Bilateral breast necrotizing leukocytoclastic vasculitis: First case report

Leukocytoclastic vasculitis (LCV) is a very rare immune complex-mediated condition affecting the small vessels walls. We present the case of a 48-year-old woman with necrotizing bilateral breast LCV on treatment with glatiramer acetate for multiple sclerosis. Bilateral mastectomies and debridement of the anterior abdominal wall were required due to the rapidly evolving necrotizing process. Rapid assessment and a multidisciplinary approach are fundamental in treating this rare life-threatening condition.     

Long‐term outcome of antineutrophil cytoplasmic antibody‐associated small vessel vasculitis after renal transplantation

The survival after renal transplantation of patients with antineutrophil cytoplasmic antibody (ANCA)‐associated to systemic vasculitis is as good as in other diseases, although most of the reports are based on small numbers of patients. Furthermore, it is not known whether comorbidities (cardiovascular [CV] disease and cancer) are more frequent than in general population. We report our experience and the analysis of the published data on this topic. The outcome after transplantation in 49 patients with ANCA‐associated small vessel vasculitis was compared with a control group. The relapse rate of vasculitis was 0.01 per patient per year. Comparison with the control patients revealed no difference in long‐term outcome, CV mortality or incidence of malignancies. In the published literature, patients with ANCA at transplantation and with Wegener's granulomatosis are at greater risk of relapse. Taking our own results together with the review of the literature, we conclude that patient and graft survival rates compare favorably with those in control group that the recurrence rate is very low and that there is no increase in the incidence of cancer or in CV mortality. Patients with ANCA at transplantation and with Wegener's granulomatosis have a higher relapse rate.     

长期硅接触致原发性小血管炎肾损害1例及文献复习

目的：探讨长期硅接触在原发性小血管炎及其肾损害发病中的意义。方法：报道我院新近诊断的1例长期硅接触所致原发性小血管炎肾损害病例,分析其诊治经过,并对相关文献进行复习。结果：患者66岁,硅接触30余年,近期出现血肌酐升高伴蛋白尿、镜下血尿。血清p-ANCA阳性,肾脏病理示新月体肾小球肾炎,经糖皮质激素及环磷酰胺治疗肾功能缓解,但蛋白尿及血尿持续存在。结论：长期硅接触可能是原发性小血管炎及其肾损害的病因之一。     

A case of renal transplantation with granulomatous nephritis and arteritis associated with Pseudomonous aeruginosa infection

Abstract:  A case with graft function failure due to granulomatous interstitial nephritis and arteritis probably associated with a urinary tract infection by Pseudomonas aeruginosa within two months of kidney transplantation is reported. The recipient was a 42-yr-old male who had end-stage renal disease associated with chronic glomerulonephritis. He received a kidney transplant that was donated from a 54-yr-old male who died because of a subarachnoid hemorrhage. On the 60th day after operation, graft function failure occurred. A biopsy specimen showed granulomatous interstitial nephritis and vasculitis. At the same time, P. aeruginosa was detected by urine culture. The graft function is improved by treatment with the antibiotic faropenem for the urinary tract infection. On the basis of these clinical features, it was suggested that the present case developed granulomatous interstitial nephritis and arteritis associated with urinary tract infection by P . aeruginosa .     

Grade III bleeding haemorrhoids in a 6‐year‐old boy

We report a case of grade 3 bleeding haemorrhoids in a 6‐year‐old boy, who had a history of bleeding per rectum since he was 1‐year‐old. The diagnosis was made on anoscopic examination after ruling out other colorectal pathologies by a full‐length colonoscopy. There was no evidence of portal hypertension. He was successfully treated with haemorrhoidectomy. A congenital weakness of the haemorrhoid vein wall was thought to be the probable cause of disease for want of any other causative factor. Though rare to be found at the age of 6 years without any obvious aetiology, haemorrhoids should be considered as one of the causes of bleeding per rectum in children.     

Squamous Cell Carcinoma From Marjolin's Ulcer of the Foot in a Diabetic Patient: Case Study

Squamous cell carcinoma (SCC) has been commonly reported by foot and ankle specialists. Marjolin's ulcer is a malignancy that involves a posttraumatic scar or ulceration that can develop into SCC from chronic neuropathic pedal wounds, venous stasis, or decubitus ulcerations. Most Marjolin's ulcers are found in the lower extremity, specifically the feet, and it is twice as common in females as males. Biopsy of the tumor is the reference standard to diagnose SCC, and wide excision of SCC is the most common treatment option. The present case study describes an 83-year-old diabetic wheelchair-bound female who presented to the wound care clinic with a right heel nonhealing pressure ulceration. After biopsy and surgical excision, the patient was found to have SCC. This case was followed up for 5 years in which the patient had successful excision of the tumor with no recurrence. The clinical significance of our case study is to assist in the diagnosis, management, and prognosis of patients with SCC. In addition, this study has shown that adequate excision of the tumor margins and depth is necessary to prevent potential recurrence and metastasis.     

Two distinct cases with COVID‐19 in kidney transplant recipients

The fatality of novel coronavirus disease 2019 (COVID‐19) is precipitously increased in patients with underlying comorbidities or elderly people. Kidney transplant (KT) recipients are one of the vulnerable populations for infection. COVID‐19 infection in KT recipients might be a complicated and awkward situation, but there has been a lack of reports concerning this group. Herein, we demonstrated two distinct cases with different clinical progress. The first case was a 36‐year‐old man who underwent KT 3 years ago. He was diagnosed with COVID‐19 expressing relevant symptoms. Following administration of lopinavir/ritonavir and hydroxychloroquine with reduced immunosuppressant, he recovered from COVID‐19. However, the unexpected fluctuations in tacrolimus trough levels needed to be managed because of drug‐to‐drug interaction. The second case was developed in a 56‐year‐old man without any symptoms. He received a second KT from an ABO‐incompatible donor 8 years ago. He was diagnosed with COVID‐19 by screening due to exposure history. During the hospitalization period, the chest infiltrative lesion showed a wax and wane, but he successfully recovered by administration of hydroxychloroquine with azithromycin. These apparently different cases suggest that assertive screening and management could improve the clinical course. In addition, antiviral agents should be used cautiously, especially in patients on calcineurin inhibitors.     

Cytomegalovirus‐Induced Polyarteritis Nodosa in a Liver Transplant Recipient

Polyarteritis nodosa (PAN) is a necrotizing vasculitis that has been associated with viral infections, especially hepatitis B virus. We hereby report a case of tissue‐invasive cytomegalovirus (CMV)‐induced PAN in a liver transplant recipient presenting with acute kidney injury and active urinary sediment. Treatment directed against both PAN and CMV resulted in improvement in kidney function, normalization of urinary indices and resolution of the CMV infection. There was no recurrence of either PAN or CMV after a 3‐year follow‐up period.     

Influenza vaccination does not result in an increase in relapses in patients with ANCA-associated vasculitis.

BACKGROUND: Vaccination against influenza has been suggested to induce relapses of ANCA-associated vasculitis but evidence is lacking. In this study, we assessed whether vaccination against influenza increases the occurrence of relapses in patients with ANCA-associated vasculitis. METHODS: Two hundred and thirty consecutive patients with ANCA-associated vasculitis from our out-patient clinics of a tertiary referral center, with at least 1 year of follow-up, were included. Retrospectively, the relapse rate per 100 patients at risk in patients who had been vaccinated against influenza within the preceding year and in patients who not had been vaccinated within that time period were calculated. RESULTS: The relapse rate per 100 patients at risk was lower in patients who had been vaccinated against influenza (3.4) than in patients who had not been vaccinated (6.3), when analyzed for the entire year and for every quarter of the year. Also, the disease-free survival per separate year according to the vaccination status was lower in all 5 years in patients who had been vaccinated, being statistically significant in 2 years. CONCLUSION: Vaccination against influenza does not increase the relapse rate in patients with ANCA-associated vasculitis.     

A novel therapy for an unusual problem: IL‐1 receptor antagonist for recurrent post‐transplant pericarditis

Heart transplant (HTx) recipients are at increased risk of pericardial disease. Idiopathic recurrent pericarditis has not been previously described following HTx. We describe a 35‐year‐old male who was admitted with pericarditis and moderate pericardial effusion 10 months after HTx. Two weeks before his admission, his prednisone had been tapered off. A thorough infectious workup and endomyocardial biopsy was unrevealing. He was started on colchicine with the addition of tapering prednisone regimen of 40 mg daily due to unresolved pain. Over the next several years, he had three recurrent episodes of pericarditis requiring re‐initiation of prednisone with extensive investigations negative for rejection, autoimmune, and infectious causes. Cardiac MRI confirmed pericardial inflammation. Due to his recurrent course and inability to wean off prednisone, anakinra, an IL‐1 receptor antagonist, was started at 100 mg sc daily. This allowed successful discontinuation of prednisone. He is now 34 months post‐transplant without recurrence on anakinra and colchicine maintenance. Due to the overlap between idiopathic recurrent pericarditis and auto‐inflammatory diseases, there is growing evidence for utilizing IL‐1 receptor antagonists in this condition. While pericarditis is common in the HTx population, this is the first report of successful use of an IL‐1 receptor blocker for pericarditis in this population.     

Lymphocytic vasculitis of the prostate transition zone

Study Type – Pathology (case series) Level of Evidence 4 What's known on the subject? and What does the study add? Lymphocytic vasculitis of the prostate is an exceedingly rare form of localised vasculitis that presents without systemic involvement, and is illustrated with anecdotal case reports; often as localised polyarteritis nodosa‐like vasculitis. True incidence and clinical significance of lymphocytic vasculitis of the prostate in surgical specimens is virtually unknown. The present findings support that lymphocytic vasculitis of the prostate was present in 67 (12.4%) of 540 specimens. Lymphocytic vasculitis of the prostate was present in 14 (93.3%) of 15 specimens with prostatic infarction (P < 0.001) with a risk of 124.68 (OR [odds ratio]; 95% CI [confidence interval] 16.07–967.07) as compared with BPH cases not associated with lymphocytic vasculitis.

OBJECTIVE

• ? To present our experience of lymphocytic vasculitis of the prostate in men with benign prostatic hyperplasia (BPH) without systemic involvement, as this is an exceedingly rare form of localised vasculitis and the incidence in surgical specimens and clinical significance of lymphocytic vasculitis is virtually unknown.

PATIENTS AND METHODS

• ? A sequential cohort series of 540 surgical specimens removed because of BPH‐related symptoms, including simple prostatectomy (374 men) and transurethral resection of the prostate (166), comprised the study group.
• ? All men had histological diagnosis of BPH and received surgical therapy only. None of the men had had previous surgery or granulomatous prostatitis.
• ? The mean (range) age at diagnosis was 67.8 (38–89) years.

RESULTS

• ? Lymphocytic vasculitis of the prostate was present in 67 (12.4 %) of 540 specimens. It was seen in a variable number of small‐ to medium‐sized parenchyma arteries with segmental to transmural lymphocytic inflammation, within the morphological spectrum of a polyarteritis nodosa (PAN)‐like lesion seen at the periphery of BPH nodules.
• ? In four cases, focal fibrinoid necrosis was seen in vessels with otherwise typical lymphocytic vasculitis features. Immunohistochemical staining showed a T cell predominant polymorphic cellular infiltrate with a minor component of B cells and monocytes. Six cases additionally had eosinophils (<1% of inflammatory cells).
• ? Lymphocytic vasculitis of the prostate was present in 14 (93.3%) of 15 specimens with prostatic infarction (P < 0.001) with a risk of 124.68 (odds ratio [OR]; 95% confidence interval [CI] 16.07–967.07) as compared with BPH cases not associated with lymphocytic vasculitis. Logistic regression multivariate analysis selected both lymphocytic vasculitis of the prostate and patient age as significant predictors of prostate infarction with lymphocytic vasculitis being the most significant (P < 0.001; OR 128.12; 95% CI 16.298–1007.202). Follow‐up information was available in all cases, range 2–16 years, and none of the patients developed systemic disease.
• ? A validation set of 1665 additional cases including radical prostatectomy, cystoprostatectomy, and needle biopsies showed lymphocytic vasculitis of the prostate being associated to prostate infarction on univariate and multivariate logistic regression (P < 0.001; OR 228.34; 95% CI 45.17–1154.22) analyses.

CONCLUSIONS

• ? Lymphocytic vasculitis in men with BPH is associated with prostatic infarction and should be considered a form of localised vasculitis with PAN‐like morphology that does not necessitate additional evaluation for systemic disease.
• ? The potential clinical relevance of lymphocytic vasculitis warrants further investigation.

     

Comparison of ultrasound‐guided core biopsy versus fine‐needle aspiration biopsy in the evaluation of salivary gland lesions

Ultrasound‐guided core biopsy provides many benefits compared with fine‐needle aspiration cytology and has begun to emerge as part of the diagnostic work‐up for a salivary gland lesion. Although the increased potential for tumor‐seeding and capsule rupture has been extensively discussed, the safety of this procedure is widely accepted based on infrequent reports of tumor‐seeding. In fact, a review of the literature shows only 2 cases of salivary tumor seeding following biopsy with larger‐gauge needle characteristics, with 2 reported cases of salivary tumor seeding following fine‐needle aspiration cytology. However, the follow‐up interval of such studies (<7 years) is substantially less than the 20‐year follow‐up typically necessary to detect remote recurrence. Studies on tumor recurrence of pleomorphic adenoma, the most common salivary gland lesion, suggest that as many as 16% of tumor recurrences occur at least 10 years following initial surgery, with average time to recurrence ranging anywhere from 6.1 to 11.8 years postoperatively. Despite the benefits of ultrasound‐guided core biopsy over fine‐needle aspiration biopsy, which include both improved consistency and diagnostic accuracy, current studies lack adequate patient numbers and follow‐up duration to confirm comparable safety profile to currently accepted fine‐needle aspiration cytology. In this report we: (1) compare the relative benefits of each procedure, (2) review evidence regarding tumor seeding in each procedure, (3) discuss time course and patient numbers necessary to detect tumor recurrence, and (4) describe how these uncertainties should be factored into clinical considerations. © 2012 Wiley Periodicals, Inc. Head Neck, 35 : 1657–1661, 2013     

Preemptive treatment of early donor‐specific antibodies with IgA‐ and IgM‐enriched intravenous human immunoglobulins in lung transplantation

This retrospective study presents our 4‐year experience of preemptive treatment of early anti‐HLA donor specific antibodies with IgA‐ and IgM‐enriched immunoglobulins. We compared outcomes between patients with antibodies and treatment (case patients) and patients without antibodies (control patients). Records of patients transplanted at our institution between March 2013 and November 2017 were reviewed. The treatment protocol included one single 2 g/kg immunoglobulin infusion followed by successive 0.5 g/kg infusions for a maximum of 6 months, usually combined with a single dose of anti‐CD20 antibody and, in case of clinical rejection or positive crossmatch, with plasmapheresis or immunoabsorption. Among the 598 transplanted patients, 128 (21%) patients formed the case group and 452 (76%) the control group. In 116 (91%) patients who completed treatment, 106 (91%) showed no antibodies at treatment end. Fourteen (13%) patients showed antibody recurrence thereafter. In case versus control patients and at 4‐year follow‐up, respectively, graft survival (%) was 79 versus 81 (P = .59), freedom (%) from biopsy‐confirmed rejection 57 versus 53 (P = .34), and from chronic lung allograft dysfunction 82 versus 78 (P = .83). After lung transplantation, patients with early donor‐specific antibodies and treated with IgA‐ and IgM‐enriched immunoglobulins had 4‐year graft survival similar to patients without antibodies and showed high antibody clearance.     

Multiple anal fistulae in a 5‐year‐old boy

We report a case of multiple fistulae in a 5‐year‐old boy, who had a history of episodes of pain and pus discharge from openings around the anus since he was 4 years of age. He underwent simultaneous fistulotomy at three different sites under anaesthesia with complete healing.     

Rituximab for remission induction in recurrent ANCA‐associated glomerulonephritis postkidney transplant

Kidney transplantation (KTX) is the treatment of choice for patients with end‐stage renal disease (ESRD) due to ANCA‐associated vasculitis (AAV). Recurrent ANCA‐associated glomerulonephritis (GN) occurs after KTX and may adversely affect allograft survival. Cyclophosphamide (CYC) combined with glucocorticoids has been the cornerstone of treatment for recurrent GN. Rituximab (RTX), a B‐cell‐depleting monoclonal antibody, is approved for remission induction in AAV. We report the clinical presentation and outcomes of five KTX recipients treated with RTX for biopsy‐confirmed recurrent GN. The median age at the time of KTX was 26 years (four Caucasian, three females). All patients were in remission with four being ANCA positive at time of KTX. Recurrent GN occurred at a median of 26 months post‐KTX. All relapses were treated with RTX and glucocorticoids. Four patients achieved disease remission; the fifth patient was refractory to treatment with RTX and CYC. Follow‐up biopsies (n = 3) showed resolution of active GN in two patients and persistent active GN in one patient. RTX is an alternative to CYC for remission induction in recurrent AAV‐associated GN in KTX patients.     

Rapid recurrence of ANCA-negative pauci-immune vasculitis after cadaveric renal transplantation

We report a patient with renal failure secondary to antineutrophil cytoplasmic autoantibody (ANCA)-negative pauci-immune systemic vasculitis who developed rapid recurrence of vasculitis involving skin, eye, and kidney allograft 1 month after cadaveric renal transplant while being maintained on cyclosporine, mycophenolate mofetil, and prednisolone. Despite early institution of aggressive treatment with plasma exchange, the disease remained uncontrolled and resulted in substantial morbidity and subsequent graft failure within 1 year post-transplantation. This case confirms that ANCA-negative vasculitides may recur in transplanted patients taking mycophenolate mofetil. Transplantation is contraindicated when active disease is evident. © 2001 by the National Kidney Foundation, Inc.     

Sjogren's Syndrome Complicated with IgA Nephropathy and Leukocytoclastic Vasculitis

We report a case of primary Sjögren's syndrome (SS) with cutaneous leukocytoclastic vasculitis and IgA nephropathy. The accurate diagnosis of SS was established based on objective signs and symptoms of ocular and oral dryness, a characteristic appearance of a biopsy sample from a minor salivary gland, and the presence of anti-SS-A autoantibody. A second autoimmune disorder was not present, so the diagnosis of primary SS was established. A histologic finding of skin biopsy of purpuric lesion was typical for leukocytoclastic vasculitis. Renal biopsy was performed for nephrotic range proteinuria. The pathologic finding of renal biopsy was IgA glomerulonephritis with crescent formation. The patient was treated with small doses of glucocorticoids and maintenance hemodialysis. Leukocytoclastic vasculitis is one of the most characteristic extraglandular manifestations of SS. However, IgA nephropathy associated with SS and leukocytoclastic vasculitis is a rare finding. SS patients with glomerulonephritis present a more diverse outcome, even requiring hemodialysis. Therefore, renal biopsy is warranted in SS with glomerulonephritis and systemic vasculitis.     

Aorto‐innominate artery bypass for migrated stent

We report a case of a 64‐year‐old female who first presented with a transient ischemic attack in 2007 due to an innominate artery stenosis, which indicated an endovascular stent placement. In 2008, she presented with recurrence of symptoms and was diagnosed with in‐stent restenosis alongside an unusual occurrence of retrograde migration into the ascending aortic arch. We performed an aorto‐innominate bypass through a median sternotomy. The patient was discharged without any complications thereafter, and the graft has shown excellent patency. As of 2019, the patient remains well.     

Outbreak of Swine Erysipelas in a Semi‐Intensive Wild Boar Farm in Spain

Swine erysipelas (SE) is a disease caused by the bacterium Erysipelothrix rhusiopathiae and is one of the best‐known and most serious diseases affecting domestic pigs. However, few studies exist concerning the susceptibility of wild boars to this disease and the role of this species as a reservoir. This study investigates and describes an outbreak of SE that occurred on a semi‐intensive wild boar breeding farm housing 40 boars in Extremadura (SW Spain) on 11–18 February 2010. Seven animals died, of which four were examined post‐mortem. Of these, three (two females and one male) were approximately 3 months old, and one was 1 year old (male). Lesions were consistent with acute septicaemia, consisting of cutaneous erythema/cyanosis and petechial haemorrhages in kidneys, urinary bladder, lungs and meninges. The 1‐year‐old male also had proliferative polyarthritis. Histopathology confirmed the presence of disseminated intravascular coagulation and vasculitis. Additionally, a bilateral acute panuveitis with concurrent necrotizing vasculitis and diffuse corneal oedema, neither of which have been described before in this disease, were found in the 3‐month‐old male boar. E. rhusiopathiae was isolated from all four animals in pure cultures from several tissues. Of these four animals, antibodies against E. rhusiopathiae, using an indirect ELISA test, were only detected in the 1‐year‐old male boar with polyarthritis. Posteriorly, of nine live adults tested for antibodies, four (including an adult male with polyarthritis) were positive.     

Giant cell arteritis presenting as a stroke in the internal carotid artery territory: a case-based review

Giant cell arteritis (GCA) is a large-vessel vasculitis, typically affecting the aorta and its branches. The involvement of vertebral and internal carotid arteries occurs in a limited number of cases, and stroke as a presenting symptom of GCA is extremely unusual: this subset of the disease has a poor prognosis and rarely responds to immunosuppression.We report the case of a 70-year-old woman, who presented to the Emergency Department for ischemic stroke, which appeared to be the first and only symptom of GCA. The prompt administration of steroids and tocilizumab (TCZ) led to clinical and radiological resolution, with no residual disability at 6-month follow-up.Our case-based review, highlighting the rarity of a large vessel vasculitis presenting only with a cerebrovascular accident, provides new evidence for the efficacy of TCZ even in more unusual varieties of GCA: in these cases, TCZ should be immediately prescribed, in order to prevent mortality and severe long-term morbidity.