Studydroid is shutting down on January 1st, 2019

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Histiocytosis
group of diverse disorders with a common primary event: the accumulation and infiltration of monocytes, macrophages, and dendritic cells in the affected tissues
Histiocyte society classification
Dendritic cell related
Macrophage related
Malignant disorders
Histiocytosis - dendritic related


  • Langerhans cell histiocytosis


  • Xanthogranuloma

Histiocytosis - Macrophage related


  • Familial and reactive hemophagocytic lymphohistiocytosis (HLH)


  • Sinus histiocytosis with massive lymphadenopathy (rosai dorfman disease)

Histiocytosis - malignant diorders



  • Monocyte related, monocytic leukemia



  • Dendritic-cell related



  • Localized or macrophage related



  • Disseminated (malignant histiocytosis)

Langerhans cell histiocytosis
    previous classification
 formerly was divided into 3 disease categories: eosinophilic granuloma, Hand-Schüller-Christian disease, and Letterer-Siwe disease
Langerhans cell histiocytosis - classification
Unifocal
Multifocal, unisystem
Multifocal,multisystem
Langerhans histiocytosis symptoms
provokes a non-specific inflammatory response, which includes fever, lethargy, and weight loss

Deposits - bone,skin,bone marrow, lymph nodes,endocrime - esp pit,lungs, less common GI tract, CNS
Langerhans histiocytosis treatment
Solitary lesion may be amenable through excision or limited radiation
systemic diseases often require chemotherapy. Use of systemic steroid is common, singly or adjunct to chemotherapy. Local steroid cream is applied to skin lesions.
Sarcoidosis

•Multi system non caseating granulomatous disease
Sarcoidosis symptoms

•50% Asymptomatic – incidental finding CXR

•Lungs >90%


Fatigue


Skin


Lymphadenopathy


Ocular,hepatic,renal,HPB,neurological,cardiac,


musculoskeletal



Sarcoidosis diagnosis
Histology

ACE

CXR
Sarcoidosis Management
60% asymptomatic, 60%of these resolve spontaneously

Treat when organ function threatened

Steroids, also chemotherapy agents


Amyloidosis
Abnormal deposition of fibrillar protein and polysaccharide complexes

modern classification of amyloid disease tends to use an abbreviation of the protein that makes the majority of deposits, prefixed with the letter A. For example, amyloidosis caused by"Transthyretin"  is termed "ATTR"
Older Amyloidosis classification
Primary or secondary
Systemic or localised
Amyloidosis Epidemiology
More common africo americans, puertoricans 3-4 decade
Amyloidosis H&N symptoms
macroglossia

vocal cord deposition

anterior subglottic mass,orbital deposition
Amyloidosis Diagnosis
biopsy, stain with congo red
Amyloidosis treatment
Conservative excision if symptomatic
Polyarteritis nodosa
systemic vasculitis characterized by necrotizing inflammatory lesions that affect medium-sized and small muscular arteries, preferentially at vessel bifurcations, resulting in microaneurysm formation, aneurysmal rupture with hemorrhage, thrombosis, and, consequently, organ ischemia or infarction.
Polyarteritis nodosa
male>female,30% active hep B infection
Polyarteritis nodosa symptoms
symptoms result from ischaemic damage to affected organs, often the skin, heart, kidneys, and nervous system.

typical PAN patient might present with fever, night sweats, weight loss, skin/mucosal ulcerations or tender nodules, and severe muscle and joint pains developing over weeks or months.
Polyarteritis nodosa diagnosis
3/10 American College of Rheumatology (ACR) established criteria 
Polyarteritis nodosa treatment
Steroids, cyclophosphamide
=90% remission/cure

Systemic lupus erythematosis(SLE)
autoimmune disorder characterized by multisystem microvascular inflammation
SLE Aetiology
Genetic predisposition

EBV

?silica dust & smoking

Drug related

Hormonal - Increased female, male klinefelters
SLE symptoms
related to immune complex deposition

classic = joint pain fever, rash woman childbearing age


  • Constitutional,Musculoskeletal,Dermatologic


  • Renal,Neuropsychiatric,Pulmonary


  • Gastrointestinal,Cardiac,Hematologic
    Oral ulcers





SLE diagnosis
ana, anti ds-dna

4/11 American College of rheumatology criteria


SLE treatment
Steroids,cyclophosphamide

Prognosis variable
Wegeners Granomatosis
idiopathic vasculitis mainly affecting upper respiratory tract, lungs and kidney
 
Acute inflammation with necrotising granulomatous angiitis involving small dermal vessels
Wegeners Granulomatosis symptoms
General - loss of apetite, fever, fatigue

Upper rep tract - septal ulcer/perf,epistaxsis, subglottic stenosis,sinusitis etc

Lungs

Kidneys

Musculoskeletal

Eyes

Skin

Heart

Nervous sytem





Wegeners Granulomatosis Diagnosis
Histology

C ANCA,PR3
Wegeners Granulomatosis Treatment

•Prednisilone

•Cyclophosphamide

•Methotrexate
Azathioprine
Wegeners Granulomatosis Prognosis
–No treatment
•90% mortality/2 yrs
–Treatment
•90% in remission/2 yrs
Scleroderma
Autoimmune connective tissue disease
Excess collagen deposition
Scleroderma types
1.localised scleroderma
-skin only
2.systemic scleroderma
-systemic organs and skin
Systemic scleroderma
1.Limited cutaneous systemic scleroderma
-skin limited to hands/feet,face, lower arms/legs
-perv crest
2.diffuse cutaneous systemic scleroderma
-extends other parts
-also crest
Scleroderma tests
ANA
Nail fold capillary biopsy

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