Vasculitis involving the breast: a clinical and histopathologic analysis of 34 patients.

Center for Vasculitis Care and Research, Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH, USA.
Medicine (Impact Factor: 5.72). 04/2008; 87(2):61-9. DOI: 10.1097/MD.0b013e31816a8d1f
Source: PubMed


Vasculitis of the breast (VB) may be an isolated finding or a manifestation of systemic vasculitis. In the current study we sought to characterize isolated VB (IVB) and compare it to VB in the setting of systemic vasculitis. We studied VB cases in the literature and patients cared for at our institution. We analyzed clinical, laboratory, and histologic features (including vessel size and type of inflammatory infiltrates); course of illness; biopsy procedure; and treatment. Based on the presence of localized or systemic disease at the time of disease presentation and during the follow-up, we divided patients into 3 groups: IVB (Group 1), VB with proven or indirect evidence of systemic vasculitis (Group 2), and VB with possible systemic involvement (Group 3). We identified a total of 34 cases of VB (30 from PubMed [National Library of Medicine, Bethesda, MD] and 4 from our pathology database). All patients presented with breast lesions, which were the only expression of disease in 16 (47%). Eighteen, 6, and 10 patients belonged to Group 1, 2, and 3, respectively. Constitutional symptoms were present less often in Group 1. Musculoskeletal symptoms occurred only in Groups 2 and 3. Patients in Groups 2 and 3 had higher erythrocyte sedimentation rates and lower hemoglobin levels, and also received corticosteroids more frequently than those in Group 1. No differences were found in the other analyzed parameters between groups. In summary, VB is uncommon, and in about half of the cases, occurs in the form of IVB. Histologic characteristics do not correlate with disease extent. In IVB patients, constitutional and musculoskeletal manifestations are usually absent. Such patients generally do not require systemic therapy and may be cured by resection alone.

Download full-text


Available from: José Hernández-Rodríguez, Oct 27, 2014
  • [Show abstract] [Hide abstract]
    ABSTRACT: In the field of autoimmunity, much has been learned from studying circulating and tissue bound immune-reactive cells, cytokines and antibodies. However, what has brought those cells to the site of injury, for most forms of vasculitis remains a mystery. Might the etiology of at least certain forms of vasculitis be related to generation of neoantigens in the native vessel, making that vessel the target of a pathogenic immune response? How might one explain organ targeting and patterns of disease that are so critical to the diagnostic process? Embryologists have demonstrated great diversity in the vasculature of different organs. Unique quantitative and qualitative features become apparent in vascular territories as early as the third week of gestation. These differences are later amplified by the effects of further development, aging, infection, spontaneous mutations and other co-morbidities. Based on data from these observations a testable hypothesis would be that many forms of vasculitis may begin with emergence of new antigens within affected vessel walls and the resulting immune response may in fact be a normal reaction to perceived foreign protein(s).
    Bulletin of the NYU hospital for joint diseases 02/2008; 66(3):224-7.
  • [Show abstract] [Hide abstract]
    ABSTRACT: The primary systemic vasculitides are autoimmune disorders characterized by chronic immune responses directed against vascular structures. They commonly affect small or medium-sized vessels in the peripheral nervous system (PNS), producing vasculitic neuropathies. Some patients develop vasculitis clinically restricted to the PNS, known as nonsystemic vasculitic neuropathy (NSVN), the most commonly encountered vasculitic neuropathy in pathologically based series. Diabetic and nondiabetic radiculoplexus neuropathies are clinical variants of NSVN. NSVN is clinically similar to systemic vasculitis-associated neuropathies except for reduced severity. Patients most commonly present with progressive, stepwise pain, weakness, and numbness over multiple months. Almost all exhibit a multifocal or asymmetric, distally accentuated pattern of involvement. The most commonly affected nerves are the common peroneal nerve in the leg and the ulnar nerve in the arm. Sedimentation rate is mildly to moderately elevated in 50%; other markers of systemic inflammation are generally normal. Electrodiagnostic studies reveal a predominantly axonal, asymmetric, sensorimotor polyneuropathy, but pseudo-conduction blocks may occur. Definite diagnosis requires biopsy evidence of vascular inflammation and signs of active or remote vascular damage. In biopsies lacking definite vasculitis, the diagnosis is suspected if axonal alterations are accompanied by perivascular inflammation and such supportive features as Wallerian-like degeneration, asymmetric fiber loss, hemosiderin, vascular immune deposits, neovascularization, myofiber necrosis/regeneration, focal perineurial damage, and endoneurial purpura. NSVN preferentially affects larger epineurial arterioles. Epineurial infiltrates are composed primarily of T cells and macrophages, suggesting that cellular cytotoxicity is the primary effector mechanism. Systemic vasculitides with progressive neuropathy are usually treated with cyclophosphamide and prednisone. No randomized controlled trial of therapy has been performed in NSVN, but data from retrospective cohorts suggest that combination therapy is more effective than steroid monotherapy. Once remission has been induced, cyclophosphamide should be replaced with azathioprine or methotrexate. Refractory patients can be treated with intravenous immunoglobulin, mycophenolate, rituximab, infliximab, or alemtuzumab. Although long-term outcome is reasonably good, more than one third of patients relapse, infrequent patients die from the disease or its treatment, and still others develop chronic pain.
    Frontiers of neurology and neuroscience 02/2009; 26:26-66. DOI:10.1159/000212368
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Gynecologic vasculitis (GynV) has been reported as part of systemic vasculitis (SGynV) and as single-organ (isolated gynecologic) vasculitis (IGynV). In the current study, we analyzed the clinical and histologic characteristics of patients with GynV and sought to identify features that differentiate the isolated from the systemic forms of the disease. We used pathology databases from our institution and an English-language literature search (PubMed) to identify affected patients with biopsy-proven GynV. Using a standardized format for data gathering and analysis, we recorded clinical manifestations, laboratory and histologic features, and surgical and medical therapies. Patients were analyzed as 2 subsets: IGynV and SGynV.A total of 163 patients with GynV were included (152 from the literature and 11 from the Cleveland Clinic pathology database). The incidence of vasculitis among all gynecologic surgeries in our institution over 16 years was 0.15%. Half of the patients presented with vaginal bleeding. Other less common presentations included the finding of an asymptomatic abdominal mass, uterine prolapse, atypical cervical smear, and pelvic pain. Constitutional and musculoskeletal symptoms were reported in 24% of patients. One hundred fifteen (70.6%) patients had IGynV, and 48 (29.4%) had SGynV. Compared to patients with SGynV, those with IGynV were younger (median age, 51 yr; range, 18-80 yr vs. median, 68 yr; range, 32-83 yr; p = 0.0001) and presented more often with vaginal bleeding (57% vs. 25%; p = 0.0002) and less frequently with asymptomatic pelvic masses (6% vs. 35%; p = 0.0001). IGynV was less often associated with constitutional or musculoskeletal symptoms (7% vs. 74%; p = 0.0001). Patients with IGynV were much less likely to have abnormal erythrocyte sedimentation rates (26% vs. 97%; p = 0.0001) and anemia (17% vs. 80%; p = 0.0001) than patients with SGynV. None of the patients with IGynV received corticosteroids, whereas almost all patients with SGynV received corticosteroids and about one-third also received cytotoxic therapy. In IGynV, the site most often involved was the uterus, particularly the cervix, whereas in SGynV lesions were more often multifocal, affecting mainly ovaries, fallopian tubes, and myometrium. Nongranulomatous inflammation occurred in most patients with IGynV, while the predominant histologic pattern noted in SGynV was granulomatous.While vasculitis was the only lesion in 32% of the resected specimens, leiomyomas (18.4%) and endometrial carcinoma (8.3%) were the most frequent concomitant benign and malignant (nonvasculitic) lesions, respectively. Except for benign ovarian abnormalities, which were more frequent in SGynV than in IGynV (21% vs. 4%; p = 0.001), other benign (50%) and malignant (18%) conditions were similarly present in both groups. Among SGynV patients, giant cell arteritis was diagnosed in 29 of the 48 (60.4%) patients, and one-third presented without symptoms of vascular involvement or polymyalgia rheumatica. In summary, GynV is rare and most often occurs as a single-organ disease. It is usually an incidental finding in the course of surgery. The isolated form is associated with the absence of systemic symptoms and normal acute phase reactants, and does not require systemic therapy. Among systemic vasculitides, giant cell arteritis is the most frequently reported form of systemic vasculitis with gynecologic involvement.
    Medicine 06/2009; 88(3):169-81. DOI:10.1097/MD.0b013e3181a577f3 · 5.72 Impact Factor
Show more