INTRODUCTION: Rheumatoid arthritis (RA) is one of systemic chronic progressive inflammatory disorders based on immunological disharmonies. Poorly controlled systemic inflammation in RA often leads to renal diseases such as secondary amyloidosis. CASE PRESENTATION: A 30-year-old man complained of swelling and tenderness of multiple joints gradually worsened the past 7 years. His laboratory examination showed anemia, positive rheumatoid factor (RF) and anti-citrullinated protein antibody (ACPA). C-reactive protein (CRP) was 48.7 mg/L (Normal value is <5 mg/L), increase in serum creatinine and protein was +3 in urine. His estimated glomerular filtration rate (e-GFR) was 58.3 mL/min/1.73 m2 Radiologic examinations of joints revealed features that support the diagnosis of rheumatoid arthritis. Renal biopsy was done revealed amyloid deposit. He was diagnosed with rheumatoid arthritis and secondary renal amyloidosis. CONCLUSION: Early proper diagnosis of RA is important and immunosuppressive drugs might slow disease progression by controlling the inflammatory process We discussed the importance of early diagnosis and the use of better treatment in managing RA to prevent renal amyloidosis.
Amyloidosis ; Arthritis, Rheumatoid ; Early Diagnosis

Amyloidosis ; classification ; diagnosis ; Cardiomyopathies ; classification ; diagnosis ; Humans

Study two cases of primary amyloidosis with the pathognomonic symptom of macroglossia , the diagnosis and treatment of primary amyloidosis. The first one presented with lesions of the tongue, peripheral neuropathy and the bone marrow aspiration revealed <30% plasmocyte with neither bone destruction nor heart, kidney injury. The second case showed diffuse infiltration of amyloid, not only caused the macroglossia but also led to the injury of the heart, pleura, kidneys, subcutaneous tissues, and the autonomic nervous system with postural hypotension
Amyloidosis ; Macroglossia ; Hypotension, Orthostatic ; diagnosis ; Therapeutics

Amyloidosis ; Biopsy ; Humans ; Liver Cirrhosis/diagnosis*

Adult ; Amyloidosis ; diagnosis ; pathology ; Female ; Humans ; Liver Diseases ; diagnosis ; pathology

Aged ; Amyloidosis ; complications ; diagnosis ; Diagnostic Errors ; Dyspnea ; etiology ; Edema ; etiology ; Female ; Humans ; Immunoglobulin Light-chain Amyloidosis

Primary systemic light chain amyloidosis or immunoglobulin light-chain amyloidosis (AL) is the most common type of systemic amyloidosis.AL is a proteotoxic clonal plasma cell disease, a hematological malignancy, characterised by overproduction of immunoglobulin light chains that form characteristic abnormally folded and aggregated, insoluble fibrillar deposits in various organs, including kidneys, heart, liver, and autonomic and peripheral nerves, etc, these processes lead to organ dysfunction and death. Systemic amyloidosis have various types with different causes, thereby its clinical diagnosis and treatment are more difficult. Recent developments on studies that have significantly aided the management of patients with AL include diagnostic techniques for definitive typing of amyloid deposits by using flow cytometry and immunophenotype analysis. These methods can detect abnormalities of bone marrow plasma cell clones, such as CD38(+), CD138(+), CD56(+), CD19(-) in AL patients. The monitoring abnormal plasma cells with immunoglobulin light chain restriction and abnormal plasma cell phenotypic characteristics contributes to the early diagnosis of AL and detection of minimal residual disease after treatment, which greatly improved AL treatment and prognosis. In this review the diagnosis and typing, clinical characteristics, flow cytometry, immunophenotyping of bone marrow cells, immunoglobulin light chain restriction and phenotypic characteristics of abnormal plasma cells of AL are briefly summarized.
Amyloidosis ; diagnosis ; Bone Marrow Cells ; cytology ; Humans ; Immunoglobulin Light-chain Amyloidosis ; Plasma Cells ; cytology

Abdominal Pain/etiology* ; Amyloidosis/diagnosis* ; Humans ; Immunoglobulin Light-chain Amyloidosis/complications* ; Immunoglobulin lambda-Chains

OBJECTIVETo analyze the endocardial, myocardial, and epicardial longitudinal systolic strain (LSsys) in the left ventricle (LV) segments and walls in patients with cardiac involvement due to primary amyloidosis (AL-CA) and hypertrophic cardiomyopathy (HCM).

METHODSTwenty patients with biopsy-proven AL-CA, 20 with asymmetric HCM, and 20 age-matched healthy volunteers were analyzed for their clinical characteristics and underwent conventional echocardiography for evaluating LV wall thickness, left atrial and ventricle size, systolic and diastolic function and 2-dimensional velocity vector imaging for evaluating the endocardial, myocardial and epicardial LSsys of the LV segments and walls. AL-CA and HCM patients also underwent cardiac magnetic resonance to evaluate the late gadolinium enhancement (LGE) features.

RESULTSCompared with the control group, AL-CA and HCM groups, with similar clinical symptoms and physical signs, both showed increased LV wall thickness, left atrial diameter, E/A ratio, septal E/e' ratio and the prevalence of granular sparkling. LV segments and walls endocardial LSsys were significantly lower in AL-CA patients than in HCM patients and the control subjects. The endocardial-epicardial LSsys difference in all the left ventricle walls were significantly smaller in AL-CA group than in the control group, but this difference appeared variable in HCM group. The LGE also presented with different features in AL-CA and HCM: AL-CA group showed subendocardial LGE in almost all the LV walls, but HCM group showed patchy LGE with a regional, multifocal distribution.

CONCLUSIONAL-CA is characterized by a significantly reduced endocardial LSsys in the LV segments and an uniform decrease of the endocardial-epicardial LSsys difference in all the LV walls, but the changes in HCM appear variable, and 2-dimensional velocity vector imaging is therefore a useful modality to differentiate AL-CA from HCM.

Amyloidosis ; diagnosis ; Cardiomyopathy, Hypertrophic ; diagnosis ; Diagnosis, Differential ; Diastole ; Echocardiography ; Heart Ventricles ; physiopathology ; Humans ; Immunoglobulin Light-chain Amyloidosis ; Systole

Systemic sclerosis as a connective tissue disease could affect all internal organs of the body and could also manifest as a cutaneous lesion. Cardiac involvement leading to cardiac manifestations in systemic sclerosis patients is not rare. However, cardiac amyloidosis combined with systemic sclerosis is extremely rare. Although there were no definite treatment options in this case, symptomatic treatment is the cornerstone of the management plan. In this case report, we described a correct diagnosis and symptomatic medical care of early reactive cardiac amyloidosis with systemic sclerosis and summarize the current state of the relevant literature.
Amyloidosis* ; Cardiomyopathy, Restrictive ; Connective Tissue Diseases ; Diagnosis* ; Humans ; Scleroderma, Systemic*