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Background: Ankylosing spondylitis (AS) is a chronic inflammatory condition primarily affecting the spine and sacroiliac joints, often associated with human leukocyte antigen B27 (HLA-B27) positivity. While musculoskeletal symptoms are typical manifestations, AS can also lead to systemic complications, including secondary systemic amyloidosis (SSA), also known as Amyloid A (AA) amyloidosis, involving multiple organs. Case presentation: We present a case of a 45-year-old Asian male with a complex medical history, including diabetes and hypertension, who developed AS complicated by SSA. The patient exhibited a diverse range of symptoms, including cardiac, renal, gastrointestinal, and musculoskeletal manifestations. He reported shortness of breath on exertion, orthopnea, pedal edema, generalized weakness, back pain, neck pain, low-grade fever, decreased appetite, frothy urine, and significant weight loss over the past year. Diagnostic evaluations revealed HLA-B27 positivity and histologically confirmed AA amyloidosis, providing a comprehensive understanding of the systemic involvement. Conclusion: This case report highlights the intricate interplay between AS and SSA, particularly AA amyloidosis, with a focus on its systemic impact beyond musculoskeletal symptoms. The tragic outcome, marked by severe cardiac involvement, underscores the challenges in managing such complex cases. This report emphasizes the importance of early diagnosis and treatment of AS to prevent severe complications, along with vigilant monitoring and individualized treatment plans, as well as the need for further research to enhance our understanding and improve management strategies for AS-related amyloidosis. [ABSTRACT FROM AUTHOR]

Alkaptonuria (AKU) is a rare autosomal recessive metabolic disorder caused by mutations in the homogentisate 1,2-dioxygenase (HGD) gene, leading to the accumulation of homogentisic acid (HGA), causing severe inflammatory conditions. Recently, the presence of serum amyloid A (SAA) has been reported in AKU tissues, classifying AKU as novel secondary amyloidosis; AA amyloidosis is characterized by the extracellular tissue deposition of fibrils composed of fragments of SAA. AA amyloidosis may complicate several chronic inflammatory conditions, like rheumatoid arthritis, ankylosing spondylitis, inflammatory bowel disease, chronic infections, neoplasms, etc. Treatments of AA amyloidosis relieve inflammatory disorders by reducing SAA concentrations; however, no definitive therapy is currently available. SAA regulation is a crucial step to improve AA secondary amyloidosis treatments. Here, applying a comprehensive in vitro and in silico approach, we provided evidence that HGA is a disruptor modulator of SAA, able to enhance its polymerization, fibril formation, and aggregation upon SAA/SAP colocalization. In silico studies deeply dissected the SAA misfolding molecular pathway and SAA/HGA binding, suggesting novel molecular insights about it. Our results could represent an important starting point for identifying novel therapeutic strategies in AKU and AA secondary amyloidosis-related diseases. [ABSTRACT FROM AUTHOR]

Abstract Background Ankylosing spondylitis (AS) is a chronic inflammatory condition primarily affecting the spine and sacroiliac joints, often associated with human leukocyte antigen B27 (HLA-B27) positivity. While musculoskeletal symptoms are typical manifestations, AS can also lead to systemic complications, including secondary systemic amyloidosis (SSA), also known as Amyloid A (AA) amyloidosis, involving multiple organs. Case presentation We present a case of a 45-year-old Asian male with a complex medical history, including diabetes and hypertension, who developed AS complicated by SSA. The patient exhibited a diverse range of symptoms, including cardiac, renal, gastrointestinal, and musculoskeletal manifestations. He reported shortness of breath on exertion, orthopnea, pedal edema, generalized weakness, back pain, neck pain, low-grade fever, decreased appetite, frothy urine, and significant weight loss over the past year. Diagnostic evaluations revealed HLA-B27 positivity and histologically confirmed AA amyloidosis, providing a comprehensive understanding of the systemic involvement. Conclusion This case report highlights the intricate interplay between AS and SSA, particularly AA amyloidosis, with a focus on its systemic impact beyond musculoskeletal symptoms. The tragic outcome, marked by severe cardiac involvement, underscores the challenges in managing such complex cases. This report emphasizes the importance of early diagnosis and treatment of AS to prevent severe complications, along with vigilant monitoring and individualized treatment plans, as well as the need for further research to enhance our understanding and improve management strategies for AS-related amyloidosis.

Abstract Background Ankylosing spondylitis (AS) is a type of chronic inflammation that is most prevalent in young adults and is characterized by an inflammatory enthesiopathy that gradually develops toward ossification and ankylosis. If inflammation is left unchecked, it can potentially lead to complications such as secondary amyloidosis, also known as AA amyloidosis, involving the deposition of amyloid serum A protein. Our case presents with a thyroid localization of AA amyloidosis which is secondary to this AS. Such a case has been described in only four cases in the literature. Cardiac localization of AA amyloidosis has been exceptionally described in the literature. Case presentation We report the case of a young patient with severe AS complicated by secondary amyloidosis with thyroid, cardiac, and probably renal localization. He was treated with anti-TNF therapy, and his condition improved significantly. Conclusions Our case presents several localizations of AA amyloidosis secondary to this AS. Although cardiac involvement is rare in secondary AA amyloidosis, it should always be screened for, even in a cardiacly asymptomatic patient.

Background: Ankylosing spondylitis (AS) is a type of chronic inflammation that is most prevalent in young adults and is characterized by an inflammatory enthesiopathy that gradually develops toward ossification and ankylosis. If inflammation is left unchecked, it can potentially lead to complications such as secondary amyloidosis, also known as AA amyloidosis, involving the deposition of amyloid serum A protein. Our case presents with a thyroid localization of AA amyloidosis which is secondary to this AS. Such a case has been described in only four cases in the literature. Cardiac localization of AA amyloidosis has been exceptionally described in the literature. Case presentation: We report the case of a young patient with severe AS complicated by secondary amyloidosis with thyroid, cardiac, and probably renal localization. He was treated with anti-TNF therapy, and his condition improved significantly. Conclusions: Our case presents several localizations of AA amyloidosis secondary to this AS. Although cardiac involvement is rare in secondary AA amyloidosis, it should always be screened for, even in a cardiacly asymptomatic patient. [ABSTRACT FROM AUTHOR]

Alkaptonuria (AKU) is a rare autosomal recessive metabolic disorder caused by mutations in the homogentisate 1,2-dioxygenase (HGD) gene, leading to the accumulation of homogentisic acid (HGA), causing severe inflammatory conditions. Recently, the presence of serum amyloid A (SAA) has been reported in AKU tissues, classifying AKU as novel secondary amyloidosis; AA amyloidosis is characterized by the extracellular tissue deposition of fibrils composed of fragments of SAA. AA amyloidosis may complicate several chronic inflammatory conditions, like rheumatoid arthritis, ankylosing spondylitis, inflammatory bowel disease, chronic infections, neoplasms, etc. Treatments of AA amyloidosis relieve inflammatory disorders by reducing SAA concentrations; however, no definitive therapy is currently available. SAA regulation is a crucial step to improve AA secondary amyloidosis treatments. Here, applying a comprehensive in vitro and in silico approach, we provided evidence that HGA is a disruptor modulator of SAA, able to enhance its polymerization, fibril formation, and aggregation upon SAA/SAP colocalization. In silico studies deeply dissected the SAA misfolding molecular pathway and SAA/HGA binding, suggesting novel molecular insights about it. Our results could represent an important starting point for identifying novel therapeutic strategies in AKU and AA secondary amyloidosis-related diseases.

Serum amyloid A protein A (SAA) is a normal serum protein (serving as a precursor of fibrillar tissue protein AA), synthesized in the liver and a rapidly responding marker of the acute phase of inflammation. A constant high concentration of SAA is one of the factors in the development of AA-amyloidosis. As a rule, secondary amyloidosis develops in patients with long-term and poorly controlled inflammatory diseases, including rheumatic diseases, one of which is ankylosing spondylitis (AS).Objective: to assess the level of SAA in AS patients and its relationship with indicators of disease activity.Patients and methods. The study included 124 patients with AS who met the modified New York 1984 criteria. The disease activity and functional status of patients were assessed according to the recommendations of Russian experts. SAA and CRP, ESR in blood serum were measured in all patients.Results and discussion. The median SAA concentration was 12.5 mg/L [4; 71.6]. Of 124 patients, 31% had SAA levels 5 mg/L. A strong correlation was found between the levels of SAA and CRP (r=0.80, p

Amaç: Amiloidozun koroner arterlere etkisini ortaya koymak ve eğer artmış bir koroner arter hastalığı riski varsa erken tanı ile olguların yaşam süresini artırmada katkı sağlamak amaçlanmaktadır.Gereç ve Yöntem: Çalışmaya 6 ay süresince Nefroloji Polikliniğinimize başvuran, kronik böbrek yetmezliğine girmemiş, biyopsi ile tanı almış 20 sekonder amiloidoz olgusu ve sağlıklı 20 gönüllüden oluşan kontrol grubu dahil edildi. Hasta ve kontrol grubuna Toshiba Aquilion 16 dedektörlü çok kesitli bilgisayarlı tomografi cihazı ile koroner arter kalsiyum skorlama tetkiki yapıldı. Öykülerinde koroner arter hastalığı, hipertansiyon, diyabetes mellitus, kronik böbrek yetmezliği, hiperlipidemi, obesite olan olgular çalışma dışı bırakıldı. Bulgular: Hasta grubumuzda koroner arter kalsiyum skoru ortalaması 16,05 ±10,4 kontrol grubunda 6,4±4,36 idi. İki grup arasındaki istatistiksel fark anlamlı bulunmadı p=0,739 . Gruplar arasında yaş, cinsiyet, sigara ve alkol kullanımı, sistolik ve diastolik kan basıncı, açlık kan şekeri ve kan lipid parametreleri, kan üre nitrojen, kreatinin, C reaktif protein ve fosfor düzeyleri açısından anlamlı farklılık saptanmadı. Kan albumin ve total kalsiyum düzeyleri ile idrar protein/kreatinin arasındaki fark ise anlamlı idi p

BackgroundAA amyloidosis due to subcutaneous injection of drugs of abuse has been described in the USA, but all the existing literature is from more than 20 years ago. There is more recent literature from Europe. We have observed a high incidence of AA amyloidosis in the county hospital in San Francisco.DesignHere, we describe 24 patients who had kidney biopsy-proven AA amyloidosis from our hospital from 1998 to 2013. All the patients were thought to have AA amyloidosis from skin popping of illicit drugs after having exhausted the intravenous route. These patients with biopsy-proven AA amyloidosis were analysed further.ResultsAll patients were found to have hepatitis C infection, hypertension was not common, most had advanced kidney failure, and acidosis was common as was tubulointerstitial involvement on the kidney biopsy. Other organ involvement included hepatomegaly and splenomegaly in a number of patients; direct myocardial involvement was not seen, but pulmonary hypertension, history of deep vein thrombosis and pulmonary embolism were common. The prognosis of these patients was poor. The mortality rate approached 50% 1 year after biopsy, and most of the patient needed dialysis shortly after diagnosis. Cessation of drug use seemed beneficial but rarely achievable.ConclusionAA amyloidosis from skin popping is common in San Francisco. Most patients with renal involvement end up on dialysis, and mortality rates are exceedingly high.

Copyright of Harran University Journal of the Faculty of Veterinary Medicine / Harran Üniversitesi Veteriner Fakültesi Dergisi is the property of Harran University, Faculty of Veterinary Medicine and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

В обзоре литературы изложены современные данные о частоте, механизмах развития, клинических проявлениях, диагностике и лечении вторичного амилоидоза (АА-амилоидоза) почек у детей с ювенильным ревматоидным артритом (ЮРА). Частота встречаемости АА-амилоидоза почек при ЮРА у детей составляет от 0,8 до 2 %, у взрослых с длительностью ЮРА 28,3 года — 8,9 %. В последние годы на фоне иммунобиологической терапии частота АА-амилоидоза почек у взрослых уменьшилась до 2 %. На сегодняшний день установлен основной механизм развития АА-амилоидоза, который заключается в постоянном или периодическом повышении концентрации сывороточного амилоида А (SAA). Установлено, что на синтез SAA влияют провоспалительные цитокины: интерлейкин-1 (ИЛ-1), ИЛ-6, фактор некроза опухоли альфа (ФНО-α), ИЛ-2, ИЛ-11 и другие. Для реализации амилоидогенного потенциала SAA необходимо не только воздействие воспалительного процесса в организме, но и его продолжительность. Обсуждается роль генетических факторов в развитии АА-амилоидоза почек при ЮРА. АА-амилоидоз почек чаще всего развивается у детей при системной форме, у взрослых — при системной и полиартикулярной формах ЮРА. Первым симптомом АА-амилоидоза почек является изолированная протеинурия, которая трансформируется в нефротический синдром. Особенности нефротического синдрома заключаются в отсутствии в большинстве случаев гиперхолестеринемии, сочетании у некоторых больных с артериальной гипертензией, гематурией, нарушением функции почек. Основным методом, подтверждающим диагноз АА-амилоидоза почек, является прижизненное морфологическое исследование почек с окраской биоптатов конго красным. Повышение уровня SAA в крови у детей с ЮРА отражает степень воспалительного процесса и рассматривается как фактор риска развития АА-амилоидоза почек. Применение иммунобиологических препаратов (тоцилизумаб, анакинра) при АА-амилоидозе почек у детей с ЮРА имеет терапевтическую эффективность.

Clinical observation of a patient suffering from rheumatic disease, which is identified as ankylosing spondylitis in the last 5 years and is described for more than 20 years. The progression of ankylosing spondylitis was complicated by the development of nephrotic syndrome and a progressive decrease in renal function to the level of CKD 4 (estimated glomerular filtration rate (eGFR) EPI 19.8 ml/min), anaemia and cachexia. A gum biopsy did not reveal amyloid; a kidney biopsy confirmed the presence of AA amy­loidosis. Adalimumab therapy was started at a dose of 40 mg per week, after 2 months — once every 2 weeks. At the same time, the patient received olmesartan and symptomatic therapy. The three-year administration of adalimumab has led to a tight control of the activity of ankylosing spondylitis and a gradual restoration of renal function to eGFR 62 ml/min. During this treatment, the patient delivered of 2 children. A detailed description of kidney biopsy is given. Treatment with adalimumab and olmesartan is still conti­nuing.

Amyloid goiter is a very rare manifestation of amyloidosis. Here, we describe the case of a 40-year-old male, who presented with upper airway obstructive symptoms including hoarseness and breathing difficulty, to highlight the clinical and pathological features of secondary amyloidosis of the thyroid gland and the difficulties in making a preoperative diagnosis. The patient had previously been wounded in the war in Bosnia, which resulted in the right kidney being surgically removed. Further, he had undergone two surgical interventions on both hips due to osteomyelitis and was on a chronic dialysis program due to a progressively poor left kidney function that had eventually resulted in complete loss of renal function. Thyroid function tests were normal, and the patient clinically was euthyroid; biochemical investigations were within normal limits. Results from sonography, computed tomography scan of the neck, scintigraphy and fine-needle aspiration cytology were nondiagnostic. Therefore, a thyroid biopsy was carried out, and amyloid deposits were found. After preoperative work-up, total thyroidectomy was performed with no complications. We conclude that amyloid goiter may have no major impact on thyroid function even when a substantial amount of amyloid has replaced the normal thyroid parenchyma, as was the case in our patient. Amyloid goiter should be suspected in all patients with a progressive, rapidly growing bilateral thyroid enlargement with concomitant inflammatory processes or in patients undergoing hemodialysis treatment.

Introduction: Hyperechoic renal cortex in a normal-sized kidney has a range of causes, some of which irreparably damage the kidney and should initiate further investigations. Case Report: We present a 72-year-old woman with longstanding bronchiectasis, noticed to have worsening renal function. Renal tract ultrasonography showed hyperechoic normal-sized kidneys. Renal biopsy confirmed amyloidosis. Discussion: Imaging findings in renal amyloidosis tend to be non-specific and are not always present. However, ultrasonography findings of hyperechoic cortex in normal-sized kidneys should not be ignored. Conclusion: Renal impairment from blood chemistry in addition to hyperechoic kidneys may be the key to diagnosing secondary amyloidosis in a patient with bronchiectasis, a rare association but important to recognise. [ABSTRACT FROM AUTHOR]

Cerebral vascular disorders are one of the leading causes of disability and mortality in patients with chronic kidney disease (CKD). The article presents the currently available data on risk factors (RF) for the development of cerebrovascular disorders in pre-dialysis patients with CKD. Two groups of RF are identified: traditional and non-traditional (specific). Traditional RF, which include arterial hypertension, diabetes mellitus and hypercholesterolemia, independently affect the cerebral vascular bed and get worse against the background of CKD. Specific RF is associated with features of the CKD pathogenesis. It includes increased blood levels of homocysteine, β2-microglobulin, impaired calcium-phosphorus metabolism, accumulation of uremic toxins and toxins of intestinal bacteria, anemia and other factors. In the present review, special attention is paid to specific RF and pathogenetic mechanisms of the development of cerebrovascular disorders in predialysis patients with CKD. Timely detection of cerebral risk factors may lead to the improvement of early diagnosis and prevention of cerebral vascular disorders, optimization of therapy for patients with CKD.

Abstract Background Amyloid infiltration of endocrine glands has been reported, mostly in the thyroid, pancreas, adrenals, and testes, but affected patients do not frequently exhibit overt endocrine insufficiency. Here we report the case of a patient with complete anterior hypopituitarism probably due to a known systemic amyloidosis. Case presentation Our male Caucasian patient was diagnosed with Crohn’s disease at the age of 22 years. At the age of 37, he developed secondary renal amyloidosis, which resulted in end-stage renal failure. He received a living-donor kidney transplant at the age of 57, without initial complication. Two months later, he developed extreme fatigue, weight loss, and dyspnea. A hormonal evaluation demonstrated complete anterior pituitary insufficiency. A pituitary magnetic resonance imaging was performed and showed a diffusely hypointense anterior gland on both T1-weighted and T2-weighted images with reduced gadolinium enhancement, highly suggestive of amyloid infiltration of the pituitary. Treatment was initiated with levothyroxine, orally administered hydrocortisone, and testosterone enanthate, rapidly allowing progressive marked clinical improvement and nearly complete resolution of symptoms. Conclusions Pituitary amyloid infiltration should be considered in patients with a known systemic amyloidosis who develop symptoms of hypopituitarism and magnetic resonance imaging features compatible with protein deposits.

Objective: This study aimed to determine the effect of amyloidosis on coronary arteries with evaluation of coronary artery calcium score with computed tomography. Material and Methods: This prospective study involved 20 secondary amyloidosis subjects that diagnosed with renal biopsy and 20 healthy subjects as control group. The patients with coronary artery disease, hypertension, diabetes mellitus, chronic renal failure, hy-perlipidemia, obesity were excluded. Coronary artery calcium scoring was performed to the patient and control groups with Toshiba Aquilion 16 detector multislice computed tomography. Results: The mean coronary artery calcium score was 16.05 ± 10.4 in the amyloidosis group and 6.4 ± 4.36 in the control group. The statistical difference between the two groups was not significant (p = 0.739). No significant difference was found between the groups in terms of age, gender, smoking and alcohol use. The difference between blood albumin and total calcium levels and urine protein / creatinine were significant (p <0.05). The mean body mass index was 25.1 ± 0.64 in the patient group and 22.26 ± 0.71 in the control group (p = 0.005). Conclusion: Coronary artery calcium scoring should not be the first radiologic diagnostic test to determine the risk of coronary artery disease in amyloidosis. [ABSTRACT FROM AUTHOR]

Copyright of Pocki is the property of Zaslavsky O.Yu and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The pathogenesis of autoinflammatory diseases has shed light on the concept of inflammation in general and on our understanding of the role of the innate immune system. The autoinflammatory diseases have a large spectrum with varying features of inflammation. The most common autoinflammatory diseases are those associated with periodic fevers. The delay in diagnosis of these four common diseases (familial Mediterranean fever, cryopyrin-associated periodic fever syndrome, mevalonate kinase deficiency, and TNF receptor-associated periodic fever syndrome) results in secondary amyloidosis of the kidney. The new work towards classification criteria for these diseases is presented. Recently a group of autoinflammatory diseases that are associated with vasculitis have also been identified. These are stimulators of interferon genes (STING)-associated vasculopathy of infancy (SAVI), which is a monogenic defect associated with excessive activity in interferon alpha and deficiency of adenosine deaminase 2, which is characterized by a polyarteritis nodosa-like picture. These monogenic diseases are now in our differential diagnosis of vasculitides. Secondary amyloidosis is a complication of autoinflammatory diseases. Understanding the inflammatory mechanisms in these diseases has led to the use of targeted biologics for this complication. It is hoped that enlightening the mechanisms underlying these monogenic autoinflammatory diseases will also teach us about the pathways in common diseases. [ABSTRACT FROM AUTHOR]

A review of the literature revealed secondary amyloidosis (AA) associated with lung cancer in only 14 cases. With respect to the histological subtype, 8 of the cases were squamous cell carcinoma (SCC).Herein, we describe a patient with pulmonary SCC-associated AA. The patient presented with severe nephrotic syndrome 3 years after the diagnosis of malignancy and had no history of chronic inflammatory diseases. Our reason for evaluating these cases is that they are rare in the literature, and most are SCC-associated AA. [ABSTRACT FROM AUTHOR]

Aim: The aim of the study is to compare cardiovascular findings in amyloidosis developed secondary to familial Mediterranean fever (FMF), with those of patient, and healthy control groups. Method: The study was performed on 32 secondary amyloid patients, 33 FMF patients and 65 healthy control individuals. Left ventricular functions were measured using two-dimensional, M-mode, conventional and tissue Doppler echocardiographic images. Aortic elasticity was analyzed using 2-D, M-mode echocardiographic images. Carotid-intima media thickness (CIMT) was measured using carotid artery B-mode ultrasonography. Results: Age, sex and serum lipids were comparable among groups (p>0.05). Aortic wall elasticity properties were similar between groups (p>0.05). Mitral annular E/A ratios were significantly lower in the amyloid group compared with controls ((p=0.004). CIMT was markedly higher in the amyloid group (p=0.001). In addition, CIMT values was higher in FMF patients than healthy controls (p=0.03). Ejection fraction and left ventricular mass index were similar among groups (p>0.05, for both). Conclusion: These findings suggested that subclinical myocardial involvement may be present in patients who developed amyloidosis secondary to FMF: It is noted that CIMT values were higher in patients with secondary amyloidosis. [ABSTRACT FROM AUTHOR]

Introduction: The most important complication of familial Mediterranean fever (FMF) is secondary amyloidosis, which can lead to kidney failure. Genetic variability in the genes of various components of the renin-angiotensin system may play a role in the pathogenesis of the kidney disorders.  The aim of the present study was to investigate the association between angiotensin converting enzyme (ACE) gene I/D variant and risk of developing FMF-related amyloidosis in Turkish patients.Materials and Methods: A total of 240 individuals consisting of 40 patients with FMF-related amyloidosis, 100 FMF patients without amyloidosis, and 100 healthy controls were recruited. For all of the participants, ACE I/D variant was detected by the polymerase chain reaction using specific primers.Results: A significant difference was found between the patients with FMF-related amyloidosis and the control group as for genotype distribution of ACE I/D variant (P < .05). The ACE D/D and I/D genotypes were more frequent in the patients with FMF-related amyloidosis while the I/I genotype was less frequent in the same patients. The FMF patients (with and without amyloidosis) had significantly higher percentages of the D/D and I/D genotypes than the healthy controls (P < .05). Comparison between the subgroups of FMF patients, divided into those with and without amyloidosis, yielded a significant correlation according to ID+II versus DD genotypes (P < .03, odds ratio, 3.24; 95% confidence interval, 1.05 to 12.01).  Conclusions. Based on these observations, the ACE I/D variant D/D genotypes implicate a possible risk in the FMF-related amyloidosis among Turkish population. [ABSTRACT FROM AUTHOR]

No abstract available. Article truncated after first page. A 53-year-old man presents with fatigue and dyspnea on exertion. An admission chest radiograph (Figure 1) was obtained. What is the best term or phrase used to describe the salient radiographic abnormality? 1. Diffuse thick-walled cavitary lesions; 2. Interstitial and alveolar pulmonary edema with effusions; 3. Miliary nodules; 4. Patchy consolidation; 5. Tension pneumothorax.

Common variable immunodeficiency (CVID) usually manifests in the second or third decade of life with recurrent bacterial infections and hypoglobulinemia. Secondary renal amyloidosis with history of pulmonary tuberculosis is rare in CVID, although T cell dysfunction has been reported in few CVID patients. A 40-year-old male was admitted to our hospital with a 3-month history of recurrent respiratory infections and persistent pitting pedal edema. His past history revealed 3 to 5 episodes of recurrent respiratory tract infections and diarrhoea each year since last 20 years. He had been successfully treated for sputum positive pulmonary tuberculosis 8 years back. Laboratory studies disclosed high erythrocyte sedimentation rate (ESR), hypoalbuminemia and nephrotic range proteinuria. Serum immunoglobulin levels were low. CD4/CD8 ratio and CD3 level was normal. C3 and C4 complement levels were normal. Biopsy revealed amyloid A (AA) positive secondary renal amyloidosis. Glomeruli showed variable widening of mesangial regions with deposition of periodic schiff stain (PAS) pale positive of pink matrix showing apple green birefringence on Congo-red staining. Immunohistochemistry was AA stain positive. Immunofluorescence microscopy revealed no staining with anti-human IgG, IgM, IgA, C3, C1q, kappa and lambda light chains antisera. Patient was treated symptomatically for respiratory tract infection and was discharged with low dose angiotensin receptor blocker. An old treated tuberculosis and chronic inflammation due to recurrent respiratory tract infections were thought to be responsible for AA amyloidosis. Thus pulmonary tuberculosis should be considered in differential diagnosis of secondary causes of AA renal amyloidosis in patients of CVID especially in endemic settings.

We report this case of secondary amyloidosis associated with Castleman's disease. A 51-year-old man presented with systemic symptoms of generalized weakness, fatigue, unintended weight loss, anorexia and progressively worsening abdominal distension. On examination he was found to have an indurated right-sided submandibular mass and tense ascites. He was found to have multiorgan dysfunction with deranged liver function tests and renal failure. Ascitic fluid analysis revealed evidence of spontaneous bacterial peritonitis. Biopsy of the submandibular mass revealed angiofollicular lymph node hyperplasia consistent with a diagnosis of Castleman's disease. A subsequent liver biopsy showed extensive deposition of amyloid protein. Bone marrow biopsy also showed the presence of amyloid and increased kappa light chain-restricted plasma cells. The patient was not considered a candidate for chemotherapy or solid organ transplantation in view of active sepsis and poor physical condition. Secondary systemic amyloidosis complicating Castleman's disease is very rare. Untreated secondary systemic amyloidosis often has a rapidly fatal course, such as seen in our patient.

Secondary amyloidosis is a consequence of chronic inflammatory diseases that results in deposition of amyloid in organs disrupting its function. Oral involvement with this process is exceedingly rare. A 63-year-old Pakistani female with a history of rheumatoid arthritis and ankylosing spondylitis was seen in the Oral Medicine Clinics for painful oral nodules that were diagnosed as AA-type, or secondary amyloidosis, following an incisional biopsy. Similar deposits were identified in vital organs on subsequent examination by the patient's physicians. Due to widespread systemic involvement, our patient died within 6-months of her diagnosis. CONCLUSION: This case serves as a reminder that oral presentations may be the first identifiable signs of systemic diseases. It also emphasizes the importance of multidisciplinary clinical practice, and the lack of it in our country. [ABSTRACT FROM AUTHOR]

Secondary amyloidosis is a rare but serious complication of inflammatory bowel disease that may influence the prognosis even more than the underlying disease. Due to a better knowledge of the association of secondary amyloidosis to inflammatory bowel disease, early diagnosis of this complication is becoming more frequent, but its treatment continues to pose a challenge. We report 4 cases of patients with Crohn's disease and amyloidosis diagnosed in the inflammatory bowel disease Units of Toledo and Ciudad Real, which represent 0.68% of the patients with Crohn's disease of our health areas. There have been not cases of amyloidosis in patients with ulcerative colitis. In our 4 patients the secondary amyloidosis was clearly related to Crohn's disease, which was often of fistulising type. The predominant clinical picture of amyloidosis was nephrotic syndrome. The patients responded to medical and surgical treatment of Crohn's disease and colchicine, which improved renal function in all cases except in one who required kidney transplantation.La amiloidosis sistémica adquirida es una complicación rara pero grave de la enfermedad inflamatoria intestinal crónica, pudiendo condicionar el pronóstico más que la propia enfermedad de base. Debido al mejor conocimiento de la asociación de amiloidosis secundaria a enfermedad inflamatoria intestinal, el diagnóstico temprano se hace cada vez con mayor frecuencia, pero su tratamiento continúa siendo un reto. Describimos 4 casos clínicos de pacientes con enfermedad de Crohn (EC) y amiloidosis diagnosticados en las Unidades de EIIC de Toledo y Ciudad Real, lo que representa el 0,68% de los caso de EC de nuestras áreas sanitarias. No se ha descrito ningún caso de amiloidosis en pacientes con colitis ulcerosa. En los 4 pacientes la AA estaba claramente relacionada con la EC, y predominaron las formas estenosantes-perforantes. El cuadro clínico de presentación de la amiloidosis en la mayoría de los casos fue un síndrome nefrótico. Los pacientes respondieron al tratamiento médico-quirúrgico de la EC y a la colchicina, con lo que mejoró la función renal en todos los casos salvo en uno que precisó trasplante renal.

Amyloidosis is a rare disorder characterized by the deposition of abnormal proteins in extracellular tissues, resulting in the dysfunction of vital organs and, eventually, death. The occurrence of amyloidosis due to primary Sjogren's syndrome (pSS) is a rare finding. This study describes a rare case of pSS complicated by amyloid-associated amyloidosis., Case Presentation: A 35-year-old male was diagnosed with nephrotic syndrome and secondary amyloidosis caused by pSS. He had microscopic hematuria, a creatinine level of 6.59 mg/dl, and an elevated erythrocyte sedimentation rate of 107 mm/hrs. Furthermore, investigations of antinuclear antibodies, antimitochondrial antibodies, SSA, SSA native, and Ro-52 recombinant as well as rheumatoid factor showed positive results. After establishing the diagnosis of pSS through clinical, physical, and laboratory assessments, a renal biopsy was performed, which revealed the occurrence of secondary amyloidosis., Clinical Discussion: The risk of developing secondary amyloidosis depends on the extent of elevated serum amyloid levels as well as persistent subclinical inflammation. The definitive diagnosis of amyloidosis requires histological confirmation of amyloid fibril deposition in tissue., Conclusion: Secondary renal amyloidosis is an unusual condition in patients with pSS. Still, it should be regarded in the differential diagnosis of patients with proteinuria and/or renal failure, and a renal biopsy should be performed., Competing Interests: The authors declare that they have no competing interests., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc.)

Background and Aims: Amyloidosis is a rare complication of inflammatory bowel disease [IBD]; its low prevalence has hindered both descriptive and therapeutic studies. The aim of this study was to estimate the prevalence of amyloidosis in IBD and the risk factors associated with this complication. Methods: This paper presents an observational study, followed by a systematic review of the epidemiological and clinical characteristics of the disease and a review of the diagnostic and therapeutic options. Results: The prevalence of amyloidosis among IBD patients is 0.53% (95% confidence interval [CI]: 0.32-0.75), although epidemiological data suggest that it may be under-diagnosed. The phenotype most frequently associated with amyloidosis is males with aggressive and extensive Crohn's disease, fistulising behaviour, perianal disease, and extra-intestinal complications, with the development of proteinuria and renal failure. Conclusions: Identifying risk factors of amyloidosis in IBD patients and screening for proteinuric renal dysfunction are useful to improve diagnostic accuracy. Referral of biopsies to a tertiary centre should also be considered, to improve diagnostic accuracy. Although there is no reliable evidence on the effectiveness of treatment, it seems reasonable to treat the underlying disease with potent immunosuppression to minimise inflammatory activity, thereby switching off amyloidogenesis. [ABSTRACT FROM AUTHOR]

In this multicenter, retrospective study, we evaluated the efficacy and safety of biologic therapies, including anti-TNFs, in secondary (AA) amyloidosis patients with ankylosing spondylitis (AS) and rheumatoid arthritis (RA). In addition, the frequency of secondary amyloidosis in RA and AS patients in a single center was estimated. Fifty-one AS (39M, 12F, mean age: 46.7) and 30 RA patients (11M, 19F, mean age: 51.7) with AA amyloidosis from 16 different centers in Turkey were included. Clinical and demographical features of patients were obtained from medical charts. A composite response index (CRI) to biologic therapy-based on creatinine level, proteinuria and disease activity-was used to evaluate the efficacy of treatment. The mean annual incidence of AA amyloidosis in RA and AS patients was 0.23 and 0.42/1000 patients/year, respectively. The point prevalence in RA and AS groups was 4.59 and 7.58/1000, respectively. In RA group with AA amyloidosis, effective response was obtained in 52.2 % of patients according to CRI. RA patients with RF positivity and more initial disease activity tended to have higher response rates to therapy ( p values, 0.069 and 0.056). After biologic therapy (median 17 months), two RA patients died and two developed tuberculosis. In AS group, 45.7 % of patients fulfilled the criteria of good response according to CRI. AS patients with higher CRP levels at the time of AA diagnosis and at the beginning of anti-TNF therapy had higher response rates ( p values, 0.011 and 0.017). During follow-up after anti-TNF therapy (median 38 months), one patient died and tuberculosis developed in two patients. Biologic therapy seems to be effective in at least half of RA and AS patients with AA amyloidosis. Tuberculosis was the most important safety concern. [ABSTRACT FROM AUTHOR]

ObjectivesThis study evaluated whether diffusion-weighted magnetic resonance imaging (DW-MRI) can be used to diagnose secondary renal amyloidosis looking specifically at the diagnostic efficacy of two apparent diffusion coefficient (ADC) measurement methods as they were used with DW-MRI.MethodsThe study included 24 amyloid nephropathy (AN) patients, 20 chronic kidney disease (CKD) patients, and 20 healthy volunteers (HV). ADC values were measured using two different methods: 1) the method of the region of interest indicators (ROIs) and 2) the method of drawing whole renal parenchyma (WP). The correlation between the two methods was evaluated.ResultsROIs could differentiate AN-CKD (p = 0.007). ROIs and WP could differentiate AN-HV (p < 0.05). However, none of the methods could differentiate CKD-HV (p > 0.05). The sensitivity and specificity of the ROIs method in differentiating AN from CKD patients for 1.8 × 10−3cutoff ADC values were 79% and 60% and for AN-HV patients 79% and 70%. ADC values of AN patients with GFR > 60 mL/min were lower than that of HV (p < 0.01).ConclusionDW-MRI is a useful and non-invasive diagnostic tool in diagnosing secondary renal amyloidosis and differentiating renal amyloidosis from other CKDs. ROIs had the highest sensitivity and specificity for assessing the involvement of renal amyloidosis. MRI diagnosis of AN may obviate a renal biopsy for diagnosis. [ABSTRACT FROM PUBLISHER]