% CI 1.66–14.92, p = 0.003), ILD presence (OR 9.71, 95% CI 2.99–31.47, p < 0.001), anti-MDA5 positivity (OR 16.0, 95% CI 1.60-159.3, p = 0.006), anti-Ro52 positivity (OR 11.6, 95% CI 2.93–46.34, p < 0.001), heparin use (OR 4.42, 95% CI 2.68–7.24, p < 0.001), intravenous immunoglobulin (IVIG) use (OR 11.7, 95% CI 2.26–60.54, p < 0.001), and steroid dose (OR 1.03, 95% CI 1.00-1.05, p = 0.005) were identified as risk factors for the development of SIH in the univariate analysis. The death rate due to hemorrhage was 50%. No single risk factor was found to be associated with death. As a result, SIH may occasionally arise in patients with DM. Rheumatologists should be aware that patients with dysphagia and/or ILD, who are on heparin, getting high doses of steroids, and test positive for anti-MDA5 and/or anti-Ro52 antibodies may develop SIH in the early stages of DM.

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Dilemma in management of hemorrhagic myositis in dermatomyositis

Cited by 10 publications

References 17 publications

Spontaneous Intramuscular Hemorrhage in Anti-MDA5 Positive Dermatomyositis: A Case Series and Literature Review

Spontaneous Intramuscular Hemorrhage in Anti-MDA5 Positive Dermatomyositis: A Case Series and Literature Review

A Case of Hemorrhagic Myositis Associated With Prophylactic Heparin Use in Dermatomyositis

Evaluation of the frequency and risk factors of spontaneous intramuscular hemorrhage associated with dermatomyositis

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