Peripheral Gangrene as the Initial Presentation of Systemic Lupus Erythematosus in Emergency Department

Alalawi, Zainab M; Almahroos, Fatemah; Albloushi, Basmah

doi:10.7759/cureus.6667

Cited by 8 publications

(11 citation statements)

References 10 publications

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“…Although 8 of our patients were diagnosed with SLE while an adolescent [age 12-20 years], long duration of lupus (>/ = 4 years) is a known risk factor for the development of gangrene. 6,9 With a mean duration of SLE before the onset of the gangrene of 18.5 SD = 11.5 years, the development of gangrene in our cohort was consistent with previous reported studies, with most patients having the first episode of gangrene at a late stage of the disease.…”

Section: Discussionsupporting

confidence: 90%

“…Peripheral digital gangrene is a rare manifestation of SLE, being particularly unusual as a first presentation of the disease. 3,6,9 Although there are no recent reports about the prevalence in SLE patients, three previous studies reported a prevalence of gangrene in SLE patients between 0.6% and 5.8%. 1,6,10 In our cohort the prevalence was about 1%.…”

Section: Discussionmentioning

confidence: 99%

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A review of the development of gangrene in patients with systemic lupus erythematosus – A 44-year follow-up study

Rua

Isenberg

2023

Lupus

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Objectives This review addresses the question of what happens long-term to those systemic lupus erythematosus (SLE) patients who develop gangrene. It also seeks to find common clinical and serological features, risk factors and triggers and how best to manage this challenging complication. Methods We reviewed 850 patients with SLE attending a UK tertiary referral center, followed up over 44 years, assessing their demographics, clinical and serological features, treatment in the acute phase, their long-term outcome and long-term management. Results Ten out of 850 patients (1.2%) developed gangrene; the mean age of onset was 17 years (range 12–26 years) Eight out of 10 patients had a single episode of gangrene. One of the other two was not willing to have anticoagulation. The first episode of gangrene ranged from presentation to 32 years after SLE onset, mean duration of SLE at the onset of the gangrene was 18.5 years SD 11.5 years. Anti-phospholipid (PL) antibodies were over-represented in the patients with gangrene. All had active SLE at the time the gangrene developed. All patients were treated with intravenous (IV) iloprost infusions, and the antiphospholipid-antibody positive patients were anti-coagulated, most staying on long term anticoagulation. Underlying possible triggers were treated appropriately. Two patients who did not respond to the initial treatment needed further immunosuppression. All patients suffered digit loss. Conclusion Although rare, gangrene is a sinister, potentially late developing complication of SLE, it rarely recurs. It is associated with anti-phospholipid antibodies, active disease, and other possible triggers such as infection and cancer. Anticoagulation therapy, steroids and iloprost, and further immunosuppression may be needed to stop the evolution of gangrene.

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Section: Discussionsupporting

confidence: 90%

Section: Discussionmentioning

confidence: 99%

A review of the development of gangrene in patients with systemic lupus erythematosus – A 44-year follow-up study

Rua

Isenberg

2023

Lupus

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“…However, 3 of our patients concurrently received immunosuppressive agents in the form of cyclophosphamide or mycophenolate mofetil for their systemic manifestations. This was in stark contrast to all previous reports, wherein the protocol was to initiate cyclophosphamide along with steroids as first line agent [4,11,13]. Other agents of choice in previous reports included rituximab and IV prostanoids in refractory cases [1,12].…”

Section: Discussionmentioning

confidence: 74%

“…The actual prevalence of digital gangrene in MCTD remains unknown. Therefore, as new data is emerging from the developing world, it appears that the clinical characteristics and distribution of digital gangrene are different from western cohorts with MCTD and SLE being the commonly associated disorders rather than systemic sclerosis [10][11][12]. A cohort of lupus patients from India estimated the prevalence of digital gangrene in SLE as 5% [13] notably higher than previous data ranging from 0.6 to 1.2% suggesting that digital gangrene maybe commoner in South Asian region.…”

Section: Discussionmentioning

confidence: 92%

Clinical characteristics and outcomes of digital gangrene in connective tissue disorders: a longitudinal single-centre experience from Jodhpur, India

et al. 2022

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Epidemiology, clinical presentation, and outcomes for digital gangrene in connective tissue disorders (CTD) remain underreported from tropical countries like India. In this series, we aimed to explore the clinical profile and outcomes of patients who presented with digital gangrene and a diagnosis of CTD. Hospital-based longitudinal observational study. Patients with digital gangrene and underlying diagnosis of CTD presenting to our tertiary-care centre in Jodhpur, India between1 st January 2018 and 31 st June 2021 were included. Clinical outcomes including mortality, limb outcomes, functional status and other systemic involvement were assessed. Of the 312 patients registered in the rheumatology clinic during this period, 22 (7%) patients were found to satisfy the inclusion criteria. Mean age was 46 years and 90% were females. The most common underlying diagnosis was Mixed connective tissue disorder (MCTD). Digital gangrene was the presenting symptom in 13 (60%) patients. Half of the patients received only corticosteroids as immunosuppression. Two died due to systemic complications. Complete resolution occurred in 17 (85%), autoamputation in 3, and infection requiring surgical drainage in one patient. All surviving patients reported good functional limb outcome on 6 months follow-up. MCTD is an important cause of digital gangrene in rheumatology practice. In patients presenting with digital gangrene, an active search for an underlying CTD is imperative, as this could result in timely initiation of appropriate limb-saving therapy. Corticosteroids alone with rapid tapering may be an appropriate option to consider in the initial management of digital gangrene in CTD. Key Points• Mixed connective tissue disorder is an important cause of digital gangrene in rheumatology practice in western India.• In patients presenting with digital gangrene, an active search for an underlying connective tissue disorder is imperative, as this could result in timely initiation of appropriate therapy and can prove limb saving. • Corticosteroids alone with rapid tapering may be an appropriate option to consider in the initial management of digital gangrene in connective tissue disorders.

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“…8 However, some cases of digital gangrene as a primary event in SLE have been reported. 3,6,[9][10][11] The majority of cases reported in the literature have proved that digital gangrene occurs in young SLE patients and that the occurrence of this complication in older adult patients is extremely rare. 2,6,9,10 To the best of the authors' knowledge, only three cases of digital gangrene have been reported in patients with SLE at an older age (after 50 years old).…”

Section: Discussionmentioning

confidence: 99%

Case Report: Extensive digital gangrene as a primary manifestation of late-onset systemic lupus erythematosus

et al. 2022

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Background: Digital gangrene is a rare but serious complication of systemic lupus erythematosus (SLE). It occurs usually in middle-aged patients with longer disease duration. Case: Herein we report the case of a 56-year-old man (with no history suggestive of Raynaud’s phenomenon, diabetes mellitus, smoking, trauma, infection, or chemical exposure), who presented with SLE and digital gangrene was among the first signs. He presented with a one-month history of joint pain, hair loss, photosensitivity, mouth ulcers, malar rash, dyspnea, and digital pain. Physical examination revealed painful and diffuse erythematous skin lesions in the extremities and back, as well as cyanosis in the fingers. We noted lymphocytopenia (600 cells/mm3), and an elevated C-reactive protein (15.1 mg/l) on laboratory tests. Immunological tests were positive for antinuclear antibodies (ANA) with Title 1:400. Pulmonary computed tomography revealed pulmonary fibrosis, and pulmonary function tests revealed the restrictive pulmonary disease. Diagnosis of SLE with lung involvement was retained. The immunological assessment in search of elements in favor of a vascular origin of the patient's skin lesions was negative. Treatment was initiated with 200 mg/day hydroxychloroquine. For dermal and pulmonary involvement, intravenous (IV) pulse therapy was used with methylprednisolone (1,000 mg/d for three consecutive days monthly) and cyclophosphamide (1 g/month). Calcium blocking agents were also prescribed. However, the lesions did not improve. The patient was given two infusions of rituximab (1 g) at a 14-day interval with a marked improvement of the majority of vasculitis lesions, and a partial improvement of dyspnea. Conclusions: Digital gangrene is a rare complication of late-onset SLE, especially as a primary manifestation.

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Peripheral Gangrene as the Initial Presentation of Systemic Lupus Erythematosus in Emergency Department

Cited by 8 publications

References 10 publications

A review of the development of gangrene in patients with systemic lupus erythematosus – A 44-year follow-up study

A review of the development of gangrene in patients with systemic lupus erythematosus – A 44-year follow-up study

Clinical characteristics and outcomes of digital gangrene in connective tissue disorders: a longitudinal single-centre experience from Jodhpur, India

Case Report: Extensive digital gangrene as a primary manifestation of late-onset systemic lupus erythematosus

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