fibrin and Vasculitis

Topics: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Arteries; Capillaries; Endothelium, Vascular; Fibrin; Humans; Kidney Glomerulus; Necrosis; Neutrophils; Peripheral Nerves; Thrombosis; Vasculitis; Veins

Topics: Cell Adhesion Molecules; Fibrin; Humans; Iron; Iron Overload; Macrophages; Matrix Metalloproteinases; Multiple Sclerosis; Vasculitis

The salient features of systemic vasculitis are endothelial swelling, inflammatory infiltrates, and fibrinoid necrosis of the arterial wall. Of these three, the concept of fibrinoid necrosis is undoubtedly the most elusive. Is it really necrosis, defined as unprogrammed cell death, that we are looking at? And does the adjective 'fibrinoid', meaning fibrin-like, cover its most important attribute? In early case reports on systemic vasculitis the term was used with caution, but over the years it has grown in status to become the most characteristic histopathological manifestation of systemic vasculitis in patients with anti-neutrophil cytoplasmic antibodies (ANCA), suggesting that the clue to the auto-immune mechanisms that damage the vessel wall lies in the necrotic lesion. But what is this assumption based on? This review discusses the history of fibrinoid necrosis in vasculitis, focusing on the ideas that have been postulated over the years regarding this lesion. Special attention will be paid to its occurrence in the kidney in systemic vasculitis.

Topics: Antibodies, Antineutrophil Cytoplasmic; Arteries; Autoimmunity; Fibrin; Humans; Kidney; Necrosis; Vasculitis

Pulmonary vascular inflammatory disorders may involve all components of the pulmonary vasculature, including capillaries. The principal histopathologic features of pulmonary capillaritis include capillary wall necrosis with infiltration by neutrophils, interstitial erythrocytes, and/or hemosiderin, and interalveolar septal capillary occlusion by fibrin thrombi. Immune complex deposition is variably present. Patients often present clinically with diffuse alveolar hemorrhage, which is characterized by dyspnea and hemoptysis; diffuse, bilateral, alveolar infiltrates on chest radiograph; and anemia. Pulmonary capillaritis has been reported with variable frequency and severity as a manifestation of Wegener's granulomatosis, microscopic polyarteritis, systemic lupus erythematosus, Goodpasture's syndrome, idiopathic pulmonary renal syndrome, Behçet's syndrome, Henoch-Schönlein purpura, IgA nephropathy, antiphospholipid syndrome, progressive systemic sclerosis, and diphenylhydantoin use. In addition to history, physical examination, and routine laboratory studies, certain ancillary laboratory tests, such as antineutrophil cytoplasmic antibodies, antinuclear antibodies, and antiglomerular basement membrane antibodies, may help diagnose an underlying disease. Diagnosis of pulmonary capillaritis can be made by fiberoptic bronchoscopy with transbronchial biopsy, but thoracoscopic biopsy is often employed. Since many disorders can result in pulmonary capillaritis with diffuse alveolar hemorrhage, it is crucial for clinicians and pathologists to work together when attempting to identify an underlying disease. Therapy depends on the disorder that gave rise to the pulmonary capillaritis and usually includes corticosteroids and cyclophosphamide or azathioprine. Since most diseases that result in pulmonary capillaritis are treated with immunosuppression, infection must be excluded aggressively.

Topics: Anemia; Bronchoscopy; Capillaries; Diagnosis, Differential; Dyspnea; Erythrocytes; Fibrin; Hemoptysis; Hemorrhage; Hemosiderin; Humans; Immunosuppressive Agents; Lung; Lung Diseases; Necrosis; Neutrophils; Pulmonary Alveoli; Pulmonary Embolism; Thoracoscopy; Vasculitis

Normocomplementemic urticarial vasculitis is a rare autoimmune disorder characterized by leukocytoclasia, fibrin deposits, and extravasated erythrocytes affecting multiple organ systems. Current treatment modalities, including corticosteroids and immunosuppressive agents, are of limited efficacy and an expansive side effect profile. Omalizumab has been reported to be effective in urticarial vasculitis, but its long-term effectiveness and tolerability have not yet been evaluated. We report a case of long-standing normocomplementemic urticarial vasculitis treated with omalizumab only, for almost 3 years. The patient reported a significant improvement in quality of life after the first few doses with a significant improvement in the urticaria control test. The treatment was well tolerated and no adverse events were reported after 3 years. Our patient was treated with 300 mg of omalizumab, as it was previously linked with a better improvement in quality of life. We were able to extend our patient’s treatment intervals, suggesting that this is feasible in patients treated with omalizumab who achieve a complete response. We recommend that larger and long-term studies are conducted to assess the efficacy and effectiveness of omalizumab in patients with urticarial vasculitis. J Drugs Dermatol. 2022;21(10):1124-1126. doi:10.36849/JDD.6739.

Topics: Adrenal Cortex Hormones; Fibrin; Humans; Immunosuppressive Agents; Omalizumab; Quality of Life; Urticaria; Vasculitis

Pre-existing comorbidities such as obesity or metabolic diseases can adversely affect the clinical outcome of COVID-19. Chronic metabolic disorders are globally on the rise and often a consequence of an unhealthy diet, referred to as a Western Diet. For the first time in the Syrian hamster model, we demonstrate the detrimental impact of a continuous high-fat high-sugar diet on COVID-19 outcome. We observed increased weight loss and lung pathology, such as exudate, vasculitis, hemorrhage, fibrin, and edema, delayed viral clearance and functional lung recovery, and prolonged viral shedding. This was accompanied by an altered, but not significantly different, systemic IL-10 and IL-6 profile, as well as a dysregulated serum lipid response dominated by polyunsaturated fatty acid-containing phosphatidylethanolamine, partially recapitulating cytokine and lipid responses associated with severe human COVID-19. Our data support the hamster model for testing restrictive or targeted diets and immunomodulatory therapies to mediate the adverse effects of metabolic disease on COVID-19.

Topics: Animals; COVID-19; Cricetinae; Cytokines; Diet, High-Fat; Dietary Carbohydrates; Disease Models, Animal; Edema; Fibrin; Hemorrhage; Humans; Interleukin-10; Interleukin-6; Lipid Metabolism; Lipidomics; Lipids; Liver; Lung; Male; Mesocricetus; Obesity; SARS-CoV-2; Severity of Illness Index; Sugars; Vasculitis; Virus Shedding

The accumulation of immunoreactants and fibrinoid necrosis of postcapillary vessel walls are common pathological features of cutaneous immune complex vasculitis. In more advanced lesions, these immunoreactants are subject to proteolysis. Mast cell chymase is a powerful enzyme that can degrade several substrates including the extracellular matrix. Heparin can influence the catalytic properties of chymase.. To study the effects of recombinant human (rh) chymase on fibrinogen, coagulation and fibrinolysis, and to relate these effects to the pathogenesis of vasculitis.. The colocalization of chymase and fibrin in vasculitis specimens was analysed by immunohistochemical double staining. Fibrinogen and fibrin were treated with rh-chymase and the effects were studied in vitro by sodium dodecylsulfate polyacrylamide gel electrophoresis and a variety of clotting and fibrin gel experiments. The effects of rh-chymase on vasculitis cryosections were analysed by direct immunofluorescence.. Chymase-positive mast cells were associated with fibrin-positive vessels in vasculitis cryosections. Rh-chymase degraded the alpha-, beta- and gamma-chains of fibrinogen, while heparin enhanced the degradation of the beta-chain. Rh-chymase pretreatment of fibrinogen prolonged thrombin-induced clotting time. Fibrinogen degradation products induced by rh-chymase increased the clotting time of human plasma. Rh-chymase degraded fibrin gel prepared from fibrinogen or human plasma. Immunofluorescence staining positivity of fibrin in vasculitis cryosections decreased after pretreatment with rh-chymase for 24 h, and heparin enhanced this effect.. Mast cell chymase may constitute a previously unrecognized endogenous anticoagulant and fibrinolytic enzyme, and may be involved in the clearance of fibrin from vessel walls in aged vasculitis lesions.

Topics: Blood Vessels; Chymases; Enzyme Assays; Fibrin; Fibrin Fibrinogen Degradation Products; Fibrinogen; Humans; Immunohistochemistry; Mast Cells; Proteolysis; Recombinant Proteins; Skin; Vasculitis

Cutaneous infection with the mite Sarcoptes scabiei var. hominis is associated with epidermal and dermal changes. After noting superficial fibrin thrombi in two biopsies with scabies mites, we comprehensively reviewed the histopathologic findings in scabietic infections to determine the frequency of this finding.. Twenty five biopsies of scabies infection were retrieved from the archives of our institution; only cases containing scabietic mite parts or scybala were included. The microscopic features were documented.. Nearly half (40%) of the cases showed fibrin thrombi within vessels of the superficial dermis. Other frequent findings included dermal eosinophils (88% of cases), epidermal spongiosis (76% of cases), lymphocyte atypia (64%), a superficial and deep infiltrate (52% of cases), dermal neutrophils (52%) and endothelial cell swelling (52%). Half of the cases contained polarizable mite elements. Less commonly encountered features included extravasated erythrocytes (44%), dermal edema (32%), pink 'pigtails'(28%), intraepidermal pustules (24%), plasma cells (20%) and vasculitis (4%).. The pathologic characteristics of scabietic infection are wide-ranging. Spongiosis, superficial and deep inflammation, and dermal eosinophils and neutrophils are seen in the majority of cases. Superficial fibrin thrombi are not uncommon in scabietic infection, and may provide a helpful diagnostic clue when mites are not visible on initial sections.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Animals; Child; Child, Preschool; Eosinophils; Female; Fibrin; Humans; Infant; Lymphocytes; Male; Middle Aged; Neutrophils; Sarcoptes scabiei; Scabies; Skin; Thrombosis; Vasculitis; Young Adult

Topics: Adolescent; Cecal Diseases; Diagnosis, Differential; Female; Fibrin; Humans; Ileal Diseases; Lupus Erythematosus, Systemic; Syncope; Thrombosis; United States; Vasculitis; Weight Loss

Cocaine use can be associated with a wide spectrum of rheumatic manifestations. It poses a diagnostic challenge as the patients usually withhold the information of cocaine use, and no serological tests are available to establish this diagnosis. We report a patient with vasculopathic syndrome secondary to cocaine use. Despite initial denial of drug abuse, skin biopsy suggested the diagnosis, which was subsequently confirmed by urine drug testing. Differentiating cocaine-associated pseudovasculitis from true vasculitis is necessary, as conventional treatment is usually ineffective without complete abstinence from cocaine use and may be associated with significant morbidity as well as mortality.

Topics: Administration, Oral; Adult; Cocaine; Cocaine-Related Disorders; Female; Fibrin; Glucocorticoids; Humans; Prednisone; Skin; Substance Abuse Detection; Thrombosis; Treatment Outcome; Vasculitis

Fibrin ring granulomas are rare lesions whose pathophysiology has remained somewhat elusive. It has been suggested that these peculiar lesions are related to focal vasculitis with endothelial injury and deposition of immune complexes. Fibrin ring granulomas have been described in Q fever; in viral infections such as cytomegalovirus, Epstein-Barr virus, and hepatitis A virus; and in other conditions. We submit the first reported case of fibrin ring granuloma in a patient with chronic hepatitis C infection, in whom other potential etiologies have been excluded, and offer a brief review of available literature on the pathogenesis of both conditions.

Topics: Fibrin; Granuloma; Hepatitis C, Chronic; Humans; Immune Complex Diseases; Male; Middle Aged; Vasculitis

Comparative histopathological analysis was performed in 47 incompletely embolised and resected cerebral arteriovenous malformations (AVMs).. Thirty-three AVMs were embolised with n-butyl-cyanoacrylate (NBCA), four with iso-butyl-cyanoacrylate (IBCA), seven with polyvinyl alcohol particles (PVA), one with a fibrin mixture, one with silicon pellets, and one with microcatheter balloons. Maximum exposure time (MET) of the embolising agent (interval between embolisation and surgery) ranged from <24 hours to 80 months. All AVMs were investigated regarding angionecrosis, angiofibrosis, acute inflammation, chronic inflammation, foreign-body reactions, vascular calcification, blood admixture to embolising cast, and capillary recanalisation within the AVMs. These parameters were correlated with MET, comparing different embolising agents, age, and sex.. A typical sequence of events depending on MET is observed in all embolised AVMs: acute inflammation with mural angionecrosis is soon replaced by prominent chronic granulomatous vasculitis, which remains stable and is detectable for a very long time, even in AVMs with a MET of more than 6 years.. Capillary recanalisation is always present in incompletely embolised AVMs, detectable after 3 months of MET, irrespective of the embolising agent used. Age and sex does not influence pattern and time course of tissue lesions and recanalisation in incompletely embolised AVMs.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Bucrylate; Capillaries; Catheterization; Child; Cyanoacrylates; Embolization, Therapeutic; Enbucrilate; Female; Fibrin; Follow-Up Studies; Humans; Intracranial Arteriovenous Malformations; Male; Middle Aged; Polyvinyl Alcohol; Silicon; Time Factors; Vasculitis

Anti-beta 2-glycoprotein I antibodies bind to endothelial cells through beta 2-GPI. The antibodies are present in patients with systemic lupus erythematosus and antiphospholipid syndrome and are associated with the pathogenesis of the disease. Anti-endothelial cell antibodies that react with constitutive antigens on ECs are present in patients with vasculiditis and other diseases. Both types of antibodies can activate ECs. Frequent findings in APLS and vasculitis are fibrin deposits and thromboembolic phenomena. These indicate that the coagulation system is activated. However, the mechanism of activation is not clear. ECs generate tissue factor upon stimulation with various substances. In the present study we report that monoclonal anti-beta 2-GPI antibodies and AECAs, derived from a patient with primary APLS and a patient with Takayasu's arteritis, respectively, induce a potent tissue factor in ECs. The production of TF activity, TF antigen and TF mRNA is dose and time dependent. The TF activity was induced also by F(ab)2 but not by Fc fragments and was abolished completely by pre-incubation with ant-TF antibodies. The TF that is induced in ECs by AECAs with and without beta 2-GPI specificity may activate the coagulation and thereby play a major role in the pathogenesis of fibrin deposition and thrombus formation in diseases that are associated with the presence of these antibodies.

Topics: Antibodies; Antigens; Antiphospholipid Syndrome; Apolipoproteins; beta 2-Glycoprotein I; Binding Sites, Antibody; Cells, Cultured; Endothelium, Vascular; Fibrin; Glycoproteins; Humans; Immunoglobulin Fab Fragments; Immunoglobulin Fc Fragments; Lupus Erythematosus, Systemic; Membrane Glycoproteins; RNA, Messenger; Takayasu Arteritis; Thromboembolism; Thromboplastin; Vasculitis

Livedoid vasculitis is characterized clinically by smooth or depressed ivory-white scars surrounded by hyperpigmentation and telangiectasia with or without preceding purpuric infiltrated papules and plaques and histologically by intravascular deposition of fibrin. Its etiology remains obscure and therapy very difficult.. Our purpose was to test the efficacy of low-dose danazol in the treatment of livedoid vasculitis.. Seven patients with active lesions of livedoid vasculitis were treated with low-dose danazol (200 mg, orally, daily). Laboratory coagulation and fibrinolysis parameters, including antithrombin III, protein C, protein S, tissue plasminogen activator, plasminogen, alpha 2-antiplasmin and fibrinogen, were evaluated before and during the therapy.. Six of the 7 patients completed the treatment. After the therapy, all 6 patients had rapid cessation of new lesion formation, prompt reduction in their pain and healing of ulcers. A significant elevation of plasminogen and a decrease in fibrinogen levels were noted 1 month after initiation of the therapy (p = 0.028). The level of fibrinogen seemed to parallel the disease activity in individual patients. In addition, in most of these patients, the levels of antithrombin III, protein C, protein S and alpha 2-antiplasmin tended to increase after the treatment. However, the differences were not statistically significant. Abnormalities of tissue plasminogen activator levels were less consistent. Low-dose danazol was well tolerated without major side effects.. We concluded that low-dose danazol was effective in the treatment of livedoid vasculitis, without unacceptable side effects.

Topics: Administration, Oral; Adult; alpha-2-Antiplasmin; Antithrombin III; Blood Coagulation; Blood Vessels; Cicatrix; Danazol; Estrogen Antagonists; Female; Fibrin; Fibrinogen; Fibrinolysis; Humans; Hyperpigmentation; Male; Plasminogen; Protein C; Protein S; Purpura; Remission Induction; Safety; Skin Diseases, Vascular; Skin Ulcer; Telangiectasis; Tissue Plasminogen Activator; Vasculitis; Wound Healing

Fifteen cases with suspected urticarial vasculitis which were seen during the last ten years at the Department of Dermatology in Hamburg were reviewed. The cases were reevaluated after strict formulation of criteria for vasculitis (fibrin in and around small vessels, leukocytoclasis of neutrophilic granulocytes, extravasation of erythrocytes) and discussed in the context of the international literature on urticarial vasculitis. The conclusion of our study is that urticarial vasculitis is often overdiagnosed clinically if persistent urticarial lesions occur that show some erythematous changes or a hint of hemorrhage. Furthermore, urticarial vasculitis is often overdiagnosed histopathologically because some cases of urticaria were found that presented with heavy infiltration of small vessel walls with neutrophilic granulocytes. In these cases extravasation of erythrocytes, fibrin in and around vessels and leukocytoclasis is always absent. In summary urticarial vasculitis seems to be a variation of leukocytoclastic vasculitis with less extravasation of erythrocytes and not, as frequently stated, and entity of its own.

Topics: Diagnosis, Differential; Fibrin; Humans; Retrospective Studies; Skin; Urticaria; Vasculitis

To examine the vascular changes occurring in three archival cases of acute multiple sclerosis, and to provide immunohistochemical evidence of early endothelial cell activation and vascular occlusion in this condition.. Central nervous system tissues from three cases of acute active multiple sclerosis and six non-inflammatory controls were stained using the following methods: haematoxylin and eosin, Luxol fast blue, cresyl violet, Bielschowsky's silver, and reticulin. Tissues were also immunostained with specific antibodies against collagen type IV, factor XIIIa, class II antigens, glial fibrillary acidic protein, and fibrinogen.. Early vascular endothelial cell activation which may progress to vasculitis and vascular occlusion including class II antigen expression and fibrin deposition were identified. The vascular changes were seen prior to cerebral parenchymal reaction and demyelination, and were not seen in control cerebral tissues.. It is proposed that vascular endothelial cell activation may be an early and pivotal event in the evolution of multiple sclerosis, and that demyelination may have an ischaemic basis in this condition. The vascular endothelium may contain an early element in the evolution of multiple sclerosis.

Topics: Acute Disease; Adolescent; Adult; Brain; Endothelium, Vascular; Female; Fibrin; HLA-D Antigens; Humans; Immunoenzyme Techniques; Male; Microcirculation; Multiple Sclerosis; Thrombosis; Vascular Diseases; Vasculitis

We have established a recombinant inbred strain of mouse named spontaneous crescentic glomerulonephritis-forming mouse/Kinjoh or SCG/Kj. Mice of this strain spontaneously develop rapidly progressive glomerulonephritis. This strain of mice was derived from (BXSB/Mp x MRL/Mp-lpr/lpr)F1 hybrid mice by brother x sister mating coupled with repeated histopathologic selection for breeding of mice whose parents had the highest frequency of crescent formation in the kidneys. In this strain of mice, nephritis appears earlier and is more rapidly progressive than in any other murine model of systemic lupus erythematosus. Histopathologically, the characteristic renal lesions in the mice of this strain express a most dramatic form of crescentic glomerulonephritis. The lesions in the kidneys show only slight fine granular immune deposits along the glomerular basement membrane associated with remarkable extraglomerular proliferation and hemorrhage in Bowman's space. Although selection was not based on vasculitis, mice of this strain also exhibit a high incidence of necrotizing vasculitis. These vascular lesions involve primarily small arteries and arterioles and many organs and tissues but spare the kidneys. Thus this form of vasculitis has been found to be correlated with the crescentic form of glomerulonephritis but not with lymphoid hyperplasia of the spleen. We conclude that, in this strain of mouse, the rapidly progressive glomerulonephritis is genetically restricted and that this genetic restriction is firmly linked to that responsible for the vasculitis.

Topics: Animals; Chromosome Mapping; Coronary Circulation; Crosses, Genetic; Female; Fibrin; Glomerulonephritis; Immunohistochemistry; Kidney; Male; Mice; Mice, Inbred Strains; Ovary; Proteinuria; Selection, Genetic; Sex Factors; Species Specificity; Spleen; Stomach; Vasculitis

Evidence of damage to cerebral vein walls was sought in 70 cases of multiple sclerosis. Seventy control cases were also examined. The multiple sclerosis cases showed venous intramural fibrinoid deposition (7%), recent haemorrhages (17%), old haemorrhages revealed by haemosiderin deposition (30%), thrombosis (6%) and thickened veins (19%). In all, 41% of all multiple sclerosis cases showed some evidence of vein damage. Occasional control cases showed haemosiderin deposition in the brain but, unlike the multiple sclerosis cases, these were diffuse and almost entirely related to coexistent cardiovascular or cerebrovascular disease. Haemosiderin deposition was common in the substantia nigra and other pigmented nuclei in all cases. It is concluded that the cerebral vein wall in multiple sclerosis is subject to chronic inflammatory damage, which promotes haemorrhage and increased permeability, and constitutes a form of vasculitis.

Topics: Cerebral Hemorrhage; Cerebral Infarction; Cerebral Veins; Cerebrovascular Disorders; Fibrin; Hemosiderin; Humans; Intracranial Embolism and Thrombosis; Iron; Multiple Sclerosis; Vasculitis

The study of human cutaneous leishmaniasis from Saudi Arabia (37 cases), and of Sudanese mucosal (52 cases) and visceral (27 cases) leishmaniasis revealed the occurrence of vascular alterations. Vasculitis with or without fibrinoid necrosis and fibrin thrombi were found in both the arteries and veins within the area of inflammation in cutaneous and mucosal leishmaniasis; whereas subendothelial oedema, hyalinosis and intimaproliferation were seen in the small arteries of the various organs in visceral leishmaniasis. Immunoperoxidase staining of the cutaneous leishmaniasis lesions showed the presence of IgG and IgA within the endothelial cells, in the media, and in the perivascular space of the diseased vessels. The formation of immune complexes, locally in cutaneous and mucosal leishmaniasis and circulating in visceral leishmaniasis, is considered responsible for these vascular lesions.

Topics: Blood Vessels; Fibrin; Humans; Immunoglobulin A; Immunoglobulin G; Leishmaniasis; Vasculitis

Atrophie blanche is an uncommon condition characterized by the development of white atrophic patches of skin on the lower extremities, which form as a result of fibrinoid vasculitis of superficial and mid-dermal vessels followed by necrosis and ulceration of the epidermis. We report four cases in which similar lesions developed on the legs and ankles of young Jewish Russian immigrants to Israel. Although the lesions share many features with atrophie blanche, they differ in their early age of onset, the male predilection, and the extension of the fibrinoid vasculitic process into the subcutaneous tissue. Additionally, the peculiar population clustering (Georgia, U.S.S.R.), common ethnic background, and a family history of similar lesions in close relatives seem to point to a familial or genetic predisposition underlying the development of the disease.

Topics: Adolescent; Adult; Atrophy; Biopsy; Capillaries; Female; Fibrin; Humans; Leg Ulcer; Male; Necrosis; Skin; Skin Ulcer; Vasculitis

We obtained biopsies from early, fully developed, and late lesions of erythema elevatum diutinum (EED) in a 49-year-old man. The histologic and electron-microscopic findings were compared with those reported in the literature and three other cases from our files. Early lesions show leukocytoclastic vasculitis with capillary proliferation. Later lesions show vasculitis, dermal aggregates of neutrophils, fibrosis, and areas of granulation tissue. Newly formed vessels in granulation tissue may be more susceptible to damage by immune complexes, and the early formation of granulation tissue in EED may prevent an early resolution of vasculitis. Damage to dermal connective tissue in EED incites either scarring or, rarely, a fibrohistiocytic proliferation. Ultrastructural examination of one case showed histiocytes with myelin figures and intracellular lipid and cholesterol.

Topics: Erythema; Fibrin; Granulation Tissue; Humans; Leukocytes; Male; Microscopy, Electron; Middle Aged; Skin; Vasculitis; Venules

In 50 patients suffering for 1-15 years from rheumatoid arthritis, needle-biopsy of the synovial membrane was carried out. The finding correlated with the general clinical activity of the disease. Each type of histological change was evaluated with regard to its diagnostic value in assessing clinical activity. Among the histological changes, oedema, synovial cell proliferation, lymphocyte-plasma cell proliferation and necrotic vasculitis showed a negative correlation with clinical activity, while a positive correlation was observed between clinical activity and the presence of fibrin (fresh and old), fibrinoid, fibrinoid basophilia, fibroblast proliferation, synovial cell desquamation, concentric perivascular sclerosis, fibrosis and hyalinosis. Vascular changes of the synovial membrane such as oedema, fresh fibrin exudate, necrotic vasculitis showed a negative correlation with clinical activity while hyalinization and concentric sclerosis and clinical activity were found to be in positive correlation. It is concluded that in the course of rheumatoid arthritis the histological changes do not necessarily run parallel with the clinical activity of the disease.

Topics: Arthritis, Rheumatoid; Biopsy, Needle; Edema; Endothelium; Female; Fibrin; Humans; Hyalin; Lymphocytes; Male; Muscle, Smooth, Vascular; Plasma Cells; Synovial Membrane; Vasculitis

Neuropathologic examination of an autopsy series of 54 patients of various types of CVD revealed a very high frequency of pathologic changes both in brain parenchyma (in 81%) and vessels (in 78%). A broad but continuous spectrum of primary vascular alterations was observed, ranging from fibrinoid deposits in intact or necrotizing vessel walls to fibrohyalinosis and endothelial proliferations. In acute SLE showing LE cells within brain tissues, immune complex deposits were observed for the first time in brain vessels, in addition to similar deposits in the plexus chorioideus and in hematoxylin bodies. Secondary complications are frequently affecting the brain in CVD; they are mainly sequels of systemic atherosclerosis, hypertension, thromboemboli from SLE endocarditis, cardiac, hepatic or renal dysfunctions, or infections and should be clinically differentiated from primary brain involvement in CVD to ensure the appropriate therapeutic measures.

Topics: Arthritis, Rheumatoid; Brain; Cerebral Arteries; Collagen Diseases; Complement C3; Fibrin; Humans; Immunoglobulin G; Lupus Erythematosus, Systemic; Myositis; Polyarteritis Nodosa; Purpura, Thrombotic Thrombocytopenic; Vascular Diseases; Vasculitis

A kidney and lung biopsy were performed on a patient with active Behçet's disease with renal and pulmonary involvement. Histologic, immunohistochemical and electron microscopic studies of the kidney biopsy specimen revealed a focal segmental necrotizing glomerulonephritis characterized by the presence of numerous subendothelial and occasional intramembranous deposits containing immunoglobulin G (IgG), the third component of complement (C3), the fourth component of complement (C4) and fibrin(ogen). Histologic and immunohistochemical studies of the lung biopsy specimen showed an acute venulitis and septal capillaritis associated with the presence of identical deposits within the walls of affected vessels. Circulating immune complexes were detected in the patient's serum by Raji cell assay. The findings indicate that the glomerulonephritis and pulmonary vasculitis occasionally occurring in Behçet's disease are due to the deposition of circulating antigen-antibody complexes. In addition, they strongly suggest that the majority of the major and minor manifestations of the disease, such as uveitis, cutaneous vasculitis, synovitis and meningoencephalitis, are a result of vascular immune complex deposition.

Topics: Behcet Syndrome; Capillaries; Complement C3; Complement C4; Female; Fibrin; Fibrinogen; Fluorescent Antibody Technique; Glomerulonephritis; Humans; Immune Complex Diseases; Immunoglobulin G; Kidney Glomerulus; Lung; Middle Aged; Pulmonary Alveoli; Pulmonary Circulation; Vasculitis

Psoriatic arthritis (PA) is included in the seronegative arthritis group, though it is now generally considered to represent a clinical entity. In PA, in contrast to psoriasis vulgaris and to other types of rheumatoid arthritis, only a few immunological studies have been reported. In the present report synovial joint membranes from patients with PA and control groups have been studied for the presence of (a) vascular changes, (b) fibrin, (c) immunoglobulins and complement factor C3.

Topics: Arthritis; Arthritis, Rheumatoid; Complement C3; Fibrin; Fluorescent Antibody Technique; Humans; Immunoglobulins; Psoriasis; Synovial Membrane; Vasculitis

Topics: Adult; Complement System Proteins; Female; Fibrin; Fibrinogen; Fluorescent Antibody Technique; Humans; Immunoglobulins; Pityriasis; Vasculitis; Vasculitis, Leukocytoclastic, Cutaneous

Topics: Fibrin; Humans; Peripheral Vascular Diseases; Skin; Vascular Diseases; Vasculitis