These two patients presented with typical waxy lesions on face. Cutis verticis gyrata was present in one case and carpal tunnel syndrome was seen in other case as an additional diagnostic clue. Macroglossia was present in all three cases. [ncbi.nlm.nih.gov]

• Weight Loss

  Later, she developed dyspepsia and weight loss. An oesophagogastroduodenoscopy (OGD) showed Helicobacter pylori negative erosive gastritis with a benign duodenal ulcer. Whole body CT scan was normal. [ncbi.nlm.nih.gov]

  Case Report A 60-year-old fair complexioned woman was admitted to our hospital because of dyspnoea specially on recumbency, pedal oedema, severe weakness confining her to bed and wheel-chair, weight loss, dysphagia and hoarseness of voice for the last [ijdvl.com]

  A 51-year-old man presented with systemic symptoms of generalized weakness, fatigue, unintended weight loss, anorexia and progressively worsening abdominal distension. [karger.com]

  loss The condition that is causing the amyloidosis should be treated. [medlineplus.gov]

• Fatigue

  These deposits damage normal tissues, leading to the symptoms or signs of this illness: Bleeding in the skin Fatigue Irregular heartbeat Numbness of hands and feet Rash Shortness of breath Swallowing difficulties Swollen arms or legs Swollen tongue Weak [medlineplus.gov]

  We report a case of 68 years old male presented with generalized weakness, fatigue, anorexia and impaired taste sensation. [edoj.org.eg]

  Unusual longivity for 19 years have been reported with combined melphalan, prednisolone and colchicine treatment and is the longest reported in literature. [5] Because presenting symptoms are rather commonly nonspecific (including fatigue, weight loss [ijdvl.com]

• Congestive Heart Failure

  We present a case of primary systemic amyloidosis causing constrictive pericarditis (CP) and congestive heart failure without clinical evidence of endomyocardial deposition. [ncbi.nlm.nih.gov]

  Results of a chest x-ray noted bilateral pleural effusions consistent with congestive heart failure. [mdedge.com]

  For example, kidney failure can be treated with dialysis and congestive heart failure with diuretics. Specific treatment Chemotherapy with melphalan and prednisone or dexamethasone (oral corticosteroids) can control systemic amyloidosis. [dermnetnz.org]

  Cardiac involvement, often with congestive heart failure, is a common presentation. The electrocardiogram may show low voltage with a pattern of myocardial infarction. [web.archive.org]

• Leg Edema

  During this observation period, the level of IgE gradually increased and, at 2 years after the initial diagnosis, bilateral leg edema and ascites with marked hepatosplenomegaly appeared. [ncbi.nlm.nih.gov]

  Case report and management: We report a case in a 66-year-old patient with legs edema, inappetence and frothy urine for 2 weeks. The patient was diagnosed type 2 diabetes on routine testing 10 years ago without any symptoms. [omicsonline.org]

  NT-proBNP is another “biomarker” that should be performed, especially if a person has symptoms such as swelling in the legs (edema), difficulty breathing, shortness of breath, and fatigue. It is used to detect heart stress or strain. [amyloidosis.org]

• Hodgkin Lymphoma

  Systemic AL amyloidosis due to non-Hodgkin's lymphoma: an unusual clinicopathologic association. Br J Haematol. 2004 ; 124 : 309 -314. Comenzo RL, Zhang Y, Martinez C, Osman K, Herrera GA. [doi.org]

  Palavras-Chave: Amiloidose; Cútis laxa; Mieloma múltiplo; Mucinoses INTRODUCTION Systemic amyloidosis may occur associated to clonal lymphoid diseases in which immunoglobulins are produced, including non-Hodgkin's lymphoma, Waldenström's macroglobulinemia [scielo.br]

  Although AL amyloidosis typically is viewed as a rare disease, its incidence is similar to that of Hodgkin’s lymphoma or chronic myelogenous leukemia ( 2 ). [cjasn.asnjournals.org]

• Pleural Effusion

  We report the case of a 73-year-old woman with primary systemic AL and persistent bilateral pleural effusions, refractory to diuresis and repeated thoracenteses. [ncbi.nlm.nih.gov]

  His skiagram chest showed minimal left sided pleural effusion which had no pus cells despite high protein content. The diagnosis was established on the basis of classical cutaneous lesions. [edoj.org.eg]

  Results of a chest x-ray noted bilateral pleural effusions consistent with congestive heart failure. [mdedge.com]

• Dyspnea

  A patient who presented with dyspnea and suspected interstitial pulmonary fibrosis suffered a fatal pulmonary haemorrhage with no feasible cause for bleeding. [ncbi.nlm.nih.gov]

  Some of the more common findings during a review of systems include the following: weight loss, weakness/fatigue, dyspnea, edema, paresthesias, light-headedness, syncope, constipation/diarrhea, and hoarseness/change of voice. [clinicaladvisor.com]

  Response to treatment Fourteen patients had a 50% or greater reduction of proteinuria; 6 had a 2-mm or more decrease in IVS thickness associated with resolution of heart failure; 1 patient, with lung involvement, had complete resolution of dyspnea and [doi.org]

• Cough

  Purpura of the eyelids following pinching (pinch purpura) and periorbital purpura following coughing, vomiting, the Vulsalva manoeuver, forced expiration spirometric testing and proctoscopy (post proctoscopic palpabral purpura PPP) are very characteristic [ijdvl.com]

  Periorbital purpura (raccoon eyes) can be elicited by increased intrathoracic pressure as a result of the Valsalva maneuver and coughing. Amyloid infiltration of blood vessels also manifests as petechiae and ecchymoses. [clinicaladvisor.com]

  Amyloid deposits cause weakening of the blood vessels, which may rupture after minor trauma, such as sneezing or coughing. [merckmanuals.com]

  Periorbital purpura is a result of capillary fragility and may appear after coughing, sneezing, or straining for a bowel movement. Not infrequently, purpuric lesions may arise after such simple actions as rubbing the eyelids. [rarediseases.org]

• Delayed Gastric Emptying

  Autonomic nervous system involvement by AL amyloidosis can lead to orthostatic hypotension, early satiety as a result of delayed gastric emptying, erectile dysfunction, and intestinal motility issues. [doi.org]

• Macroglossia

  Macroglossia was present in all three cases. Diagnosis was confirmed in all three cases by biopsy using haematoxylin and eosin staining and Congo red staining. Polarized microscopy was not done because of unavailability. [ncbi.nlm.nih.gov]

  In addition, the classical triad of carpal tunnel syndrome, macroglossia, and specific mucocutaneous lesions, seen in half of the patients, is absent. [e-ijd.org]

  The final diagnosis was made because of the following criteria: missing monoclonal gammopathy, no periorbital bleeding and macroglossia, and presence of an amyloidogenic mutation. Of the 5 remaining patients, 4 had AL amyloidosis. [doi.org]

  Amyloidosis deposition in the tongue of multiple myeloma occurs frequently and can result in macroglossia, which is the most common oral finding [ 7, 8, 9 ]. [edoj.org.eg]

• Jaw Claudication

  Jaw claudication in primary amyloidosis: unusual presentation of a rare disease. J. Rheumatol. 30, 2283–2286 (2003). 20. Stebbing, J., Buetens, O., Hellman, D. & Stone, J. [nature.com]

  claudication, skin lesions EMG Carpal tunnel syndrome Direct biopsy Histological Selecting biopsy site In the 1960s, rectal and gingival mucosa biopsies were considered useful indirect alternatives to deep organ biopsies for the diagnosis of amyloidosis [ncbi.nlm.nih.gov]

• Hypotension

  On examination she had postural hypotension, raised jugular venous pressure, hepatomegaly and pitting pedal oedema. Electrocardiogram (ECG) showed sinus tachycardia, low QRS voltage and a Q-wave in precordial leads V1–V4. [bjcardio.co.uk]

  Other grade 3 toxicities were diarrhea, constipation and orthostatic hypotension. [doi.org]

  Carpel tunnel syndrome is present in 25% of cases, sensory neuropathy (18%), postural hypotension (16%) are the other common manifestations. [edoj.org.eg]

  […] colchicine treatment and is the longest reported in literature. [5] Because presenting symptoms are rather commonly nonspecific (including fatigue, weight loss, paresthesia, hoarseness, oedema, dyspnoea and lightheadedness or syncope secondary to orthostatic hypotension [ijdvl.com]

• Tachycardia

  Further examinations were planned but she died of ventricular tachycardia attack. Pathological autopsy revealed primary systemic amyloidosis. [ncbi.nlm.nih.gov]

  Electrocardiogram (ECG) showed sinus tachycardia, low QRS voltage and a Q-wave in precordial leads V1–V4. A coronary angiogram was normal. IgG kappa monoclonal protein was detected in her serum and urine Figure 1. [bjcardio.co.uk]

  […] death in AL, 8 and, in our preliminary experience with conventional high-dose dexamethasone, we observed a relevant proportion of treatment-related fatal arrhythmias. 6 Therefore, patients in whom the Holter ECG detected couplets and/or ventricular tachycardia [doi.org]

• Hepatomegaly

  Examination and tests in this patient also revealed anemia, hepatomegaly, infiltrative cardiomyopathy, polyneuropathy and immunoglobulin l deposition, favoring a diagnosis of primary amyloidosis (AL type). [ncbi.nlm.nih.gov]

  Key words: AL Amyloidosis, Proteinuria, Hepatomegaly Advertisement Journal of Contemporary Medical Education SUBMIT YOUR ARTICLE NOW [scopemed.org]

  Physical examination at the time was significant for a firm, indurated right-sided submandibular mass, hepatomegaly and mild epigastric tenderness. [karger.com]

  On examination she had postural hypotension, raised jugular venous pressure, hepatomegaly and pitting pedal oedema. Electrocardiogram (ECG) showed sinus tachycardia, low QRS voltage and a Q-wave in precordial leads V1–V4. [bjcardio.co.uk]

• Purpura

  KEYWORDS: Bullae; Oral purpura; Systemic amyloidosis [ncbi.nlm.nih.gov]

  Purpura of the eyelids following pinching (pinch purpura) and periorbital purpura following coughing, vomiting, the Vulsalva manoeuver, forced expiration spirometric testing and proctoscopy (post proctoscopic palpabral purpura PPP) are very characteristic [ijdvl.com]

• Peripheral Neuropathy

  Chronic peripheral neuropathy occurs as a manifestation of AL amyloidosis in 25 % of cases. It may exceptionally be complicated by neuroarthropathy. [ncbi.nlm.nih.gov]

  Neurologic symptoms include a sensory peripheral neuropathy, presenting in a stocking and glove distribution. An "idiopathic" carpal tunnel syndrome can also occur. [visualdx.com]

  Cardiomyopathy and peripheral neuropathy were identified in only systemic amyloidosis cohort (65% and 50%, respectively). [n.neurology.org]

  One was African-American, one had peripheral neuropathy, and the third was referred for evaluation of hereditary amyloidosis and was diagnosed with Durie-Salmon stage I multiple myeloma. [doi.org]

• Kidney Failure

  Fifty-three percent of deaths were due to cardiac amyloid, 12% to respiratory failure, 7% to macroglobulinemia, 7% to liver failure, and 7% to kidney failure. [jco.ascopubs.org]

  Complications such as heart failure, kidney failure, and other problems can sometimes be treated, when needed. How well you do depends on which organs are affected. Heart and kidney involvement may lead to organ failure and death. [nlm.nih.gov]

  Possible Complications Health problems that may result from secondary systemic amyloidosis include: Endocrine failure Heart failure Kidney failure Respiratory failure When to Contact a Medical Professional Call your provider if you have symptoms of this [mountsinai.org]

  Symptoms of kidney disease and kidney failure can include fluid retention, swelling, and shortness of breath.In addition to kidneys, AL amyloidosis may affect the heart, peripheral nervous system, gastrointestinal tract, blood, lungs and skin. [en.wikipedia.org]

Further workup demonstrated a monoclonal gammopathy consistent with systemic amyloidosis. This case demonstrates the need to consider amyloidosis in the differential diagnosis of patients presenting with an atypical acquired strabismus. [ncbi.nlm.nih.gov]

[…] myeloma-associated amyloidosis were positive for amyloid in 47% of cases (16/34) in one study6 and 100% of cases (16/16) in another study.7 Although positive biopsy results that are characteristic of different types of amyloidosis can be seen, a systemic workup [mdedge.com]

BM examination should be part of the diagnostic workup for all IgA and IgM M-proteins. [doi.org]

• Pericardial Effusion

  There are no reports of primary systemic amyloidosis with hemorrhagic pericardial effusion. We report this rare case with pathological consideration. [Indexed for MEDLINE] Free full text [ncbi.nlm.nih.gov]

  There was thickening of the cardiac valves and atrial septum, dilatation of the atria, inferior vena cava and hepatic veins, with systolic flow reversals, and a small circumferential pericardial effusion. [bjcardio.co.uk]

  He had an elevated jugular venous pressure but the heart sounds were normal; there were no murmurs or pericardial effusion noted. The remaining systemic examination was within normal limits. [rossscience.org]

  A small area of pericardial effusion was found. Ultrasound showed hepatomegaly (the diameter of right lobe was 22 cm) with homogeneous echo. [omicsonline.org]

• Thrombocytosis

  Her past medical history was remarkable for membranoproliferative glomerulonephritis, hypertension, pulmonary hypertension, and anemia of thrombocytosis. [mdedge.com]

• Amyloid Plaque

  Co-localization of cholesterol, apolipoprotein E and fibrillar Aβ in amyloid plaques. Mol. Brain. Res. 110, 119–125 (2003). 61. Solé-Domènech, S. et al. [doi.org]

  Co-localization of cholesterol, apolipoprotein E and fibrillar Aβ in amyloid plaques. Mol. Brain. Res. 110, 119–125 (2003). [ PubMed ] [ Google Scholar ] Solé-Domènech S. et al. [ncbi.nlm.nih.gov]

  Although the causative protein is the main constituent of amyloid plaques, the amyloid proteome is complex and a number of other proteins are frequently co-deposited. [haematologica.org]

  Note that in Alzheimer’s disease, there are “amyloid plaques” in the brain, but these are not formed from light chains, and AL patients have no increased susceptibility to Alzheimer’s disease, compared with the general population. [amyloidosis.org]

• Low-Voltage Electrocardiogram

  Amyloidosis should be suspected in certain clinical settings, including nephrotic syndrome with albuminuria rather than Bence Jones proteinuria, peripheral or autonomic neuropathy, diastolic heart failure with normal- or low-voltage electrocardiogram [hematologyandoncology.net]

• Pleural Effusion

  We report the case of a 73-year-old woman with primary systemic AL and persistent bilateral pleural effusions, refractory to diuresis and repeated thoracenteses. [ncbi.nlm.nih.gov]

  His skiagram chest showed minimal left sided pleural effusion which had no pus cells despite high protein content. The diagnosis was established on the basis of classical cutaneous lesions. [edoj.org.eg]

  Results of a chest x-ray noted bilateral pleural effusions consistent with congestive heart failure. [mdedge.com]

Only a few studies have shown the role of TNFα ihibitors in the treatment of AA amyloidosis over a long term period. [ncbi.nlm.nih.gov]

Delay in treatment worsens the prognosis. KEYWORDS: Amylosis; Charcot arthropathy; Diabetic foot; MRI; Nerve biopsy; Neuroarthropathy [ncbi.nlm.nih.gov]

Abstract Amyloidosis is a broad and complex class of diseases that comprises several etiologies, many manifestations, and a diversity of outcomes. [ncbi.nlm.nih.gov]

In the differential diagnosis of ascites, one seldom considers systemic amyloidosis as a potential etiologic factor. [jamanetwork.com]

A patient with nephrotic syndrome, without a clear etiology, should be further evaluated for primary systemic amyloidosis. [clinicaladvisor.com]

BACKGROUND: The epidemiology of systemic amyloidosis has been changing in the last decades. [ncbi.nlm.nih.gov]

"The epidemiology of AL and AA amyloidosis". Baillieres Clin Rheumatol. vol. 8. 1994. pp. 627-34. [clinicaladvisor.com]

A recent review of this complication of the disease hypothesized that the pathophysiology of these effusions is pleural amyloid deposition, disrupting lymphatic drainage. [ncbi.nlm.nih.gov]

Perhaps a more thorough study of the pathophysiology linking these two entities would open the way for the development of more effective therapeutic tools. [revistanefrologia.com]

Islet amyloid and type 2 diabetes; from molecular misfolding to islet pathophysiology. Biochim Biophys Acta. 2001;1537:179-203. Sanchorawala V, Wright DG, Seldin DC, et al. [rarediseases.org]

Pathophysiology The exact mechanism that promotes the conversion of immunoglobulin light chains into fibrils is also not entirely clear. [clinicaladvisor.com]

Early medical intervention to control chronic inflammation is imperative and could prevent morbidity and mortality related to the development of secondary amyloidosis. [ncbi.nlm.nih.gov]

This may help prevent amyloidosis. References Gertz MA. Amyloidosis. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 188. Version Info Last reviewed on: 5/21/2017 Reviewed by: Laura J. [mountsinai.org]

This may help prevent amyloidosis. Amyloidosis - secondary systemic Gertz MA. Amyloidosis. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 188. Updated by: Laura J. [medlineplus.gov]