Case Of Vasculitis Research Articles

An unexpected case of cryoglobulinaemia vasculitis that presented as respiratory failure

An unexpected case of cryoglobulinaemia vasculitis that presented as respiratory failure

155 There is more to see with Hepatitis C viremic kidney donation: A case of leukocytoclastic vasculitis in aviremic recipient

155 There is more to see with Hepatitis C viremic kidney donation: A case of leukocytoclastic vasculitis in aviremic recipient

Односторонний окклюзирующий васкулит сетчатки как первый симптом при манифестации синдрома Сусака. Клинический случай

A clinical case of unilateral occlusive retinal vasculitis, complicated by macular edema, was described as the first symptom of “possible” Susak syndrome. The presumptive diagnosis was based on the ophthalmological examination and the patient’s complaints. To confirm the diagnosis additional examinations and consultation of other specialists are necessary. Keywords: Susak syndrome, occlusive vasculitis, autoimmune vasculopathy, retinal occlusions

Rituximab-induced vasculitis: A rare occurrence

Rituximab is a chimeric murine/human monoclonal antibody that targets the CD20 antigen expressed on B lymphocytes. It is Food and Drug Administration-approved for use in pemphigus vulgaris (PV). Usual adverse effects are mild, and vasculitis has been reported very rarely. There are only three biopsy-proven case reports of rituximab-induced vasculitis in medical published literature and none in pemphigus. Here, we report a case of rituximab-induced vasculitis in a 46-year-old female with PV.

Retinal Vasculitis in a Patient with Hansen's Disease.

Hansen's disease is endemic in over 140 countries worldwide and a potentially blinding condition. We describe a case of retinal vasculitis in a patient with Hansen's disease with concomitant positive antiphospholipid antibody serology, a potentially under-reported complication in this setting. A 37-year-old Brazilian man systemically stable on triple therapy (clofazimine, rifampin, dapsone) for Hansen's disease presented for a comprehensive ophthalmic evaluation. Dilated exam revealed diffuse peripheral intraretinal hemorrhages in his right eye. Fluorescein angiography showed peripheral non-perfusion, abnormal shunt vessels and leakage from the retinal veins in the right eye and peripheral non-perfusion and vascular leakage in the fellow eye, consistent with vasculitis. Laboratory workup was notable for positive antiphospholipid antibodies (lupus anticoagulant, anticardiolipin IgM, anti-beta-2 glycoprotein 1 IgM) and normocytic anemia. As antiphospholipid antibodies are present in a large proportion of patients with Hansen's disease, it is possible that retinal vasculitis may be more common than previously thought. The extent to which retinal vasculitis occurs in Hansen's disease remains uncertain and possibly underestimated due to the frequency of anterior segment scarring, which impedes retinal evaluation. Heightened surveillance for potential retinal vascular complications is warranted.

Surgical and endovascular cerebral revascularization for cerebral vasculitis with inflammatory vessel stenosis: a case series

Autoimmune vasculitides affect the cerebral vasculature significantly in a considerable number of cases. When immunosuppressive treatments fail to prevent stenosis in cerebral vessels, treatment options for affected patients become limited. In this case series, we present four cases of pharmacoresistant vasculitis with recurrent transient ischemic attacks (TIAs) or stroke successfully treated with either extracranial-intracranial (EC-IC) bypass surgery or endovascular stenting. Both rescue treatments were effective and safe in the selected cases. Our experience suggests that cases of pharmacoresistant cerebral vasculitis with recurrent stroke may benefit from rescue revascularization in combination with maximum medical management.

When Retinal Vasculitis Wears a Mask: Unveiling Retinal Vasculitis Presented as Branch Retinal Vein Occlusion in a Young Male Patient

Introduction : Retinal vasculitis can present with a range of clinical features. Correct diagnosis and prompt treatment are essential to prevent permanent visual loss. We describe a case of retinal vasculitis with branch retinal vein occlusion (BRVO) as initial diagnosis, which is a rare manifestation in a young individual without any vascular risk factors.
 Case Illustration : A 24-year-old male was referred to our center with a sudden onset of blurry vision of his left eye. He was previously diagnosed with BRVO. The patient denied having any chronic diseases, fever, or sore throat. Visual acuity was 6/18 on the left eye and 6/6 on the right eye, respectively. The anterior segment showed no abnormalities. However, fundus examination revealed prominent retinal sheathing, retinal whitening at the inferior quadrant, and macular star. Systemic diseases, such as Bechet disease, sarcoidosis, and systemic lupus erythematosus were excluded by ancillary tests. One month after the initial examination, the patient reported worsening of vision. Funduscopic examination revealed preretinal hemorrhages and macular star. The patient then received intravitreal injection of anti-vascular endothelial growth factor (VEGF), which helped to resolve the hemorrhage and improve vision.
 Discussion : Further investigations, such as fundus fluorescein angiogram, may aid in the diagnosis and management of this condition. Anti-VEGF therapy may be a useful treatment option for retinal vasculitis-associated hemorrhages.
 Conclusion : Our case highlights the importance of considering retinal vasculitis as a differential diagnosis in young patients presenting with BRVO, even in the absence of traditional risk factors.

Scleritis and Development of Immune-Mediated Disease: A Retrospective Chart Review.

Scleritis may be idiopathic or caused by trauma, infections, or an immune-mediated disease (IMD). Our study aimed to understand the relationship between scleritis and IMD, including presenting characteristics, serologies, and treatment course. Understanding these associations may allow clinicians to risk-stratify patients and predict their clinical and treatment course. We conducted a retrospective chart review of 341 patients with scleritis seen at a tertiary care center between January 1, 2005, and December 31, 2020. Demographics, scleritis characteristics, treatment response, recurrence, and serologic data were compared among patients with idiopathic and IMD-associated scleritis. Among patients with scleritis seen, 145 patients (43%) had an associated IMD, most commonly rheumatoid arthritis (RA; 39%), vasculitis (21%), or inflammatory bowel disease (14%). In most cases, the IMD diagnosis predated the scleritis presentation (63%), though vasculitis cases were more likely to develop during or after scleritis episodes. There were no significant differences in demographics or treatment failures among patients with scleritis with and without associated IMDs. Patients with IMDs were more likely to have a recurrence of scleritis (62% vs 49%, P = 0.02). At our ophthalmology center, 43% of patients with scleritis had an associated IMD, and most patients with an IMD were symptomatic from this disease prior to scleritis presentation. RA was the most commonly associated condition and typically predated the scleritis, whereas vasculitis was more likely diagnosed during or after the scleritis episode. Scleritis among patients with IMD is more likely to recur compared to scleritis that is idiopathic.

Brolucizumab Related Retinal Vasculitis Treated with Systemic and Local Steroid, and Vitrectomy

Purpose: To describe a case of retinal vasculitis that followed intravitreal brolucizumab injections and its treatment using local and systemic steroid and vitrectomy.Case summary: An 80-year-old male diagnosed with wet age-related macular degeneration presented after experiencing redness, pain, and vision loss in his left eye following a switch in treatment from aflibercept to brolucizumab 2 weeks earlier. Before the switch, the patient’s best corrected visual acuity of the left eye was 0.4; however, on arrival at the clinic, it was finger count at 10 cm. Suspecting intraocular inflammation and retinal vasculitis caused by brolucizumab, he was treated with both local and systemic steroids and later underwent a vitrectomy. Post-surgery, the patient’s symptoms and vision improved and he received additional anti-vascular endothelial growth factor injections. One year after onset, the corrected vision of his left eye was 0.5 with no observed recurrence of intraocular inflammation.Conclusions: When reactions related to intraocular inflammation associated with brolucizumab are identified, aggressive treatment is crucial. The present case report suggests that satisfactory anatomical and visual outcomes can be achieved.

Diagnostic capabilities of spinal MR-angiography and spinal MR-tractography in Foix-Alajouanine syndrome

The Foix-Alajouanine syndrome was originally reported by these authors in 1926, as rapidly progressive vasculitis on the background of a viral infection. The pathology was represented by the huge, more than 10 times, dilation either of the lumen, or the walls of the spinal vessels, either of the arteries, or the veins. There were no signs of thrombosis, no malformations. Massive necrosis was observed in the spinal cord. Though plenty of observations of the syndrome were reported over the past 100 years, most of them deal with arteriovenous malformations and/or thrombosis, which had not been revealed originally. We present the case of spinal viral vasculitis detected by means of spinal MR-angiography. The undoubted viral etiology of vasculitis allows us to attribute this observation to Foix-Alajouanine syndrome.

An Autopsy Case of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Induced by Propylthiouracil.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a complication caused by antithyroid drugs, particularly propylthiouracil (PTU). Most patients experience organ failure due to the affects of the treatment regimen. We herein report the case of an 89-year-old woman whose severe AAV induced by PTU resulted in various instances of organ failure that eventually led to death after 9 years of PTU therapy. During autopsy, we identified five types of organ failure. As AAV is a potentially fatal disease, the development of various vasculitis symptoms during PTU therapy should therefore be carefully monitored.

A Rare Case of Double-antibody-positive Renal Vasculitis: Are we in a Hurry to Start Tuberculosis Medicines?

Double antibody positive renal vasculitis (anti GBM antibody and pANCA) is a rare form of small vessel vasculitis associated with poor outcome. It is difficult to decide which antibody is the precursor in these cases. We report here such a rare case of double antibody positive renal vasculitis which was started on anti tubercular medicine for a positive mantoux test and interferon gamma release assay. The possible reasons for these antibodies in this may be a complication of COVID-19 infection or vaccine, adverse drug effect of antitubercular medicines or a paradoxical reaction during the tuberculosis treatment. The patient could not survive beyond two months of diagnosis because of rapidly declining renal functions. This case highlights the importance of a meticulous clinical approach, supports two week tuberculosis medicines trial and an early and careful dealing of non responders during tuberculosis treatment especially in patients whose diagnosis is not microbiologically confirmed.

From macular lymphocytic arteritis to cutaneous polyarteritis nodosa: A spectrum of the same disease? Two cases of the same clinical presentation yet different histopathological findings

Macular lymphocytic arteritis (MLA) is a type of indolent cutaneous vasculitis that affects small to medium-sized blood vessels. Histopathologically, it is characterized by a predominantly lymphocytic leukocytic infiltrate (unlike polyarteritis nodosa, in which neutrophils predominate) in the blood vessels of the deep dermis and superficial hypodermis. The disease mainly affects females (17:3), with a mean age of forty years. In general, this entity is considered benign with a variable clinical presentation. The importance of knowing this entity lies in the fact that the pathological findings are discordant with the clinic and an erroneous diagnosis may lead to unnecessary treatments. This vasculitis usually has a benign course and no progression to systemic disease. Herein, we present two cases of this rare vasculitis. Key words: Macular lymphocytic arteritis, Cutaneous polyarteritis nodosa, Vasculitis, Systemic lupus erythematosus

Retinal Vasculitis After Intravitreal Pegcetacoplan: Report From the ASRS Research and Safety in Therapeutics (ReST) Committee.

Purpose: To analyze post-marketing cases of retinal vasculitis after intravitreal pegcetacoplan. Methods: The American Society of Retina Specialists (ASRS) Research and Safety in Therapeutics (ReST) Committee as well as an expert panel performed a retrospective review of cases of retinal vasculitis reported to the ASRS. Clinical and imaging characteristics were reviewed for evidence of retinal vasculitis and analyzed. Results: Fourteen eyes of 13 patients were confirmed to have retinal vasculitis by review of imaging studies. All cases occurred after the first pegcetacoplan injection. Occlusive retinal vasculopathy was confirmed in 11 eyes (79%). Patients presented a median of 10.5 days (range, 8-23 days) after pegcetacoplan injection. All eyes had anterior chamber inflammation, and 12 eyes (86%) had vitritis. Vasculopathy involved retinal veins (100%) more than arteries (73%), and 12 eyes (86%) had retinal hemorrhages. The median visual acuity (VA) was 20/60 (range, 20/30-5/200) at baseline, 20/300 (range, 20/100-no light perception [NLP]) at vasculitis presentation, and 20/200 (range 20/70-NLP) at the last follow-up. Eight eyes (57%) had more than a 3-line decrease in VA, and 6 eyes (43%) had more than a 6-line decrease in VA from baseline to the final follow-up, including 2 eyes that were enucleated. Six eyes (43%) developed signs of anterior segment neovascularization. Conclusions: There is currently no known etiology for vasculitis in this series. Optimum treatment strategies remain unknown. Infectious etiologies should be considered, and corticosteroid treatments may hasten resolution of inflammatory findings. Continued treatment of affected patients with pegcetacoplan should be avoided.

Rituximab as possible therapy in TNF inhibitor-induced IgA vasculitis with severe renal involvement

BackgroundWe observe the increasing use of tumor necrosis factor (TNF) inhibitors in patients affected by chronic inflammatory diseases. These drugs provide good control of symptoms, contributing to significant improvement in the quality of life in individuals with high disease burden. On the other hand, along with their wider use and longer follow-up periods the number of reports regarding their adverse effects is also increasing. The reported complications include drug-induced vasculitis with possible kidney involvement. In the literature we can distinguish more frequently described ANCA-associated vasculitis and more rarely occurring immunoglobulin A vasculitis. Although uncommon, such complications may present with potentially life-threatening vital organ dysfunction; therefore, adequate monitoring and effective therapy are necessary.Case presentationWe report two cases of TNF inhibitor-induced vasculitis with severe acute worsening of renal function and significant proteinuria. The first patient was receiving golimumab therapy for ankylosing spondylitis and the second patient was treated with adalimumab for psoriasis and psoriatic arthritis. In the second case dialysis treatment was necessary and the patient presented recurrence of vasculitis after rechallenge with adalimumab. Both patients underwent renal biopsy which showed findings compatible with drug-induced IgA vasculitis and both were treated successfully with corticosteroids and rituximab.ConclusionsTo the best of our knowledge this is the first report of rituximab use in drug-induced IgA vasculitis with renal involvement. Combination of corticosteroids and rituximab can be an effective therapy in case of vasculitis with kidney failure and a preferable option for selected patients with drug-induced IgA vasculitis compared to cyclophosphamide. More studies are necessary to establish suitable short- and long-term treatment. Given the rarity of this disorder, case reports and case series can provide practical guidance until additional studies become available.

A rare case of oxacillin-induced leukocytoclastic vasculitis

Leukocytoclastic vasculitis is a pathology whose mechanisms are associated with the process of vascular inflammation. It is estimated that up to 24% of the cases of vasculitis are drug-related, with beta-lactam antimicrobials being one of the pharmacological groups commonly associated with this adverse outcome. Oxacillin, a semisyntheticpenicillin, has a beta-lactam ring that confers biological activity and is most frequently associated with reports of leukocytoclastic vasculitis. However, similar cases related to this antimicrobial are rare, with only three cases identified in the literature. Against this background, we report a fourth case of leukocytoclastic vasculitis in a 56-yearold man, on oxacillin treatment, who developed the vasculitis on the 3rd day of antimicrobial use. In addition to oxacillin suspension, he was treated with 125 mg/day of intravenous methylprednisolone for seven days, followed by 20 mg/day of oral prednisone for four days, resulting in satisfactory remission of the skin lesions and no new adverse outcomes. This case provides further evidence supporting the potential causal relationship between the use of oxacillin and the development of leukocytoclastic vasculitis, albeit a rare occurrence. The positive response to therapeutic interventions, such as oxacillin suspension and corticosteroid treatment, underscores the effectiveness of these approaches in addressing this complication.

Retinal Vasculitis Following COVID-19 Infection: A Systematic Review

PURPOSE: We conducted a systematic review of literature to identify cases of vasculitis following corona-virus disease (COVID-19) infection and analyse their specific systemic and ocular features. METHODS: We used the key-words “retina” AND “vasculitis” AND vessels” AND COVID-19 to identify potential papers. We analysed case series, and case reports. RESULTS: 10 papers that describing 12 patients contained enough data to permit analysis and these were included in the study. There were seven female (58.3%) and five male patients (41.6%) with ages ranging from 11 to 66 (mean 42.6 years). Time from onset of the vasculitis following COVID-19 infection varied from three to 150 days (mean 41.0 days). Clinical findings were described in 15 of the 24 eyes of these 12 patients (62.5%) and included anterior uveitis; optic neuropathies; retinal infiltrates and clinical and unilateral or bilateral retinal vasculitis. CONCLUSIONS: Patients with retinal vasculitis appear to constitute a distinct subset and may require different treatment protocols.

Rare Case of Vasculitis Induced by Crusted Scabies Infection

Here we present a 70-year-old man who presented with a rare case of cutaneous leukocytoclastic vasculitis (LCV) secondary to a crusted scabies (Sarcoptes scabiei var hominis) mite infestation. Few cases have been reported in the literature of LCV in association with scabies as LCV is typically caused by a bacterial or viral infection or other malignancy. Since this combination of cutaneous manifestations is rare, diagnosis and treatment may be delayed, allowing for the development of advanced disease.

Vascularite systémique sévère induite par l’ibrutinib

IntroductionThe specific cutaneous toxicity of Bruton's tyrosine kinase inhibitors is poorly described. We report a case of severe systemic vasculitis induced by ibrutinib. ObservationA 73-year-old woman with chronic lymphocytic leukemia was treated with ibrutinib. Eighteen months after treatment onset, ulceronecrotic lesions on toes and tongue occurred. Skin biopsy found vasculitis of small and medium vessels. Biologic tests were negative. This vasculitis was refractory to systemic corticosteroid therapy and azathioprine. Ibrutinib was stopped on the hypothesis of drug-induced vasculitis. Skin lesions improved after discontinuation of ibrutinib. ConclusionThe mechanism of action of ibrutinib does not explain the occurrence of vasculitis and an immunoallergic mechanism is suspected.

A Case of ANCA-Associated Vasculitis with IgG4-Related Disease Treated with the C5a Receptor Inhibitor Avacopan

A Case of ANCA-Associated Vasculitis with IgG4-Related Disease Treated with the C5a Receptor Inhibitor Avacopan