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Amyloidosis
Epidemiology
Clinical presentation
Diagnosis
- Extracellular deposit of insoluble polymeric protein fibrils in tissue & organs
- Can be primary (AL type) or secondary (AA) to chronic inflammatory conditions such as:
- Inflammatory arthritis (e.g RA)
- Chronic infections (e.g bronchiectasis, tuberculosis, osteomyelitis)
- Inflammatory bowel disease (e.g Crohn's disease)
- Malignancy (e.g. lymphoma)
- Vasculitis
Clinical presentation
- Asymptomatic proteinuria or nephrotic syndrome
- Restrictive cardiomyopathy
- Hepatomegaly
- Peripheral neuropathy &/or autonomic neuropathy
- Visible organ enlargement (e.g. macroglossia)
- Bleeding diathesis
- Waxy thickening, easy bruising of skin
Diagnosis
- Tissue biopsy (e.g. abdominal fat pad)
List of Granulomatous diseases
- Bartonella henselae(cat scratch disease)
- Berylliosis
- Churg-Strauss syndrome
- Crohn disease
- Francisella tularensis
- Fungal infections (e.g., histoplasmosis,
- blastomycosis)
- Granulomatosis with polyangiitis (Wegener)
- Listeria monocytogenes(granulomatosis
- infantiseptica)
- M. leprae(leprosy; Hansen disease)
- M. tuberculosis
- Treponema pallidum(tertiary syphilis)
- Sarcoidosis
- Schistosomiasis
Th1cells secrete γ-interferon, activating macrophages. TNF-αfrom macrophages
induce and maintain granuloma formation. Anti-TNF drugs can, as a side effect, cause
sequestering granulomas to breakdown, leading to disseminated disease. Always test for
latent TB before starting anti-TNF therapy.
Features of constrictive pericarditis
Features of constrictive pericarditis
Etiology
- Idiopathic or viral pericarditis
- Cardiac surgery or radiation therapy
- Tuberculous pericarditis (in endemic areas)
Clinical presentation
- Fatigue and dyspnea on exertion
- Peripheral edema and ascites
- Increased JVP
- Pericardial knock may be heard
- Pulsus paradoxus
- Kussmaul's sign
Diagnostic findings
- ECG may be nonspecific or show atrial fibrillation or low voltage QRS complex
- Imaging shows pericardial thickening and calcification
- Jugular venous pulse tracing shows prominent x and y decents.
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