Showing papers on "Fibrinoid necrosis published in 2021"

Diagnosis and management of leukocytoclastic vasculitis

Abstract: Leukocytoclastic vasculitis (LCV) is a histopathologic description of a common form of small vessel vasculitis (SVV), that can be found in various types of vasculitis affecting the skin and internal organs. The leading clinical presentation of LCV is palpable purpura and the diagnosis relies on histopathological examination, in which the inflammatory infiltrate is composed of neutrophils with fibrinoid necrosis and disintegration of nuclei into fragments (“leukocytoclasia”). Several medications can cause LCV, as well as infections, or malignancy. Among systemic diseases, the most frequently associated with LCV are ANCA-associated vasculitides, connective tissue diseases, cryoglobulinemic vasculitis, IgA vasculitis (formerly known as Henoch–Schonlein purpura) and hypocomplementemic urticarial vasculitis (HUV). When LCV is suspected, an extensive workout is usually necessary to determine whether the process is skin-limited, or expression of a systemic vasculitis or disease. A comprehensive history and detailed physical examination must be performed; platelet count, renal function and urinalysis, serological tests for hepatitis B and C viruses, autoantibodies (anti-nuclear antibodies and anti-neutrophil cytoplasmic antibodies), complement fractions and IgA staining in biopsy specimens are part of the usual workout of LCV. The treatment is mainly focused on symptom management, based on rest (avoiding standing or walking), low dose corticosteroids, colchicine or different unproven therapies, if skin-limited. When a medication is the cause, the prognosis is favorable and the discontinuation of the culprit drug is usually resolutive. Conversely, when a systemic vasculitis is the cause of LCV, higher doses of corticosteroids or immunosuppressive agents are required, according to the severity of organ involvement and the underlying associated disease.

Temporal Arteritis Revealing Antineutrophil Cytoplasmic Antibody-Associated Vasculitides: A Case-Control Study.

Abstract: OBJECTIVE Temporal arteritis (TA) is a typical manifestation of giant cell arteritis (GCA). Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are rarely revealed by TA manifestations, leading to a risk of misdiagnosis of GCA and inappropriate treatments. This study was undertaken to describe the clinical, biologic, and histologic presentations and outcomes in cases of TA revealing AAV (TA-AAV) compared to controls with classic GCA. METHODS In this retrospective case-control study, the characteristics of patients with TA-AAV were compared to those of control subjects with classic GCA. Log-rank test, with hazard ratios (HRs) and 95% confidence intervals (95% CIs), was used to assess the risk of treatment failure. RESULTS Fifty patients with TA-AAV (median age 70 years) were included. Thirty-three patients (66%) presented with atypical symptoms of GCA (ear, nose, and throat involvement in 32% of patients, and renal, pulmonary, and neurologic involvement in 26%, 20%, and 16% of patients, respectively). Blood samples were screened for ANCAs at the time of disease onset in 33 patients, and results were positive in 88%, leading to a diagnosis of early TA-AAV in 20 patients. The diagnosis of AAV was delayed a median interval of 15 months in 30 patients. Compared to controls with GCA, patients with TA-AAV were younger (median age 70 years versus 74 years), were more frequently men (48% versus 30%), and had high frequencies of atypical manifestations and higher C-reactive protein levels (median 10.8 mg/dl versus 7.0 mg/dl). In patients with TA-AAV, temporal artery biopsy (TAB) showed fibrinoid necrosis and small branch vasculitis in 23% of patients each, whereas neither of these characteristics was evident in controls with GCA. Treatment failure-free survival was comparable between early TA-AAV cases and GCA controls, whereas those with delayed TA-AAV had a significantly higher risk of treatment failure compared to controls (HR 3.85, 95% CI 1.97-7.51; P < 0.0001). CONCLUSION TA-AAV should be considered diagnostically in cases of atypical manifestations of GCA, refractoriness to glucocorticoid treatment, or early relapse. Analysis of TAB specimens for the detection of small branch vasculitis and/or fibrinoid necrosis could be useful. Detection of ANCAs should be performed in cases of suspected GCA with atypical clinical features and/or evidence of temporal artery abnormalities on TAB.

Acute necrotizing glomerulonephritis associated with COVID-19 infection: report of two pediatric cases.

Abstract: Coronavirus disease 2019 (COVID-19) is thought to cause kidney injury via a variety of mechanisms The most common reported kidney injury following COVID-19 infection is acute tubular injury (ATI); however, the procoagulant state induced by the virus may also damage the kidneys Herein, we report two cases of acute necrotizing glomerulonephritis (GN) with fibrinoid necrosis in the context of COVID-19 infection The one with more chronic features in the kidney biopsy progressed to permanent kidney failure but the second one had an excellent response to glucocorticoid pulse therapy with subsequent normal kidney function at 2-month follow-up Both reported cases had an acute presentation of kidney injury with positive nasopharyngeal PCR test for COVID-19 Based on the data review by the researchers, this is the first report of acute necrotizing GN associated with COVID-19 infection

Acute Endotheliitis (Type 3 Hypersensitivity Vasculitis) in Ten COVID-19 Autopsy Brains

Abstract: Central nervous system (CNS) involvement in COVID-19 may occur through direct SARS-CoV-2 invasion through peripheral or cranial nerves or through vascular endothelial cell infection. The renin-angiotensin system may play a major part in CNS morbidity. Effects of hypoxia have also been implicated in CNS lesions in COVID-19. This communication reports on ten consecutive autopsies of individuals with death due to COVID-19 with decedent survival ranging from 30 minutes to 84 days after admission. All ten brains examined had neutrophilic microvascular endotheliitis present in variable amounts and variably distributed. Importantly, this acute stage of type 3 hypersensitivity vasculitis can be followed by fibrinoid necrosis and inner vascular wall sclerosis, but these later stages were not found. These results suggest that a vasculitis with autoimmune features occurred in all ten patients. It is possible that viral antigen in or on microvascular walls or other antigen-antibody complexes occurred in all ten patients proximate to death as a form of autoimmune vasculitis.

Gestational diabetes mellitus induces placental vasculopathies

Abstract: Gestational diabetes mellitus (GDM) poses significant long- and short-term risks to both the developing fetus and the mother. GDM can lead to maternal complications during pregnancy and increase the mother's risk of developing type 2 diabetes mellitus and cardiovascular disease later. The present study aimed to evaluate the maternal and fetal vasculopathies in the placenta of Saudi women with GDM. This prospective study examined 84 placentas from full-term pregnant women with no complications other than GDM; 40 placentas were collected from healthy women (controls), and 44 were collected from women diagnosed with GDM. The sampling took place in King Saud University Medical City, Riyadh, between January and August 2019. All placentas were histologically examined according to the Amsterdam Placental Workshop Group (2014, 2015). The results showed that the most common placental changes on the maternal side of the placenta in the GDM group were significant syncytial knots (77%), calcification (70%), villous agglutination (57%), decidual vasculopathy (43%), and retroplacental hemorrhage (34%). Placental infarction was the least common placental change in both groups. On the fetal side, vasculopathies included significant villous fibrinoid necrosis (70.5%), chorangiosis (50%), fibromuscular sclerosis (50%), and villous edema (38.6%). Significant villous fibrinoid necrosis, villous edema, and significant fibromuscular sclerosis were more prevalent in the GDM group. The present study concluded that gestational diabetes mellitus induces histopathological phenotypes in the full-term placenta. Increased decidual vasculopathy, syncytial knots, retroplacental hemorrhage, classification, villous agglutination, chorangiosis, villous edema, villous fibroid necrosis, and fibromuscular sclerosis may indicate GDM in the mother. Such findings in the placenta of a woman who has not been diagnosed with GDM increase the need for GDM examination in future pregnancies.

Cell Liquefactive Necrosis

Abstract: Cell Necrosis Irreversible injury to cells as a result of encounters with noxious stimuli invariably leads to cell death. Such noxious stimuli include infectious agents (bacteria, viruses, fungi, parasites), oxygen deprivation or hypoxia, and extreme environmental conditions such as heat, radiation, or exposure to ultraviolet irradiation. The resulting death is known as necrosis, a term that is usually distinguished from the other major consequence of irreversible injury, known as cell death by apoptosis. Apoptosis is a programmed or organized cell death which could be physiological or pathological. Additional information regarding this form of cell death is outside of the scope of this chapter. Necrosis as a form of cell death is almost always associated with a pathological process.When cells die by necrosis, they exhibit two major types of microscopes or macroscopic appearance. The first is liquefactive necrosis, also known as colliquative necrosis, is characterized by partial or complete dissolution of dead tissue and transformation into a liquid, viscous mass. The loss of tissue and cellular profile occurs within hours in liquefactive necrosis. In contrast to liquefactive necrosis, coagulative necrosis, the other major pattern, is characterized by the maintenance of normal architecture of necrotic tissue for several days after cell death.Liquefaction derives from the slimy, liquid-like nature of tissues undergoing liquefactive necrosis. This morphological appearance is attributable in part to the activities of hydrolytic enzymes which causes dissolution of cellular organelles in a cell undergoing necrosis. The enzymes responsible for liquefaction are derived from either bacterial hydrolytic enzymes or lysosomal hydrolytic enzymes. Other types of Necrosis In addition to liquefactive and coagulative necrosis, the other morphological patterns associated with cell death by necrosis are: Caseous Necrosis Fat Necrosis Gangrenous Necrosis Fibrinoid necrosis The other types of necrosis listed above do not represent distinct pathological entities. Rather, they are descriptive terms that are widely used to describe necrosis occurring in specific clinical scenarios or organ damage. Coagulative This is the default pattern of necrosis associated with ischemia or hypoxia in every organ in the body except the brain. Gross Appearance: tissue is firm and architecture is maintained for days after cell death. Microscopic: Preserved cell outlines without nuclei. Liquefactive The pattern of necrosis seen with infections. Also, the pattern is seen following ischemic injury in the brain. While the reason for liquefactive necrosis following ischemic injury in the brain is poorly understood, the release of digestive enzymes and constituents of neutrophils is the reason for liquefaction in infections. Gross Appearance: The tissue is in a liquid form and sometimes creamy yellow because of pus formation. Microscopic: Inflammatory cells with numerous neutrophils. Caseous A unique type of cell death seen with tuberculosis. Gross Appearance: White, soft, cheesy-looking (caseating) material Microscopic: A uniformly eosinophilic center (necrosis) surrounded by a collar of lymphocytes and activated macrophages (giant cells, epithelioid cells). The entire structure formed in response to tuberculosis is known as a granuloma. Fat Necrosis Fat necrosis occurs from acute inflammation affecting tissues with numerous adipocytes such as pancreas and breast tissue. Damaged cells release digestive enzymes which break down lipids to generate free fatty acids. Gross Appearance: Whitish deposits as a result of the formation of calcium soaps. Microscopic: Anucleated adipocytes with deposits of calcium (Seen on HE and liquefactive necrosis (wet gangrene) if a bacterial infection is superimposed. These all represents morphological patterns which are visible grossly and microscopically. Fibrinoid necrosis is usually visible only microscopically. We discuss the characteristic gross and microscopic findings in liquefactive necrosis in subsequent paragraphs.

Histopathology of chronic spontaneous urticaria with occasional bruising lesions is not significantly different from urticaria with typical wheals.

Abstract: Background Chronic spontaneous urticaria (CSU) may occasionally exhibit long-lasting lesions with bruising, usually considered a hallmark of urticarial vasculitis (UV). Histopathology of these chronic urticarial lesions has not been extensively studied. Methods Skin biopsies from patients with anti-H1 resistant CSU were evaluated for several parameters (edema, location, intensity, and cell composition of the inflammatory infiltrate, and abnormalities in the blood vessels). Results We studied 45 patients (37 female/8 male, mean age 49.3 years) with CSU, 60% of whom with occasional bruising lesions and 3 patients with hypocomplementemic UV. Histopathology in CSU showed mainly perivascular and interstitial inflammatory infiltrate (91.1%), including eosinophils (80%), neutrophils (77.8%), and lymphocytes (71.1%), vasodilatation (88.9%), intravascular neutrophils (95.6%), dermal edema (51.1%), swelling of endothelial cells (51.1%), and minor and rare fibrinoid necrosis and karyorrhexis (6.7%). Significant karyorrhexis and frank fibrinoid necrosis were observed, respectively, in two and three cases of UV. In patients with occasional bruising, mast cells occurred in fewer cases whereas eosinophils were more frequent, but no statistically significant difference was found for other parameters. Conclusions Histopathological findings were not significantly different between CSU with or without bruising lesions. Bruising may be associated with more severe forms of CSU with no histopathological signature, although UV cannot be completely excluded based on histopathology.

Case Report: Probable Cerebral Amyloid Angiopathy-Related Inflammation During Bevacizumab Treatment for Metastatic Cervical Cancer.

Abstract: Bevacizumab is an anti-angiogenic monoclonal antibody targeting Vascular Endothelial Growth Factor (VEGF) that induces the proliferation and migration of vascular endothelial cells thus, promoting vasculogenesis. Bevacizumab inhibits cancer angiogenesis, which is fundamental for either tumor development, exponential growth, or metastatic spread by supplying nutrients and oxygen. We report a new possible adverse event of bevacizumab, a Cerebral Amyloid Angiopathy-Related Inflammation (CAARI), in a 72-year-old woman with metastatic cervical cancer. After six cycles every three weeks of chemotherapy (cisplatin, paclitaxel, bevacizumab) and following two maintenance bevacizumab administrations, the patient presented a worsening confusional state. The MRI scan showed bilateral asymmetric temporo-parieto-occipital hyperintensity with numerous cortical microbleeds indicative of a CAARI. After stopping bevacizumab treatment, steroid therapy was administered resulting in rapid clinical improvement. The subsequent neurological and oncological follow-up was negative for recurrence. The patient was a heterozygote carrier for apolipoprotein-E e4 that increases the risk of sporadic Cerebral Amyloid Angiopathy (CAA), which is characterized by beta-amyloid accumulation and fibrinoid necrosis in cerebral vasculature leading to micro/macrohemorrhages and dementia. Moreover, CAA is present in 30% of people aged over 60 years without dementia. In the brains of CAA patients, there is a proinflammatory state with cerebrovascular endothelial cell alteration and elevated levels of either adhesion molecules or inflammatory interleukins that increase the blood-brain barrier permeability. Moreover, CAARI is an inflammatory form of CAA. Inhibition of VEGF, which has anti-apoptotic, anti-inflammatory, and pro-survival effects on endothelial cells, impairs their regenerative capacity and increases expression of proinflammatory genes leading to weakened supporting layers of blood vessels and, hence, to damaged vascular integrity. In our patient, bevacizumab administration may have further increased permeability of cerebral microvasculature likely impaired by an underlying, asymptomatic CAA. To our knowledge, this is the first case reporting on the development of probable CAARI during bevacizumab treatment, which should alert the clinicians in case of neurological symptom onset in older patients under anti-angiogenic therapy.

Histopathological Changes In Placentas Due To Pregnancy-Induced Hypertension And Gestational Diabetes Compared With Normal Term Placenta

Abstract: Hypertension is the most commonly known problem of restoration during pregnancy, with up to 10% of pregnancies confused. Gestational diabetes mellitus is described as the occurrence or for the first time in pregnancy of a change in glucose levels to varying degrees. There are medical problems when diabetics and hypertension worsen pregnancy and affect maternal health. Objectives: Analysis of unexplained placenta changes in patients with concomitant hypertension and gestational diabetes caused by pregnancy. Materials and Methods: This study included forty placentas, twenty were collected from uncomplicated normotensive pregnant ladies, and the rest (20 placentas) were collected from ladies with concomitant gestational diabetes and pregnancy-induced hypertension. Histological sections were prepared using routine haemotoxyline and eosin staining. Results: The histomorphological study of placenta of patients with concomitant hypertension and diabetes mellitus showed a significant number of syncytial knots, stromal fibrosis, and the number of capillaries in terminal villi in Medium-sized diffusion areas of the vascular median cover. Conclusion: Placenta examination is very important for the diagnosis of various pathological conditions, mechanisms are still far from well understood, but there is a common consensus that the pathological level depends on the type of diabetes and hypertension during pregnancy. This study provides an opinion of previous studies with a great association placental changes in patients with concomitant pregnancy-induced hypertension and diabetes. The clinical appearance and the magnitude of placental pathological changes were strongly associated. There is a wide range of microscopical changes noted as increased the numbers of the syncytial ganglia, fibrosis and the number of capillaries in the peripheral appendages

Rare Initial Manifestation of Lupus as Lobular Panniculitis of the Breast-A Case Report and Review of the Literature.

Abstract: Lupus mastitis is a rare complication of systemic or discoid lupus erythematosus with an uncommon initial presentation when limited to the breast. In this article, we report a 42-year-old woman who presented with constant pain and tenderness in her breasts. Ultrasound imaging of the left breast revealed a 14-mm oval mass, suspicious for malignancy; a needle core biopsy was performed. Sections showed necrosis of the fat lobules with associated mixed nodular lymphoplasmacytic aggregates. Karyorrhectic debris, fibrinoid necrosis of small vessels, and microcalcifications were all present while the background breast parenchyma was unremarkable. The diagnosis of lupus mastitis was rendered. Subsequent serology showed negative dsDNA but positive antinuclear antibodies, C4, and anti -Sjogren';s-syndrome-related antigen A antibodies. Clinical features of active systemic disease were not identified at the patient's follow-up dermatology appointment 1 month after the biopsy, and she elected management for her nodules with steroids. To the best of our knowledge, only 40 other cases of lupus mastitis have been reported in the English literature, of which 25 presented as a mass and only in 6 lupus mastitis of the breast was the initial presentation. In conclusion, we bring much needed awareness to lupus mastitis as the first presentation of disease.

Primary Cutaneous Cryptococcosis due to Cryptococcus neoformans in an Immunocompetent Host Treated with Itraconazole and Drainage: Case Report and Review of the Literature.

Abstract: Cryptococcus neoformans is an opportunistic germ, usually causing infections in immunocompromised patients. The main sources of infection with C. neoformans are excrement from birds, decomposing wood, fruit, and vegetables. Primary cutaneous cryptococcosis (PCC) is a clinical entity, differing from secondary cutaneous cryptococcosis and systematic infection. We report the case of an immunocompetent 60-year-old woman with PCC due to C. neoformans in her right thumb. She reported an accidental injury caused by a rose thorn while she was gardening. Clinical examination showed the presence of an erythematous ulcerated nodule with elevated borders, suppuration, and central necrosis. Skin histology examination showed cutaneous and subcutaneous fibrinoid necrosis with bleeding, abscess, neutrophil-rich cellular infiltration, and the presence of PAS-, Grocott- and mucin-positive spores. The mycological culture showed milky and creamy colonies of C. neoformans after 3 days. As there was no previous history of pulmonary cryptococcosis, we diagnosed PPC. We treated the patient surgically with accurate debridement of nonvital tissues in the right thumb. In addition, we started itraconazole treatment 100 mg twice daily for 6 months, which led to rapid clinical improvement without relapse. PCC is a rare infection that can present with quite unspecific clinical pictures including acneiform lesions, purpura, vesicles, nodules, abscesses, ulcers, granulomas, pustules, draining sinuses, and cellulitis. Prolonged systemic antifungal therapy is necessary in order to get a healing result without relapse. We summarize all the cases of PCC in immunocompetent patients published so far in the literature.

Methimazole-Induced ANCA Vasculitis: A Case Report.

Abstract: Rapidly progressive glomerulonephritis (RPGN) is a syndrome which presents rapid loss of renal function. Vasculitis represents one of the major causes, often related to anti-neutrophil cytoplasmic antibodies (ANCA). Herein, we report a case of methimazole-induced ANCA-associated vasculitis. A 35-year-old woman complained of weight loss and fatigue for 2 weeks and attended the emergency room with alveolar hemorrhage. She had been diagnosed with Graves' disease and had been taking methimazole in the past 6 months. Her physical examination showed pulmonary wheezing, hypertension and signs of respiratory failure. Laboratory tests revealed urea 72 mg/dL, creatinine 2.65 mg/dL (eGFR CKD-EPI: 20 mL/min/1.73 m2), urine analysis with >100 red blood cells per high-power field, 24 h-proteinuria: 1.3 g, hemoglobin 6.6 g/dL, white-cell count 7700/mm3, platelets 238,000/mm3, complement within the normal range, negative viral serological tests and ANCA positive 1:80 myeloperoxidase pattern. Chest tomography showed bilateral and diffuse ground-glass opacities, and bronchial washing confirming alveolar hemorrhage. A renal biopsy using light microscopy identified 27 glomeruli (11 with cellular crescentic lesions), focal disruption in glomerular basement membrane and fibrinoid necrosis areas, tubulitis and mild interstitial fibrosis. Immunofluorescence microscopy showed IgG +2/+3, C3 +3/+3 and Fibrinogen +3/+3 in fibrinoid necrosis sites. She was subsequently diagnosed with crescentic pauci-immune glomerulonephritis, mixed class, in the setting of a methimazole-induced ANCA vasculitis. The patient was treated with methimazole withdrawal and immunosuppressed with steroids and cyclophosphamide. Four years after the initial diagnosis, she is currently being treated with azathioprine, and her exams show creatinine 1.30 mg/dL (eGFR CKD-EPI: 52 mL/min/1.73 m2) and negative p-ANCA.

Amoebic encephalitis mimicking acute disseminated encephalomyelitis.

Abstract: A 16-year-old Japanese girl presented with left hemiparesis and coma, following a 2-day history of headache and fever. She had been previously well and no history of a skin wound. MR scan of the brain showed a 6 cm lesion in the right frontal white matter with mass effect and slight edge enhancement (figure 1). A craniotomy was performed for external decompression and brain biopsy. The intraoperative finding was of white matter necrosis rather than tumour or abscess. An interim biopsy showed prominent neutrophil infiltration and fibrinoid necrosis in small vessels but no evidence of a specific pathogen. We suspected either a central nervous system infection or an autoimmune disease such as acute disseminated encephalomyelitis, and so started antibiotics and pulsed corticosteroids in the intensive care unit. Figure 1 MR scan of brain on admission showing a mass lesion in the right frontal lobe. The fluid-attenuated inversion recovery (FLAIR) image …

The type of interstitial inflammatory cell infiltrate and the severity of glomerular injury; an underestimated morphologic correlation

Abstract: Introduction: The interrelation between the type of interstitial inflammatory cells and the severity of glomerulonephritis was not considered in most of the relevant medical literature. Objectives: To investigate the relationship between the type of interstitial cell infiltrate and the morphological severity of glomerular injury in different types of proliferative glomerulonephritides. Patients and Methods: We retrospectively reviewed 138 native kidney biopsies and assessed the relationship between the type of interstitial inflammatory cell infiltrate and the severity of glomerular injury in the form of cellular crescents and fibrinoid necrosis. Results: The predominant type of interstitial inflammatory cell infiltrate was lymphocytic, noted in more than half of the cases. Lymphoplasmacytic inflammatory cell infiltrate was the second most common type which observed. Fifty-five of patients had inflammation in areas of fibrosis. Cellular/fibrocellular crescents were observed in 44% of cases, and fibrinoid necrosis in 30% of cases. As compared to the ‘lymphocytic’ group, patients in the ‘lymphoplasmacytic’ group had ~3 times higher probability of presenting with crescents and fibrinoid necrosis. Conclusion: Our study highlights the significance of morphological correlations that may predict the severity of glomerular injury. Such findings would be helpful in limited or inadequate renal biopsy samples where the pathologist can alert the clinician, in the appropriate clinical context, to the possibility of having crescents and/or necrotizing lesions in the unsampled glomeruli.

Multiple myeloma characterized by synovial fibrinoid necrosis: a case report.

Abstract: A number of patients with multiple myeloma (MM) have joint lesions with the main feature of amyloidosis or tumor cell infiltration. We report a case of MM that presented as synovial fibrinoid necrosis. The rarity of this condition and the difficulty diagnosing the disease are discussed. In addition, we discussed the characteristics of amyloid arthropathy and the findings in this case.

[Joint tumors: rare but important differential diagnoses of malignant and benign tumors as well as pseudotumors in rheumatology].

Abstract: This review article elucidates the differential diagnostics of malignant and benign joint tumors, pseudotumors of the joints and the peri-implant tissue, which are rare but important entities in rheumatology and orthopedic rheumatology. The tissue of origin includes the synovium, peri-implant tissue, peri-articular fibrous tissue and peri-articular osseous tissue. Pseudotumors can be viewed as independent but heterogeneous entities. These are essentially manifested as tumor-like depositions of crystals, calcareous deposits, vascular malformations, ectasia of the synovia and joint capsule tissue and pseudocysts. Other causes for pseudotumors are focal destructive inflammation (e.g. induced by foreign bodies), high grade synovitis and focal fibrinoid necrosis (i.e. rheumatoid nodules). Methodologically, these diagnostics are based on conventional standard staining methods, immunohistochemical analyses of formalin-fixed and paraffin-embedded materials and on molecular diagnostic procedures. The latter are of great importance in cases of benign and malignant joint tumors. The most important immunohistochemical markers with respect to joint tumors are S100, SM-actin, CD68, CD34, STAT6, clusterin, Muc‑4, beta-catenin and MDM2-FISH. The following markers are recommended for the differential diagnostics and typing of periarticular tumor metastases in the pathology of rheumatic diseases: AE1/AE3, CK8, p63, TTF‑1, TGB, PSA, androgen receptor, GATA, CD56, chromogranin, CDX‑2, SAT-B2, SALL4, estrogen and progesterone receptors, CD45LCA, CD30, CD79a and S100. Necrosis, inflammatory infiltrations and reparative inflammatory changes may complicate the histopathological classification. Therefore, a correlation with clinical, microbiological and radiological data in the sense of interdisciplinary synergistic diagnostics may be required.

The effect of immunosuppressive therapy in patients with fibrinoid necrosis lesions in a large cohort of patients with IgA nephropathy.

Abstract: Background Fibrinoid necrosis is considered one of the active pathological lesions in IgA nephropathy. Whether patients with IgA nephropathy with fibrinoid necrosis lesions benefit from immunosuppressive therapy in terms of long-term outcomes remains uncertain. This study aimed to evaluate the response to immunosuppressive therapy in patients with fibrinoid necrosis lesions in a large cohort of patients with IgA nephropathy. Methods A total of 1325 patients with kidney biopsy-proven IgA nephropathy from 1994 to 2016 were recruited from the Peking University First Hospital IgA Nephropathy Database. The clinicopathological characteristics of patients with fibrinoid necrosis lesions and the effect of immunosuppressive therapy on patients with fibrinoid necrosis lesions alone or in those with fibrinoid necrosis together with crescents or endocapillary hypercellularity lesions were analyzed. Results In total, 107/1325 (8.1%) patients showed fibrinoid necrosis lesions, and 92/107 (86.0%) of these patients showed fibrinoid necrosis associated either with cellular/fibrocellular crescents or endocapillary hypercellularity lesions. The presence of fibrinoid necrosis together with crescents or endocapillary hypercellularity was an independent risk factor for the kidney composite endpoint (HR, 2.11; 95% CI, 1.16-3.84; P = 0.02) in patients without immunosuppression, while for those receiving immunosuppressive therapy, kidney outcome was improved (HR, 0.80; 95% CI, 0.46-1.39; P = 0.42). However, the predictive value of fibrinoid necrosis lesions alone did not change significantly between patients with and without immunosuppressive therapy. Conclusions The presence of fibrinoid necrosis with crescents or endocapillary hypercellularity lesions together, but not fibrinoid necrosis lesions alone, was a pathological indicator of patients who may benefit from immunosuppressive therapy.

Síndrome constitucional y anemia como debut de vasculitis en un adulto mayor. Reporte de caso

Abstract: Introduction: Vasculitis comprises a group of often serious diseases that have an unspecific onset and a late diagnosis. The following report describes a case of vasculitis that may lead to considering this disorder as a differential diagnosis from the beginning of the care process to ensure a comprehensive approach and early treatment initiation that reduce associated morbidity and mortality and improve the success rate of treatments. Case presentation: A 77-year-old female, with a 2-year history of arterial hypertension was admitted to the emergency department of a secondary care center for having experienced symptoms of asthenia, fever, hyporexia and weight loss for a month. The patient was hospitalized for further testing and, given the findings, a possible bacterial translocation secondary to intestinal neoplasm was suspected. Empirical antibiotic treatment was started, but her condition continued to worsen. Complementary tests were performed, although they were not conclusive. Due to the persistence of fever, kidney failure and anemia, a kidney biopsy was performed, revealing arterial vessel with fibrinoid necrosis and associated polymorphonuclear infiltrates, clear signs of an active vasculitis of the microscopic polyangiitis type. Several lines of treatment were used, but the patient evolved unfavorably and died. Conclusions: The presentation of this unusual case intends to contribute to the early diagnosis of this disorder by making medical staff aware of the possibility of considering it when symptoms suggest other diseases, or even when nonspecific symptoms such as anemia and weight loss occur.

Bartonella quintana Infection Manifesting as Leucocytoclastic Vasculitis Rash.

Abstract: We present the first case described in the literature of leucocytoclastic vasculitis due to Bartonella quintana infection. A 73-year-old woman presented to the hospital with persistent fevers, retro-orbital headache, generalized weakness, and left lower thigh pain for 1 week. She was found to have truncal and proximal lower extremity papules and small plaques. Serology revealed Bartonella quintana immunoglobulin M (IgM) titer of 1:256 with undetectable Bartonella quintana immunoglobulin G (IgG) and undetectable Bartonella henselae IgG and IgM. Skin biopsy of an abdominal lesion revealed fibrinoid necrosis of vessel walls in the superficial and mid-dermis consistent with leucocytoclastic vasculitis. Doxycycline 100 mg orally twice daily was initiated, after which she had defervescence within 36 hours and rapid improvement of other presenting symptoms.

Isolated intestinal polyarteritis nodosa in an elderly patient.

Abstract: Polyarteritis nodosa (PAN) is a necrotising systemic vasculitis involving medium-sized and small-sized vessels. PAN limited to a single organ is rare, particularly in the elderly population. Herein, we present a 73-year-old-woman who developed severe abdominal pain. Mesenteric angiography showed multifocal areas of segmental dilation and narrowing of the superior mesenteric, ileocolic and right colonic arteries. Exploratory laparotomy revealed multiple areas of necrosis of the jejunum for which resection was performed. Histopathological exam disclosed mesenteric vasculitis with fibrinoid necrosis of the arterial wall with leucocytic infiltrates and haemorrhages consistent with PAN. She was started on high-dose corticosteroids with an initial good response. However, 6 months later, she developed intestinal pseudo-obstruction for which oral cyclophosphamide was started. After 5 months of cyclophosphamide therapy, she remained stable without further relapses. Our case suggests that PAN should be considered in elderly patients presenting with abdominal pain even in the absence of systemic involvement.

Renal Involvement in Mediterranean Spotted Fever: Clinical and Histopathological Data.

Abstract: OBJECTIVE Mediterranean spotted fever (MSF) is a tick-borne rickettsial infection endemic to the Mediterranean coastline countries. As a result of growing tourism, imported cases have been registered in many nonendemic countries and regions. We present clinical laboratory parameters and histopathological data on renal impairment in patients with MSF. The study meets our goal of identifying kidney involvement and detecting renal damage in people with MSF. SUBJECTS AND METHODS Three hundred fifty patients with MSF with a diagnosis confirmed by immunofluorescence analysis were tested for serum urea, creatinine, and albumin. Fifty-five patients with malignant form of MSF were divided into 2 groups: 19 fatalities and 36 survivors. The percentage of patients with acute renal failure (ARF) was compared in both groups. RESULTS Subjects with elevated urea and creatinine levels increased from 5.21 to 3.47% in mild to 48.78 and 29.26% in severe MSF, respectively. Loss of serum albumin also increased from mild to severe MSF. Renal impairment comprised 60% of the cohort of 55 patients with malignant MSF: 89.4% in the group of deaths and almost twice less in the survivors. ARF developed in 84.2% of fatal cases and was >2 times less in survivors. Postmortem light microscopy of renal samples of 9 fatal cases revealed perivascular mononuclear inflammatory infiltrates, vasculitis with fibrinoid necrosis, acute tubular necrosis, interstitial edema, hemorrhage, and thrombosis. CONCLUSION Renal pathology associated with MSF rickettsial infection consists of systemic small vessel vasculitis and vascular injury, leading to ARF in the most severe cases.

Idiopathic eosinophilic pleurisy: A practical diagnostic approach.

Abstract: A 37-year-old man with fever, cough, and dyspnea with no medical history developed an eosinophilic pleural effusion and blood eosinophilia. No evidence of malignancy or pathogens was detected in the pleural effusion, and the pleural specimen obtained by thoracoscopy showed eosinophilic infiltration with inflammatory granulation tissue without fibrinoid necrosis or malignant cells. Since a myeloproliferative disorder was also excluded, the diagnosis was idiopathic eosinophilic pleurisy. Corticosteroid treatment was started and then slowly tapered, and the eosinophilic pleural effusion resolved. Considering the various etiologies of eosinophilic pleurisy, a practical clinical approach to the investigation and diagnosis of eosinophilic pleurisy is presented.