Digital Ischemia Research Articles

OA25 Not so “savy” afterall

Abstract Introduction Interferonopathies (IFN’s) are a constellation of diseases that cause recurrent pathogenic inflammation, arising primarily through antigen-independent hyperactivation of immune pathways¹. It is primarily due to abnormal production or signalling of type I IFNs². A disorder of both the innate and adaptive immune system, this condition is relatively new with the term only coined in 2011. Currently there are more than 20 interferonopathies recognized, with new interferonopathies being identified like Sting associated vasculopathy with onset in infancy (SAVI). Case description Our patient first presented with a severe panniculitis rash, Raynaud’s, swelling of her limbs and hepatosplenomegaly at five weeks of age. She developed significant respiratory distress requiring non-invasive ventilation. A CT Thorax revealed diffuse groundglass opacification suggestive of interstitial lung disease. She had elevated inflammatory markers (CRP 24 mg/L, ferritin 1448 ng/ml, ESR 88 mm/hr), a transaminitis (ALT 193 U/ml, AST 356 U/L). She had multiple positive auto antibodies (Table 1) Clinically SAVI was suspected as the most likely diagnosis. Her interferon signature gene assay demonstrated exaggerated IFI 27 and IFI44L levels. A Trio exome sequencing and IKBKG sequencing however have not been indicative of any currently recognised interferonopathy. Further genetic analysis has been requested to explore possible novel pathogenic mutations. A reasonable clinical response was seen with corticosteroid therapy in combination with Jak Inhibition. With worsening of her cutaneous disease and an increasing need for corticosteroid use a trial of anti IL-6 therapy was initiated. This was subsequently discontinued when she presented with severe acute digital ischemia. It was managed with escalating doses of ruxolitinib, pulse high dose corticosteroids and epoprostenol infusion. Her interferon signature gene assay demonstrated exaggerated IFI 27 and IFI44L levels. A Trio exome sequencing and Inhibitor of nuclear factor kappa B gene (IKBKG) sequencing however have not been indicative of any currently recognised interferonopathy. Further genetic analysis has been requested to explore possible novel pathogenic mutations. A reasonable clinical response was seen with corticosteroid therapy in combination with Jak Inhibition. With worsening of her cutaneous disease and an increasing need for corticosteroid use a trial of anti IL-6 therapy was initiated. This was subsequently discontinued when she presented with severe acute digital ischemia. It was managed with escalating doses of ruxolitinib, pulse high dose corticosteroids and epoprostenol infusion. Discussion This case enhances our current understanding of interferonopathies. Although the phenotype and course of the disease is compatible with SAVI³, genetic testing is negative to date. Presently there are eleven heterozygous gain-of-function (GoF) variants for SAVI4. With JAK inhibition as the most successful therapeutic option to date5. Our case possibly represents an as yet unreported genetic variant, further studies are awaited.This case enhances our current understanding of interferonopathies. Although the phenotype and course of the disease is compatible with SAVI³, genetic testing is negative to date. Presently there are eleven heterozygous gain-of-function (GoF) variants for SAVI4. With JAK inhibition as the most successful therapeutic option to date5. Our case possibly represents an as yet unreported genetic variant, further studies are awaited.This case enhances our current understanding of interferonopathies. Although the phenotype and course of the disease is compatible with SAVI³, genetic testing is negative to date. Presently there are eleven heterozygous gain-of-function (GoF) variants for SAVI4. With JAK inhibition as the most successful therapeutic option to date5. Our case possibly represents an as yet unreported genetic variant, further studies are awaited. Key learning points • Early suspicion for an interferonopathy in very young infants with interstitial lung disease • Expanding the potentially new genetic variants of this condition • Challenge of treatment management

A 58-year-old man with subacute digital ischemia of all limbs.

A 58-year-old man with subacute digital ischemia of all limbs.

Digital ischaemia associated with paraneoplastic systemic sclerosis

Digital ischaemia associated with paraneoplastic systemic sclerosis

Intermittent versus continuous intravenous epoprostenol for the treatment of digital ischemia

Digital ischemia can be a painful complication of Raynaud’s phenomenon or systemic sclerosis, which is caused by narrowing of blood vessels in the toes and hands. Epoprostenol is a potent vasodilator that may be used to treat digital ischemia in this patient population. Our institution provides epoprostenol infusion using two different administration techniques: a 30-h continuous infusion option and a 5-day intermittent 6-h infusion. In this retrospective chart review, we compared two administration techniques of intravenous epoprostenol administered to patients with digital ischemia. The primary outcome was to compare the efficacy of intravenous epoprostenol 30-h continuous infusion versus 5-day intermittent infusion, as defined by the presence of treatment failure. Between June 2019 and June 2020, 72 adult patient encounters met the inclusion criteria (intermittent: n = 20; continuous: n = 52). The primary outcome did not achieve a statistically significant difference between the two groups: intermittent 20% versus continuous 33.3% p = 0.390, odds ratio = 0.57 (95% confidence interval = 0.17–1.90). Adverse reactions were documented in 28% of patients across both treatment groups, and there was no difference detected when treatment groups were compared (25% vs 28.8%). Patients who received the 5-day infusion experienced a significantly longer average length of stay, with a mean of 8.9 days versus 3 days for those treated with the continuous 30-h infusion ( p < 0.05; 95% confidence interval = 2.15–9.47). This study determined that the efficacy and safety profiles of the two administration techniques may not be comparable. Each protocol offers advantages over the other, and selection should be guided by patient history and risk factors to optimize management.

Digital ischemia: a rare immune-related adverse event of immune checkpoint inhibitors-case report and review of the literature.

Immune checkpoint inhibitors (ICIs) play a crucial role in treating various cancers. While ICIs are invaluable in the fight against different cancers, they also pose the risk of immune-related adverse events (irAEs), which can range widely in symptoms and severity. Rheumatologic complications, including digital ischemia, are among the irAEs. While rare, they require early detection for effective management. The aim of the study is to present a case report on digital ischemia related to immunotherapy and to conduct a literature review on relevant cases. We present a case involving a patient from our oncology department who developed, pericarditis, digital ischemia and anti-centromere antibodies during immunotherapy with pembrolizumab for non-small cell lung cancer (NSCLC). We collaborated with our rheumatology department to initiate treatment, including corticosteroids, iloprost, and mycophenolate mofetil. Through the follow-up, the patient showed clinical improvement. A literature review identified only 10 relevant articles, highlighting the rarity of digital ischemia as an irAE. Corticosteroids and vasodilators were commonly used treatments, with amputation unavoidable in 40% of cases. IrAEs are becoming more common due to the widespread use of ICIs. For this reason, it is crucial to diagnose and treat rare IrAEs, such as digital ischemia, as early as possible to improve outcomes.

Elevated neutrophil extracellular traps in systemic sclerosis-associated vasculopathy and suppression by a synthetic prostacyclin analog

ObjectivesNeutrophils and neutrophil extracellular traps (NETs) contribute to the vascular complications of multiple diseases, but their role in systemic sclerosis (SSc) is understudied. We sought to test the hypothesis that NETs are implicated in SSc vasculopathy and that treatment with prostacyclin analogs may ameliorate SSc vasculopathy not only through vasodilation but also by inhibiting NET release.MethodsBlood from 125 patients with SSc (87 diffuse cutaneous SSc and 38 limited cutaneous SSc) was collected at a single academic medical center. Vascular complications such as digital ulcers, pulmonary artery hypertension, and scleroderma renal crisis were recorded. The association between circulating NETs and vascular complications was determined using in vitro and ex vivo assays. The impact of the synthetic prostacyclin analog epoprostenol on NET release was determined.ResultsNeutrophil activation and NET release were elevated in patients with SSc-associated vascular complications compared to matched patients without vascular complications. Neutrophil activation and NETs positively correlated with soluble E-selectin and VCAM-1, circulating markers of vascular injury. Treatment of patients with digital ischemia with a synthetic prostacyclin analog boosted neutrophil cyclic AMP, which was associated with the blunting of NET release and reduced NETs in circulation.ConclusionOur study demonstrates an association between NETs and vascular complications in SSc. We also identified the potential for an additional therapeutic benefit of synthetic prostacyclin analogs, namely to reduce neutrophil hyperactivity and NET release in SSc patients.

Severe digital ischaemia as a presentation of antisynthetase syndrome

Severe digital ischaemia as a presentation of antisynthetase syndrome

Red flags for clinical suspicion of eosinophilic granulomatosis with polyangiitis (EGPA)

BackgroundEosinophilic granulomatosis with polyangiitis (EGPA), is a rare ANCA-associated systemic vasculitis. Its overlapping features with other vasculitic or eosinophilic diseases, and the wide and heterogeneous range of clinical manifestations, often result in a delay to diagnosis. ObjectiveTo identify red flags that raise a suspicion of EGPA to prompt diagnostic testing and to present an evidence-based clinical checklist tool for use in routine clinical practice. MethodsSystematic literature review and expert consensus to identify a list of red flags based on clinical judgement. GRADE applied to generate a strength of recommendation for each red flag and to develop a checklist tool. Results86 studies were included. 40 red flags were identified as relevant to raise a suspicion of EGPA and assessed by the experts as being clinically significant. Experts agreed that a diagnosis of EGPA should be considered in a patient aged ≥6 years with a blood eosinophil level >1000 cells/µL if untreated and >500 cells/µL if previously treated with any medication likely to have altered the blood eosinophil count. The presence of asthma and/or nasal polyposis should reinforce a suspicion of EGPA. Red flags of asthma, lung infiltrates, pericarditis, cardiomyopathy, polyneuropathy, biopsy with inflammatory eosinophilic infiltrates, palpable purpura, digital ischaemia and ANCA positivity, usually anti-myeloperoxidase, among others, were identified. ConclusionThe identification of a comprehensive set of red flags could be used to raise a suspicion of EGPA in patients with eosinophilia, providing clinicians with an evidence-based checklist tool that can be integrated into their practice.

Amputated Digit Replantations: Critical Digit Ischemia Timing, Temperature, and Other Predictors of Survival.

A common consideration for replantation success is the ischemia time following injury and the preservation temperature. A classic principle within the hand surgery community describes 12 hours of warm ischemia and 24 hours of cold ischemia as the upper limits for digit replantation; however, these limits are largely anecdotal and based on older studies. We aimed to compare survival data from the large body of literature to aid surgeons and all those involved in the replantation process in hopes of optimizing success rates. The PubMed database was queried on April 4th, 2023, for articles that included data on digit replantation survival in terms of temperature of preservation and ischemia time. All primary outcomes were analyzed with the Mantel-Haenszel method within a random effects model. Secondary outcomes were pooled and analyzed using the chi-square statistic. Statistical analysis and forest plot generation were completed with RevMan 5.4 software with odds ratios calculated within a 95% confidence interval. Our meta-analysis identified that digits preserved in cold ischemia for over 12 hours had significantly higher odds of replantation success than the amputated digits replanted with 0-12 hours of warm ischemia time ( P ≤ 0.05). The odds of survival in the early (0-6 hours) replantation group were around 40% greater than the later (6-12 hours) replantation group ( P ≤ 0.05). Secondary outcomes that were associated with higher survival rates included a clean-cut amputation, increased venous and arterial anastomosis, a repair that did not require a vein graft, and replants performed in nonsmokers ( P ≤ 0.05). Overall, these findings suggest that when predicting digit replantation success, time is of the essence when the digit has yet to be preserved in a cold environment. This benefit, however, is almost completely diminished when the amputated digit is appropriately maintained in a cold environment soon after injury. In conclusion, our results suggest that there is potential for broadening the ischemia time limits for digit replant survival outlined in the literature, particularly for digits that have been stored correctly in cold ischemia.

Nonbacterial thrombotic endocarditis of mitral valve associated with a lymphoproliferative malignancy: case report and literature review

BackgroundNon-bacterial thrombotic endocarditis (NBTE) is a rare condition marked by sterile vegetations on cardiac valves, often linked to rheumatologic diseases, autoimmune disorders, and advanced solid malignancies. An early diagnosis and treatment of the associated clinical condition are mandatory, although they do not usually eliminate valvular vegetations, making anticoagulation essential to prevent embolic events. Despite variability, the prognosis of NBTE is usually unfavorable due to recurrent embolic events and the severity of the primary condition, typically advanced cancer.Case presentationWe present a case of a 57 years-old male who presented to the emergency department with a 5-day history of painful bilateral digital edema and color change episodes (from pallor to cyanosis). Physical examination revealed erythrocyanosis in the distal extremities, prompting consideration of secondary Raynaud syndrome.Despite medical therapy, progressive digital ischemia led to multiple finger amputations. During etiological investigation, anticoagulation tests and autoimmune analysis yielded negative results. A transesophageal echocardiogram was performed, revealing an irregular hyperechogenic mass on the anterior leaflet of the mitral valve without valve dysfunction, and a thoracic computed tomography scan with contrast showed an enlarged right paratracheal lymph node. Histopathological analysis from a transthoracic needle biopsy of the paratracheal lymph node revealed diffuse large B-cell lymphoma. The patient underwent aggressive R-CHOP chemotherapy, achieving a favorable complete response.ConclusionThis is a particular case involving the occurrence of NBTE and Raynaud phenomenon as the initial paraneoplastic manifestations in a previously healthy young man. Reports of NBTE associated with lymphoproliferative conditions are quite rare, with fewer than ten cases described in the literature. To our knowledge, this is the first case of NBTE specifically associated with diffuse large B-cell lymphoma.

Delayed Diagnosis of Vasopressor-induced Symmetrical Peripheral Gangrene in Septic Shock Patient

Vasopressors are powerful vasoconstrictors used in critically ill patients to raise blood pressure after volume resuscitation attempts fail. These medications, while lifesaving, carry the risk of severe and under-reported side effects, digital ischemia, and gangrene due to decreased digital perfusion. We here present a case of vasopressin-induced digital gangrene in a female patient who underwent elective surgery for nephrolithiasis without any pre-existing peripheral vascular disease or smoking. The patient developed bilateral upper and lower limb symmetrical acronecrosis. The causality assessment as per the World Health Organisation – Uppsala Monitoring Centre scale was suggestive of a “Possible adverse” event. The patient denied surgical intervention, eventually developed multiorgan failure, and died of cardiopulmonary arrest. Although lifesaving, vasopressors due to alpha constricting activity can cause severe limb ischemia with dreadful consequences. These drugs should be used judiciously with careful monitoring and tapering as soon as possible when the condition improves. The consequences could be dreadful if timely surgical intervention and measures are not taken.

A Review of 1228 In-Office Hand Surgery Procedures With Wide Awake Local Anesthesia No Tourniquet (WALANT) at a Single Private Practice.

This study examined the complication rate of Wide Awake Local Anesthesia No Tourniquet (WALANT) technique in the clinic setting with field sterility at a single private practice. We hypothesized that WALANT is safe and effective with a low complication rate. This retrospective chart review included 1228 patients who underwent in-office WALANT hand procedures at a single private practice between 2015 and 2022. Patients were divided into groups based on type of procedure: carpal tunnel release, A1 pulley release, first dorsal compartment release, extensor tendon repair, mass excision, foreign body removal, and needle aponeurotomy. Patient demographics and complications were recorded; statistical comparisons of cohort demographics and risk factors for complications were completed, and P < .05 was considered significant for all statistical comparisons. The overall complication rate for all procedures was 2.77% for 1228 patients including A1 pulley release (n = 962, 2.7%), mass excision (n = 137, 3.7%), extensor tendon repair (n = 23, 4.3%), and first dorsal compartment release (n = 22, 8.3%). Carpal tunnel release, foreign body removal, and needle aponeurotomy groups experienced no complications. No adverse events (e.g. vasovagal reactions, digital ischemia, local anesthetic toxicity, inadequate vasoconstriction) were observed in any group. Patients with known autoimmune disorders and those who were currently smoking had a statistically significant higher complication rate. Office-based WALANT procedures with field sterility are safe and effective for treating common hand maladies and have a similar complication profile when compared to historical controls from the standard operating room in an ambulatory center or hospital.

Outcomes of Wide Awake Local Anaesthesia no Tourniquet (WALANT) In Soft Tissue Surgeries Of The Hand

Introduction: Hemostasis in hand surgery can be achieved by use of an arm tourniquet but it has its own disadvantages like tourniquet-related pain, muscle and nerve injury, post-tourniquet thrombosis, and bleeding. Another method can be the use of epinephrine locally, but surgeons are concerned with digital ischemia and necrosis as seen with use of procaine in the 1970s. Recently Wide Awake Local Anesthesia No Tourniquet (WALANT) is being popular among hand surgeons in which a mixture of local anesthetic with adrenaline and sodium bicarbonate is used. Despite the absence of tourniquet related complications in WALANT technique, surgeons are reluctant to use lignocaine with epinephrine in hand surgery.&#x0D; Objectives: The objective of this study is to observe the outcomes of WALANT technique in various surgical procedures of hand.&#x0D; Methodology: This prospective study involved 103 patients undergoing soft tissue surgery of the hand at Birat Medical College Teaching Hospital. Patients meeting inclusion criteria and willing to participate in study were included. A proforma was filled by the operating surgeon and another proforma was to be filled by patient at home after discharge which was retrieved at follow-up visit. A mixture of 10 ml of 2% lignocaine with adrenaline diluted in equal volume of normal saline and 2ml of 8.4% sodium bicarbonate was used for local anesthesia.None of our patients developed digital ischemia or necrosis, one patient developed surgical site infection which recovered with oral antibiotics. The mean VAS score at the time of administration of local anesthesia was 3.8+1.2, intraoperatively it was 1.8+ 1.1 and at 2 hours postoperatively it was 2.3+1.3. VAS score at 6 hours, 12 hours and 24 hours postoperatively was 3.2+1.2, 3.4+1.4 and 3.7+1.1 respectively. Hemostasis control was excellent in 33 cases, very good in 58 cases, good in 8 cases, and poor in 4 cases.&#x0D; Conclusion: We conclude that WALANT technique is safe to use in various soft tissue surgeries of hand surgery in terms digital ischemia, necrosis or wound infection and effective in providing excellent control of hemostasis at surgical site.

Antineutrophil Cytoplasmic Autoantibody-negative Pauci-immune Necrotizing Glomerulonephritis with Plasma Cell-rich Tubulointerstitial Nephritis Complicated with Pleuritis and Digital Ischemia.

Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) predominantly affects small vessels. Almost all AAV patients are positive for myeloperoxidase- or proteinase 3-ANCA, and ANCA plays a crucial role in the pathogenesis of AAV. We herein report an ANCA-negative AAV patient with pauci-immune necrotizing glomerulonephritis and plasma cell-rich tubulointerstitial nephritis who was complicated with pleuritis and digital ischemia. ANCA-negative AAV is a rare clinical entity that is difficult to diagnose, and pleuritis and digital ischemia are rare manifestations of AAV. An early diagnosis and appropriate treatment are important, as any delay in the diagnosis may worsen the prognosis.

Raynaud’s Phenomenon as a Manifestation of Vasculitis C – ANCA: Case Report and Literature Review

Raynaud's phenomenon is a vasospastic condition affecting capillaries in the distal region of the extremities. It is divided into primary and secondary and 80-90% of patients are associated with primary or idiopathic disease, while 10-20% are secondary, with multiple etiologies including connective tissue, vascular, hematologic, and endocrinopathic diseases. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) correspond to a group of necrotizing vasculitides of medium (0.2 to 2.0 mm in diameter) and small blood vessels (arterioles, capillaries, postcapillary venules). Clinically, it is characterized by multisystem involvement, with upper and lower respiratory tract and renal damage standing out for their frequency. The least frequent manifestations are cutaneous, with digital ischemia and gangrene occurring in less than 1% of cases. The diagnosis is based on a detailed clinical history and physical examination in addition to complementary studies including a complete metabolic panel, muscle enzymes, viral panel, thyroid profile and a rheumatologic panel to assess secondary causes of Raynaud's phenomenon. Calcium antagonists are considered the first-line treatment in both etiologies, and phosphodiesterase-5 inhibitors are another alternative. This article presents a case of a patient with Raynaud's phenomenon as an initial clinical manifestation and later presentation of C-ANCA vasculitis.

Successful Combination Therapeutic Strategy for Treatment of Digital Necrosis in Systemic Sclerosis

Currently, there are no randomized trials that assess combination treatments of digital ulcers in systemic sclerosis (SSc). Here, we present a clinical case of successful treatment of digital necrosis in SSc patient with diffuse cutaneous involvement with complete recovery for a period of 2 months, using combination therapeutic strategy (felodipine, intravenous iloprost, sildenafil, anticoagulant, local treatment). Treatment of severe digital ischemia in SSc is a challenge in rheumatology. However, complete and quick recovery including in cases with digital necrosis with minimal tissue loss could be achieved with close monitoring of the patients, providing complex care with the use of combination pharmacological therapy and local treatment.

Digital Ischemia as a Rare Manifestation of C3 Glomerulopathy Associated With Monoclonal Gammopathy

Digital Ischemia as a Rare Manifestation of C3 Glomerulopathy Associated With Monoclonal Gammopathy

Distal periarterial sympathectomy surgery for chronic digital ischemia: A systematic review of the literature

ObjectiveThis study assesses the efficacy of distal periarterial sympathectomy in treating chronic digital ischemia by evaluating clinical outcomes of surgery. MethodsA systematic literature review of distal sympathectomy for chronic digital ischemia was conducted. Data extracted included study design, patient statistics, aetiology, follow-up duration, sympathectomy level, and surgical outcomes. Results21 studies were analysed, containing a total of 337 patients, 324 hands, and 398 digits. Patient age ranged from 23.2 to 56.6 years. Causes of ischemia included Scleroderma, Raynaud's disease, atherosclerosis/Buerger's disease, systemic lupus erythematosus/discoid lupus, undifferentiated rheumatic disorder/mixed connective tissue disease, CREST syndrome, trauma and unknown diagnoses. Common digital artery sympathectomy was mostly performed. Follow-up spanned 12–120 months. OutcomesDistal sympathectomy led to reduced pain in 94.7 % patients. Complete resolution of ulceration was seen in 73 % patients. Subsequent amputation was required in 28 % patients. Other complications were reported in 24.1 % patients. ConclusionsThis study indicates that distal periarterial sympathectomy may effectively treat chronic digital ischemia, offering pain relief and resolution of digital ulceration. However, risks of complications and amputation persist. Further research is required to inform patient selection and establish the optimal technique and extent of distal sympathectomy surgery, before it can be considered a valid treatment option.

Mixed cryoglobulinaemia vasculitis secondary to marginal zone lymphoma in a patient with end-stage renal failure on haemodialysis

Cryoglobulinaemia vasculitis can present with a variety of symptoms and there is limited data on the incidence and presentation of cryoglobulinaemia vasculitis in haemodialysis patients. We report a case of a 63-year-old male who had a series of presentations with rash, visual changes, abdominal pain, weight loss, fevers and digital ischaemia. This is on a background of a congenital single kidney with end-stage renal failure secondary to diabetes and hypertension, receiving haemodialysis for nearly 5 years. He initially experienced a leukocytoclastic vasculitis rash confirmed on skin biopsy, followed by multiple hospital presentations for undifferentiated abdominal pain and fever of unknown source. Jejunal biopsy revealed intestinal vasculitis. His peripheral blood flow cytometry and bone marrow biopsy were consistent with marginal zone lymphoma (indolent subtype, IgM kappa clone). Further testing revealed a type II cryoglobulinaemia consisting of an IgM kappa monoclonal band with polyclonal IgG (cryocrit 5%). A diagnosis of cryoglobulinaemia vasculitis was established and he was treated with pulsed methylprednisolone and rituximab therapy. However, after receiving three doses of rituximab the patient developed a presumed vasculitis-associated pulmonary haemorrhage for which he received treatment with five sessions of plasma exchange. His symptoms resolved and cryocrit reduced to < 1% after his final dose of rituximab. The clinical features of cryoglobulinaemia may be difficult to detect in chronic haemodialysis patients and vigilance is required.

OA18 Small vessel and large vessel vasculitis due to rheumatoid vasculitis: a case of mononeuritis multiplex with pulseless foot leading to amputation

Abstract Introduction Rheumatoid vasculitis occurs in patients with longstanding severe seropositive rheumatoid arthritis. The incidence has reduced drastically with availability of more effective medication. Rheumatoid vasculitis primarily affects small and medium vessels, and rarely large vessels as well. Case description This 77-year-old lady with deforming seropositive rheumatoid arthritis was stable on methotrexate under the care of multiple Trusts for the last 30 years. Methotrexate had been stopped a year ago due to pancytopenia and she was started on prednisolone and hydroxychloroquine. While on these medications she flared with arthralgia and had difficulty in walking attributed to ankle synovitis. She was started on adalimumab, after 3 doses of which she developed widespread skin psoriasis leading to discontinuation. Subsequently, she was admitted to our Trust with left foot gangrene and was referred to rheumatology. Further questioning revealed that numbness in both lower limbs, inability to walk and frequent falls had started following the cessation of methotrexate. On examination, there was bilateral foot drop, worse on the right side. The left foot was gangrenous with diminished pulses in dorsalis pedis and posterior tibial arteries with preserved all other pulses. There was no vasculitic rash or nail fold infarcts. Psoriatic rash had resolved and there was no active synovitis in the joints. Nerve conduction test revealed severe ongoing axonal degenerative type sensory and motor polyneuropathy in the lower limbs without any upper limb involvement. CT angiogram showed multifocal stenoses, more so in the left common iliac, external iliac and superficial femoral artery. There were no conventional vascular risk factors such as hypertension, diabetes, dyslipidaemia or smoking, and there was no past history of cardiovascular disease. PET-CT scan did not reveal any features of vasculitis or aortitis. The vasculitis screening was negative including cryoglobulins. Initially she was on IV antibiotics for left foot cellulitis, hence she was managed with steroids without any potent immunosuppressive agents. Subsequently, she underwent left sided below knee amputation. After the wound had healed, she was started on IV rituximab with low-dose methotrexate and steroid taper. Discussion Although rare, rheumatoid vasculitis can cause significant morbidity and complications. Rheumatoid vasculitis tends to occur in patients with so-called ‘burnt-out’ RA and strongly associated with seropositivity. While it can affect many organ systems, skin and the peripheral nervous system are most frequently affected. Cutaneous vasculitis occurs in around 90% of patients and is the most common clinical manifestation. This includes parable purpura, leg ulcers and nailfold infarcts. It may lead to digital ischaemia, necrosis and gangrene if large vessels are involved. Another common manifestation is a vasculitic neuropathy due to involvement of the vaso nervorum. Around 40% have a sensory neuropathy and 20% have a mixed sensory motor neuropathy like the index patient. Ocular manifestations such as scleritis have been described and may be the first indication of rheumatoid vasculitis. Aortitis in rheumatoid vasculitis may present with aortic insufficiency and aneurysm formation. Pericarditis has also been described as a possible disease manifestation in patients with rheumatoid vasculitis. Our patient did not have any of these clinical features and the PET-CT scan was unremarkable, which could have been due to the steroids she was on prior to the scan. Clinically, there was pulseless gangrene of the left foot with CT angiogram showing multiple stenosis suggesting large vessel involvement in the absence of conventional vascular risk factors. Renal involvement in rheumatoid vasculitis is very rare. PAN like renal involvement and glomerulonephritis has been described. Our patient did not have any haematuria or any significant proteinuria, and imaging did not reveal any renovascular aneurysms. Isolated nailfold infarcts do not require any systemic treatment. Systemic involvement with rheumatoid vasculitis requires aggressive treatment with high-dose steroids with cyclophosphamide or rituximab. Our patient had neuropathy and gangrene of the left foot, hence she was started on rituximab accordingly. Key learning points Rheumatoid vasculitis is a rare but serious complication which can present with a vasculitic neuropathy/mononeuritis multiplex. Rarely, it can cause ischemia and gangrene. If there is systemic involvement, aggressive therapy with steroids, cyclophosphamide or rituximab is needed.