Fortuitous Vasculitis

A 43-year-old man with a cardiac device for dilated cardiomyopathy presented with fever, night sweats, and weight loss. Investigations revealed pancytopenia, acute renal failure, abnormal lung function, and raised inflammatory markers. A renal biopsy demonstrated pauci-immune necrotizing crescentic glomerulonephritis. He was diagnosed with pulmonary–renal antineutrophil cytoplasmic antibody-negative systemic small vessel vasculitis. He commenced immunosuppression with prednisolone and cyclophosphamide with recovery from pancytopenia and improvement in renal function 3 months later. Subsequently, a bone marrow culture grew Mycobacterium fortuitum. Isolation on repeat peripheral mycobacterial blood cultures prompted treatment with ciprofloxacin and clarithromycin. Four months later, he presented with neutropenic sepsis, influenza A/H1N1, and Aspergillus flavus pneumonia. Despite treatment he deteriorated. A transthoracic echocardiogram revealed a vegetation on the right ventricular pacing wire. The device was removed. The vegetation revealed acid and alcohol fast bacilli on Ziehl–Neelsen staining and grew M. fortuitum on culture, sensitive to ciprofloxacin and clarithromycin. Despite device removal and antimicrobial therapy, the patient succumbed to treatment-related complications. The association between glomerulonephritis and endocarditis is well known; however, this is the first case to our knowledge describing pauci-immune necrotizing crescentic glomerulonephritis in the context of M. fortuitum endocarditis. Clinicians should maintain a high index of suspicion for endocarditis in patients with a cardiac device who present with fever and pauci-immune necrotizing crescentic glomerulonephritis. Patients should be investigated with mycobacterial blood cultures, at least three sets of standard blood cultures and transthoracic and transesophageal echocardiography. Clinicians should beware the perils of immunosuppression in the face of an occult sepsis.     

Fortuitous discovery of gallbladder cancer

The prognosis of gallbladder cancer is basically dependent on the histological stage at diagnosis. In practice, the discovery of a small cancer of the bladder, generally during cholecystectomy give the patient a better care for curative treatment. The advent of laparoscopy has increased the number of cholecstectomies and could increase the frequency of this situation but also raises the difficult problem of metastatic dissemination. In the literature the figures on parietal metastasis after laparoscopy have ranged from 125% to 19%. The median delay to diagnosis of recurrence is 6 months. The cause of this phenomenon (role of the pneumoperitoneum) remains poorly elucidated. Risk factors for the development of a metastasis on the trocar orifice are: rupture of the gallbladder perioperatively and extraction of the gallbladder without protection. It is important to keep in mind this exceptional but serious risk and apply rigorous operative technique. In case of suspected gallbladder we do not advocate laparoscopy. Surgery (hepatectomy, lymphodenectomy, possibly resection of the biliary tract) would be indicted for all stages except pTis and T1a, taking into consideration the localization of the tumor and the patient's general status. It is also classical to recommend resection of the trocar orifices after laparoscopic cholecystectomy. There is a dual challenge today for small-sized gallbladder cancer: improving treatment and avoiding poorer prognosis due to the specific problems raised by laparoscopy.     

Systemic vasculitis

This paper is dedicated to a general and synthetic review of a large and heterogeneous group of diseases, entitled "Systemic Vasculites". Despite its rarity in clinical practice, they can be severe and even fatal, if a proper diagnosis and adequate treatment is not made. Among the several classifications, we have adopted the Chapel Hill classification based on its pathogenesis. As they are multiple entities, we will describe only the essential clinical aspects of the diverse vasculitis, including the diagnostic criteria of the American College of Rheumatology, which are very sensitive and specific. We will conclude with a note on the global strategy of treatment of each entity.     

Medium-size-vessel vasculitis

Medium-size-artery vasculitides do occur in childhood and manifest, in the main, as polyarteritis nodosa (PAN), cutaneous PAN and Kawasaki disease. Of these, PAN is the most serious, with high morbidity and not inconsequential mortality rates. New classification criteria for PAN have been validated that will have value in epidemiological studies and clinical trials. Renal involvement is common and recent therapeutic advances may result in improved treatment options. Cutaneous PAN is a milder disease characterised by periodic exacerbations and often associated with streptococcal infection. There is controversy as to whether this is a separate entity or part of the systemic PAN spectrum. Kawasaki disease is an acute self-limiting systemic vasculitis, the second commonest vasculitis in childhood and the commonest cause of childhood-acquired heart disease. Renal manifestations occur and include tubulointerstitial nephritis and renal failure. An infectious trigger and a genetic predisposition seem likely. Intravenous immunoglobulin (IV-Ig) and aspirin are effective therapeutically, but in resistant cases, either steroid or infliximab have a role. Greater understanding of the pathogenetic mechanisms involved in these three types of vasculitis and better long-term follow-up data will lead to improved therapy and prediction of prognosis.     

Systemic vasculitis

The systemic vasculitides are a heterogeneous group of disorders characterized by inflammation of blood vessel walls. They can be classified according to the size of vessel affected and also into primary de novo vasculitides and secondary to other disease processes. Treatments differ and overlap depending on the type of vasculitis. It is therefore important to make the correct diagnosis and treat appropriately to achieve remission as there is a substantial mortality implication.     

Autoimmune testicular vasculitis

A 38-year-old man presented for evaluation of new-onset left testicular pain with swelling. Testicular ultrasound revealed multiple intraparenchymal hypoechoic areas in the inferior portion of the left testicle. The patient was treated with a radical inguinal orchiectomy. Pathologic evaluation revealed intratesticular vasculitis, with involvement of medium-sized arteries. This appears to be a rare case of isolated testicular vasculitis, in the absence of any systemic symptoms.     

Large vessel vasculitis

Takayasu arteritis is a chronic granulomatous disease of the aorta and its major branches that usually affects women during the second and third decades of life, but it has been reported in young children. This review details the clinical, pathological and radiological features, differential diagnoses and management of the condition, focusing chiefly on the disease in children. The recent definition of Takayasu arteritis is discussed. The condition should be considered in patients with unexplained arterial hypertension or unexplained inflammatory syndromes without signs of localization. Since the disease may be life-threatening and progressive, early recognition is necessary to initiate appropriate therapy. Patients with persistent ischaemic symptoms including hypertension might benefit from revascularization procedures.     

Sirolimus-induced leukocytoclastic vasculitis

A 41-year-old white woman with cystic fibrosis underwent lung transplantion that was complicated by cyclosporine-induced nephrotoxicity, which required kidney transplantation. Three years after the renal transplant, sirolimus was substituted for mycophenolate mofetil in a maintenance immunosuppressive regimen that consisted of cyclosporine and prednisone, with the hope of lowering cyclosporine concentrations and avoiding the nephrotoxic effects. Three weeks after the initiation of sirolimus, the patient developed palpable purpura on the bilateral lower extremities that resolved after discontinuation of sirolimus and reappeared with rechallenge. Punch biopsy of the initial eruption revealed leukocytoclastic vasculitis with focal fibrinoid necrosis. Sirolimus should be considered as a causative agent of cutaneous leukocytoclastic vasculitis.