Digital ischemia as the initial presentation of catastrophic antiphospholipid syndrome.

Catastrophic antiphospholipid syndrome is an infrequent disease in children, but of major relevance because of its high morbidity and mortality. We report the case of a child with digital ischaemia in whom, after aetiological screening, the diagnosis of catastrophic antiphospholipid syndrome was made.

Isquemia digital como forma de presentación de síndrome antifosfolípido catastrófico pediátrico / Digital Ischemia as the Initial Presentation of Catastrophic Antiphospholipid Syndrome

El síndrome antifosfolípido catastrófico es una entidad infrecuente en Pediatría, pero con importante relevancia dada la elevada morbimortalidad. Se expone el caso de un niño con isquemia digital en el que, tras realizar despistaje etiológico de diferentes entidades infecciosas e inflamatorias, se llegó al diagnóstico de síndrome antifosfolípido catastrófico primario.(AU)

Catastrophic antiphospholipid syndrome is an infrequent disease in children, but of major relevance because of its high morbidity and mortality. We report the case of a child with digital ischaemia in whom, after aetiological screening, the diagnosis of catastrophic antiphospholipid syndrome was made.(AU)

Digital pitting scars are associated with a severe disease course and death in systemic sclerosis: a study from the EUSTAR cohort.

OBJECTIVE: Digital pitting scars (DPS) are frequent, but little studied in SSc to date. METHODS: An analysis of SSc patients enrolled in the EUSTAR database. Primary objectives were to (i) examine DPS prevalence; (ii) examine whether DPS are associated with digital ulcers (DUs) and active digital ischaemia (DUs or gangrene); and (iii) describe other associations with DPS including internal organ complications. Secondary objectives were whether DPS are associated with (i) functional impairment; (ii) structural microvascular disease; and (iii) mortality. Descriptive statistics and parametric/non-parametric tests were used. Binary logistic regression was used to examine the association between DPS and DUs, active digital ischaemia and mortality. RESULTS: A total of 9671 patients were included with reported DPS at any time point (n = 4924) or 'never' DPS (n = 4747). The majority (86.9%) were female and mean age was 55.7 years. DPS were associated with longer disease and Raynaud's duration (both P ≤ 0.001). DPS were associated with interstitial lung disease, pulmonary hypertension, conduction blocks, telangiectases, calcinosis (all P ≤ 0.001) and joint synovitis (P = 0.021). Patients were more likely to have more severe capillaroscopic abnormality and greater hand functional impairment. Multivariable logistic regression analyses showed that DPS were associated (odds ratio) with DUs: 22.03 (19.51-24.87), active digital ischaemia: 6.30 (5.34-7.42) and death: 1.86 (1.48-2.36). CONCLUSION: DPS are associated with a severe disease course including death. The impact of DPS on hand function and ischaemia is significant. The presence of DPS should alert the clinician to a poor prognosis and need to optimize the therapeutic strategy.

A Review of 1073 Cases of Wide-Awake-Local-Anaesthesia-No-Tourniquet (WALANT) in Finger and Hand Surgeries in an Urban Hospital in Malaysia.

Background The Wide-Awake-Local-Anaesthesia-No-Tourniquet (WALANT) technique achieves an almost bloodless field for clear visualization during surgeries. WALANT utilizes lidocaine and epinephrine for anesthesia and hemostasis, respectively, without the usage of sedation and tourniquet. This avoids the potential side effects of tourniquet-related pain and sedation-related complications. However, acceptance is still low due to concerns regarding the safety of epinephrine injection in the finger. There is a persistent belief that epinephrine can cause digital ischemia. Purpose To evaluate retrospectively possible complications of hand surgeries performed using the WALANT technique. Methods All finger and hand procedures performed under the WALANT technique from June 2016 to May 2021 in an urban tertiary hospital were studied retrospectively. Results There were a total of 1073 cases, of which 694 were females and 379 were males. The mean age was 55 years. Finger surgeries (e.g., trigger finger release, excision of finger lesions, removal of implants) consisted of 707 cases; and the rest (366 cases) were hand surgeries (e.g., carpal tunnel release, excision of hand lesions, removal of implants). In all cases reviewed, there were no instances of circulatory compromise. There were also no circumstances where usage of reversal with phentolamine is recorded. Conclusion We believe that performing finger and hand surgeries using the WALANT technique is safe and beneficial. The usage of WALANT in hand surgeries avoids tourniquet pain. However, WALANT should be used with caution in those with vascular insufficiency or disease.

Ischemic toe after surgery for lesser toe deformities: An algorithm.

Ischemia after correction of lesser toe deformities is usually due to temporary vasospasm and can rarely cause gangrene. The published literature on dealing with the issue and been reviewed and used to suggest an algorithm for a logical step by step approach to a pale or white toe when encountered in the postoperative period.

The perils of dressings: a cautionary tale of ischaemia.

A cohort of patients presented to Queen Victoria Hospital, UK, with iatrogenic toe ischaemia following application of a different, newly available post-procedure dressing with different properties to those usually used. This resulted in ischaemia with extensive skin and soft tissue damage, requiring debridement surgery and, in some cases, skin grafting. We aim to highlight the risk of morbidity from dressing application to the digits. This is a key learning skill for anyone who may either perform dressings or evaluate dressings on digits in the community and across multiple specialties in hospital. This article follows a thorough root cause analysis and addresses other possible causes of an acutely painful erythematous toe post-Zadek's procedure.

Digital ischaemia of the upper limbs in middle age: consider arterial thoracic outlet syndrome until proven otherwise!

OBJECTIVES: Patients presenting with digital upper limb ischaemia are occasionally referred to rheumatology services to rule out vasculitis. We aimed to present two cases of delayed diagnosis of arterial thoracic outlet syndrome (aTOS) in middle-aged patients presenting with digital ischaemia in order to raise awareness of this important pathology that requires timely surgical intervention. METHODS: Two cases of progressive ischaemia of the right upper extremity caused by primarily undiagnosed compression of the subclavian artery by an accessory cervical rib are presented. The case notes, radiological images, intra-operative and postoperative findings for both patients were reviewed. Patients were followed up after ≥6 months to assess prognosis. RESULTS: Both patients had a working diagnosis of Buerger's disease and had been treated with prostaglandin infusions before establishment of the diagnosis of arterial thoracic outlet syndrome. Both patients were heavy smokers, and one patient had bilateral symptoms and a history of axial SpA and positive HLA-B27. Late presentation in one patient led to the loss of three fingers and the need for plastic reconstructive surgery after cervical rib resection and revascularization. In the other patient, surgical thrombectomy of the upper limb arteries along with resection of a cervical rib and repair of the subclavian artery with an interposition graft were necessitated to heal digital ulcers successfully. CONCLUSION: A high index of suspicion of aTOS should be maintained in middle-aged patients presenting with digital or upper limb ischaemia even in the presence bilateral symptoms or relevant risk factors of other diagnoses, such as smoking or a positive rheumatological history.

Anti-phospholipid syndrome leading to digital ischaemia and rare organ complications in systemic sclerosis and related disorders.

Antiphospholipid syndrome (APS) is an acquired, autoimmune thrombophilia that can occur as a primary disorder (with no associated disease) or secondary to infection, medication usage and autoimmune rheumatic diseases (ARDs). The association between APS and systemic lupus erythematosus (SLE) is well established, and practicing rheumatologists check for APS antibodies in the routine assessment of SLE, particularly if clinical features such as thrombotic events or pregnancy loss are present. APS secondary to systemic sclerosis (SSc)-related disorders is less widely recognised and easily overlooked. We describe 5 cases that highlight the varied breadth of clinical manifestations of APS in the context of SSc and related disorders. These cases range from uncomplicated Raynaud's phenomenon, digital ulceration/necrosis, critical digital ischaemia/gangrene and rare internal organ complications of APS in SSc-spectrum disorders. To our knowledge, our cases include the first reported case of secondary APS contributing to digital necrosis in the context of RACAND syndrome (Raynaud's phenomenon, anti-centromere antibodies and necrosis of the digits) and the first reported case of secondary APS in SSc causing posterior reversible encephalopathy syndrome (PRES). The case series is accompanied by a comprehensive review of the literature relevant to each case. Rheumatologists should be alert to the possibility of APS in SSc-spectrum disorders and should routinely check APS antibodies in all patients at diagnosis, and again later in the disease course if new features emerge that could indicate the presence of thrombotic events or other recognised APS manifestations. Key points • APS should be considered in all patients with digital ischaemic symptoms. • APS may be an important driver of SSc-related digital ulceration/necrosis. • Identification of SSc-associated APS opens up new therapeutic options for acute management and secondary prevention.

Digital Ischemia as the Initial Presentation of Catastrophic Antiphospholipid Syndrome. / Isquemia digital como forma de presentación de síndrome antifosfolípido catastrófico pediátrico.

Catastrophic antiphospholipid syndrome is an infrequent disease in children, but of major relevance because of its high morbidity and mortality. We report the case of a child with digital ischaemia in whom, after aetiological screening, the diagnosis of catastrophic antiphospholipid syndrome was made.

Adrenaline Auto-injector injuries to digits; a systematic review and recommendations for emergency management.

AIMS: Accidental injury to digits with Adrenaline Auto-injectors (AAIs) is becoming increasingly common. Digital AAI injury causes painful ischaemia that can lead to necrosis and patient anxiety. There is a lack of understanding amongst surgeons regarding how to manage these injuries. We aimed to determine an optimal treatment algorithm for their management. METHODS: We conducted a systematic review using the search engines MEDLINE, PubMed, EMBASE, CINAHL, BNI, AMED, Google Scholar. Search items included ("epinephrine OR adrenaline") AND ("Digit" OR "Finger" OR "Thumb") AND ("Injury" OR "Accidental"). RESULTS: A total of 49 articles were identified describing 111 cases. In 58 cases; 52% of cases were managed with phentolamine, 24% were managed with nitroglycerine and 7% were treated with warm soaks. The remaining 17% of cases were managed with a variety of alternative treatments. Mean recovery time following treatment with phentolamine infiltration was 33 min, whilst symptoms persisted for several hours in some cases with observation/warm soaks and nitroglycerine. Phentolamine was more effective when injected into the AAI puncture site (mean resolution time: 17 min) in comparison to injection as a digital block (74 min). CONCLUSION: Phentolamine is the most effective method of reversing symptoms and treating ischaemic digits when compared to alternative therapies. Symptoms resolved much quicker when phentolamine was infiltrated into the site of injury compared to being infiltrated as a digital block. We propose a treatment algorithm for management of these injuries. Hand surgeons should be aware of AAI injuries and be able to advise on their management.

Cutaneous Vasculitis and Digital Ischaemia Caused by Heterozygous Gain-of-Function Mutation in C3.

It is now increasingly recognized that some monogenic autoinflammatory diseases and immunodeficiencies cause vasculitis, although genetic causes of vasculitis are extremely rare. We describe a child of non-consanguineous parents who presented with cutaneous vasculitis, digital ischaemia and hypocomplementaemia. A heterozygous p.R1042G gain-of-function mutation (GOF) in the complement component C3 gene was identified as the cause, resulting in secondary C3 consumption and complete absence of alternative complement pathway activity, decreased classical complement activity, and low levels of serum C3 with normal C4 levels. The same heterozygous mutation and immunological defects were also identified in another symptomatic sibling and his father. C3 deficiency due GOF C3 mutations is thus now added to the growing list of monogenic causes of vasculitis and should always be considered in vasculitis patients found to have persistently low levels of C3 with normal C4.

The challenge of establishing treatment efficacy for cutaneous vascular manifestations of systemic sclerosis.

INTRODUCTION: The cutaneous vascular manifestations of systemic sclerosis (SSc) comprise Raynaud's phenomenon, cutaneous ulceration, telangiectasia formation and critical digital ischaemia; each of which are associated with significant disease-related morbidity. Despite the availability of multiple classes of vasodilator therapy, many of which have been the subject of RCTs, a limited number of pharmacological interventions are currently approved for the management of cutaneous vascular manifestations of SSc. Areas covered: A major challenge has been demonstrating treatment efficacy with examples of promising therapies yielding contrasting results in controlled trial settings. Differences between consensus best-practice guidelines, evidence-based recommendations and marketing approvals in different jurisdictions has resulted in geographic variation in clinical practice concerning the management of cutaneous vascular manifestations of SSc. Difficulty demonstrating treatment efficacy risks waning industry engagement for drug development programmes in this field. This article highlights the key challenges in establishing treatment efficacy and barriers that must be overcome to support successful clinical trial programmes across the spectrum of cutaneous vascular manifestations of SSc. Expert commentary: The paucity of approved treatments for cutaneous vascular manifestations of SSc relates as much to challenges in clinical trial design and the need for reliable clinical trial endpoints, as to lack of therapeutic options.

Reduced perfusion in systemic sclerosis digital ulcers (both fingertip and extensor) can be increased by topical application of glyceryl trinitrate.

OBJECTIVES: In patients with systemic sclerosis (SSc), fingertip digital ulcers (DUs) are believed to be ischaemic, and extensor surface DUs a result of mechanical factors/microtrauma. Our aim was to assess blood flow response to topical glyceryl trinitrate (GTN) compared to placebo in SSc DUs, looking for differences in pathophysiology between fingertip and extensor lesions. METHOD: This was a double-blind, randomised, crossover, placebo-controlled study. Sixteen (6 fingertip, 10 extensor) DUs were each studied twice (one day apart): once with GTN and once with placebo ointment. Perfusion at the DU centre ('DUCore') and periphery ('DUPeriphery'), as measured by laser Doppler imaging was performed before and immediately after ointment application, then every 10min, up to 90min post-application. We calculated the area under the response curve (AUC) and the ratio of peak perfusion to baseline, then compared these between GTN and placebo. RESULTS: Perfusion was lower in the DUCore compared to the DUPeriphery (ratio of 0.52). The microvessels of the DUCore were responsive to GTN, with an increase in perfusion, with a similar effect in both fingertip and extensor DUs. The AUC and peak/baseline perfusion difference in means (ratio, 95% confidence interval) between GTN and placebo at the DUCore were 1.2 (1.0-1.6) and 1.2 (1.0-1.5) respectively, and at the DUPeriphery were 1.1 (0.8-1.6) and 1.0 (0.9-1.2) respectively. CONCLUSION: DUs (both fingertip and extensor) were responsive to topical GTN, with an increase in perfusion to the ischaemic DU centre. If both fingertip and extensor DUs have a (potentially reversible) ischaemic aetiology, this has important treatment implications.

Raynaud's phenomenon.

Raynaud's phenomenon (RP) is a major cause of pain and disability in patients with autoimmune connective tissue diseases (CTDs), particularly systemic sclerosis (SSc). The clinician must perform a comprehensive clinical assessment in patients with RP to differentiate between primary (idiopathic) and secondary RP, in particular (for rheumatologists), secondary to an autoimmune CTD, as both the prognosis and treatment may differ significantly. Key investigations are nailfold capillaroscopy and testing for autoantibodies (in particular, those associated with SSc). Patients with RP and either abnormal nailfold capillaroscopy or an SSc-specific antibody (and especially with both) have a high risk of transitioning to an autoimmune CTD. Both nailfold capillaroscopy and autoantibody specificity may help the clinician in predicting organ-based complications. The management of CTD-associated RP requires a multifaceted approach to treatment, including patient education and conservative ('non-drug') measures. Patients with CTD-associated RP often require pharmacological treatment, which in the first instance is usually a calcium channel blocker, although other agents can be used. There is an increasing tendency to use phosphodiesterase type 5 inhibitors early in the treatment of CTD-associated RP. Oral therapies are commonly associated with side effects (often due to systemic vasodilation) that may result in failure of dose escalation and/or permanent discontinuation. Intravenous prostanoid therapy and surgery (e.g., botulinum toxin injection and digital sympathectomy) can be considered in severe RP. Patients with CTD-associated RP can develop a number of ischaemic digital complications (primarily ulcers and critical ischaemia), which may be associated with significant tissue loss. Future research is required to increase the understanding of the pathogenesis and natural history of RP (to drive therapeutic advances), and to explore/develop drug therapies, including those that target the mechanisms mediating cold-induced vasoconstriction, and locally acting therapies free of systemic side effects.

Raynaud's phenomenon and bilateral olecranon bursitis co-existing in a patient with chronic hepatitis B and D treated with pegylated interferon.

Pegylated interferon remains the first line treatment for patients with hepatitis D virus and more than one year therapy may be necessary. Interferon a has the most extensive clinical application and is used for the treatment of chronic hepatitis B and D virus as well as HCV infections. The attachment of polyethylene glycol to interferon increases its half-life. Treatment with peg interferon is associated with many troublesome and occasionally with serious or even life-threatening side effects. In this case report, we have described a patient with chronic hepatitis B and D, who developed Raynaud's phenomenon, ischaemic digital necrosis and bilateral olecranon bursitis during Pegylated interferon therapy. The patient underwent a very extensive workup in order to determine the underlying cause of his digital ischaemia and olecranon bursitis, which was finally determined to be secondary to the use of Pegylated interferon.

Digital ischaemia during cooling is independently related to nailfold capillaroscopic pattern in patients with Raynaud's phenomenon.

OBJECTIVE: The aim of the study was to assess the association between plethysmographically measured vasospasms during stepwise cooling and recovery, as an index for digital ischaemia, and nailfold capillaroscopic pattern (NCP) severity in patients with primary or secondary RP, including SSc. METHODS: In 381 consecutive patients with suspected RP without a history of digital ulcers, NCP (assessed by widefield videocapillaroscopy), fingertip photoelectric plethysmography during cooling and recovery and clinical characteristics were analysed. NCPs were graded as follows: normal, non-specific, early and active. The mean ischaemic time was defined as the mean time of perfusion loss during cooling and recovery of five fingers. RESULTS: In the patients with loss of perfusion during cooling and recovery, the NCP was normal in 152, non-specific in 96, early in 61 and active in 39 patients. The mean ischaemic time was positively associated with the severity of NCP, with P < 0.05 for each two- or three-grade increase and independent of underlying SSc. The difference was most pronounced during recovery. CONCLUSION: We demonstrate that the degree of vasospasm and ischaemia provoked by stepwise cooling and recovery are positively associated with NCP in patients with RP of different aetiologies and without a history of digital ulcers.

Consensus best practice pathway of the UK Scleroderma Study Group: digital vasculopathy in systemic sclerosis.

OBJECTIVE: Digital vasculopathy (comprising RP, digital ulceration and critical digital ischaemia) is responsible for much of the pain and disability experienced by patients with SSc. However, there is a limited evidence base to guide clinicians in the management of SSc-related digital vasculopathy. Our aim was to produce recommendations that would be helpful for clinicians, especially for those managing patients outside specialist centres. METHODS: The UK Scleroderma Study Group set up several working groups to develop a number of consensus best practice pathways for the management of SSc-specific complications, including digital vasculopathy. RESULTS: This overview presents the background and best practice consensus pathways for SSc-related RP, digital ulceration and critical ischaemia. Examples of drug therapies, including doses, are suggested in order to inform prescribing practice. CONCLUSION: A number of treatment algorithms are provided that are intended to provide the clinician with accessible reference tools for use in daily management.

Case report: radial artery occlusion post-radial angiogram in essential thrombocytosis.

Coronary angiographies that are performed via the radial artery generally have lower bleeding complications, however, patients are at risk of radial artery occlusion, with resultant digital ischaemia. This report describes a case of digital ischaemia after transradial coronary angiography in a patient with essential thrombocytosis. Risk factors for thrombo-occlusive complications, and potential prevention strategies are also explored.

Gangrene of the digits of the right lower limb in a patient with homozygous sickle cell disease and ulcerative colitis.

Thrombosis may play an important role in the pathophysiology of certain complications of sickle cell disease (SCD). While the association between SCD and ulcerative colitis (UC) is still debatable, inflammatory bowel disease is known to be associated with an increased incidence of thromboembolic disease. We report a case of a 16-year old girl known to have homozygous SCD and also diagnosed with UC who presented with digital ischemia of her right lower limb. This led to gangrene and subsequent amputation of the first, second and third digits of that limb. This case highlights that patients with both UC and SCD may have an increased risk of thromboembolism and raises the question as to whether patients with UC and SCD should be screened for thrombophilia.