Crystal induced arthropathies-a comparative study of 40 patients with apatite rheumatism, chondrocalcinosis and primary synovial chondromatosis.

Introduction: Apatite rheumatism (AR), chondrocalcinosis (Ch-C), and primary synovial chondromatosis (prSynCh) are regarded as distinct clinical entities. The introduction of the non-staining technique by Bély and Apáthy (2013) opened a new era in the microscopic diagnosis of crystal induced diseases, allowing the analysis of MSU (monosodium urate monohydrate) HA (calcium hydroxyapatite), CPPD (calcium pyrophosphate dihydrate) crystals, cholesterol, crystalline liquid lipid droplets, and other crystals in unstained sections of conventionally proceeded (aqueous formaldehyde fixed, paraffin-embedded) tissue samples. The aim of this study was to describe the characteristic histology of crystal deposits in AR, Ch-C, and prSynCh with traditional stains and histochemical reactions comparing with unstained tissue sections according to Bély and Apáthy (2013). Patients and methods: Tissue samples of 4 with apatite rheumatism (Milwaukee syndrome), 16 with chondrocalcinosis, and 20 with clinically diagnosed primary synovial chondromatosis were analyzed. Results and conclusion: Apatite rheumatism, chondrocalcinosis, and primary synovial chondromatosis are related metabolic disorders with HA and CPPD depositions. The authors assume that AR and Ch-C are different stages of the same metabolic disorder, which differ from prSynCh in amorphous mineral production, furthermore in the production of chondroid, osteoid and/or bone. prSynCh is a defective variant of HA and CPPD induced metabolic disorders with reduced mineralization capabilities, where the deficient mineralization is replaced by chondroid and/or bone formation. The non-staining technique of Bély and Apáthy proved to be a much more effective method for the demonstration of crystals in metabolic diseases than conventional stains and histochemical reactions.

[Mönckeberg's sclerosis -- crystal-induced angiopathy]. / Mönckeberg-sclerosis -- kristály indukálta angiopathia.

INTRODUCTION: Mönckeberg's sclerosis is a special form of arteriosclerosis characterized by calcification and ossification of the media of medium size arteries mainly of lower extremities. AIMS: The aim of the authors was to examine medium size arteries with Mönckeberg's sclerosis in 22 amputated lower legs of 16 patients in order to demonstrate different crystals in the wall of blood vessels. METHODS: The methodology was based on previous findings of the authors indicating that in different metabolic disorders many crystals remain demonstrable in unstained histological sections unlike in haematoxylin-eosin stained sections. RESULTS: In unstained sections the authors observed rhomboid or prismatic calcium pyrophosphate dihydrate and clusters of elongated narrow hydroxyapatite crystals in the wall of medium size arteries of all examined cases. Both types of crystals showed axis parallel positive birefringence under polarized light. The intensity of birefringence of hydroxyapatite crystals was weaker in comparison with that of calcium pyrophosphate dihydrate crystals. Occasionally, other crystals which were different in shape from both calcium pyrophosphate dihydrate and hydroxyapatite crystals were also observed. CONCLUSIONS: It seems likely that similarly to crystal deposition induced arthropathy, calcium pyrophosphate dihydrate, hydroxyapatite and other crystals cause fibrosis and intimal proliferation, which may contribute to progressive occlusion of blood vessels resulting in ischemic symptoms. Based on this observation Mönckeberg's sclerosis may be defined as a crystal-induced angiopathy.

Recurrent pancreatic arteritis and vasculogenic relapsing pancreatitis in rheumatoid arthritis - a retrospective clinicopathologic and immunohistochemical study of 161 autopsy patients.

The aim of this study was to determine: the prevalence, and histological characteristics of vasculitis in the pancreas, and to follow the formal pathogenesis of multifocal pancreatitis due to arteritis and/or arteriolitis (multifocal vasculogenic pancreatitis). A randomized autopsy population of 161 in-patients with rheumatoid arthritis (RA) was studied. Systemic vasculitis (SV) complicated RA in 36 (22.36%) of 161 cases; tissue samples of pancreas were available for histologic evaluation in 28 patients. Pancreatitis and vasculitis were characterized histologically and immunohistochemically. Vasculogenic, multifocal pancreatitis was not recognized clinically. Vasculitis of the pancreatic arterioles and small arteries (branches of splenic artery, upper and lower gastroduodenal arteries) can lead to local ischaemia and to regressive changes in the pancreas. This vasculogenic process is more or less widespread and multifocal, depending on the number of involved vessels and is followed by reactive inflammation, depending on the stages of the pathological process. Because of the recurrent nature of vasculitis with time these regressive changes accumulate within the pancreas and may contribute to an unexpected circulatory failure and sudden death of the patient. Vasculogenic microinfarcts in the pancreas may be clinically characterized by unexplained recurrent abdominal symptoms and spontaneous remissions which insidiously may lead to metabolic failure resistant to therapy.

[Lethal complications and associated diseases of rheumatoid arthritis--a retrospective clinicopathologic study of 234 autopsy patients]. / Szövodmények és társult megbetegedések rheumatoid arthritisben--234 elhunyt beteg patológiai és klinikai adatainak retrospektív elemzése.

OBJECTIVE: Complications and/or associated diseases in rheumatoid arthritis can present atypical clinical manifestations which may lead to an incorrect or delayed diagnosis. The aim of this study was to determine: (1) the complications of rheumatoid arthritis, the accompanying diseases, and the mortality of these, (2) the clinically missed diagnoses of complications and/or associated diseases, (3) the possible links between coexistent complications of rheumatoid arthritis and/or diseases associated with it, furthermore the possible role of these in the mortality of rheumatoid arthritis patients. METHODS: Between 1970 and 1999 10,860 patients died at the National Institute of Rheumatology, and among them 234 with rheumatoid arthritis (diagnosed clinically according to the criteria of the American College of Rheumatology). The associated and basic disease, complication(s), and causes of death were determined on the basis of clinical records and in each case the autopsy findings were confirmed by a review of extensive histological material (50-100 tissue blocks from each patient). RESULTS: The complications of rheumatoid arthritis led to death in 152 (65%) of 234 patients. The complications of RA were clinically recognized in 109 (46.6%, 71.7 rel%) and missed in 43 (18.4% 28.3 rel%) of 152 patients. More than two thirds of lethal complications related to rheumatoid arthritis were diagnosed clinically. The remaining 82 (35%) of 234 rheumatoid arthritis patients died of associated diseases; the cause of death was clinically recognized in 78 (33.3%, 95.1 rel%) of 82 cases. There was a significant and positive correlation (1) between vasculitis and cardiac insufficiency (chi2 = 6.37, p <0.01), vasculitis and tuberculosis (chi2 = 4.18, p <0.04), or miliary tuberculosis (chi2 = 3.86, p <0.04); (2) between tuberculosis and miliary tuberculosis (chi2 = 54.84, p <0.001); and (3) between septic infection and purulent arthritis (chi2 = 97.04, p <0.001). There was a significant and inverse correlation between atherosclerosis and vasculitis (chi2 = 5.10, p <0.02), atherosclerosis and amyloidosis (chi2 = 14.58, p <0.001), or atherosclerosis and septic infection (chi2 = 3.81, p <0.05). There was a significant and inverse correlation between atherosclerosis and lethal cases of vasculitis (chi2 = 9.31, p <0.002), of amyloidosis (chi2 = 6.82, p <0.009), of sepsis (chi2 = 3.81, p <0.05) furthermore, between atherosclerosis with lethal outcome (n = 60 of 106) and vasculitis (chi2 = 12.06, p <0.001), or amyloidosis (chi2 = 13.22, p<0.002), or sepsis (chi2 = 10.82, p <0.001), or purulent arthritis (chi2 = 4.18, p <0.04). CONCLUSION: The most important life threatening complications of rheumatoid arthritis (vasculitis, AA amyloidosis and sepsis) are present and lead to death with higher probability in the younger age group (without atherosclerosis), while in older patients who have atherosclerosis these represent a lower risk of death.

Electron microscopic characteristics of beta2-microglobulin amyloid deposits in long-term haemodialysis.

The electron microscopic features of beta2-microglobulin amyloid, deposited in the synovial membrane, are presented and discussed. The patient, a 69-year-old woman underwent chronic hemodialysis for 3 years. Because of constant pain and destructive arthropathy, endoprosthesis of the hip joints were implanted. Extra- and intracellular filamentous-fibrillar amyloid deposits have been demonstrated in ultrathin sections. The extracellular amyloid deposits showed a loose, filamentous or fibrillar structure at the periphery and a dense central core. The loose, filamentous structure may represent an early stage of fresh, newly deposited beta2-microglobulin amyloid, while the condensed and fragmented amyloid filaments may be an advanced "mature" stage of amyloid deposition.