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Block 3b1 PHM > ILD's! > Flashcards

ILD's! Flashcards

1
Q

What is the general distribution of ILD’s based on age?

A

Youth: Sarcoidosis & Collagen vascular diseases

Older: smoking-related ILD’s/IPF

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2
Q

How do PFT’s look w/ ILD’s?

A

Normal or High FEV1/FVC ratio

Low FEV1 and FVC

Low TLC \*confirms restriction\*
Low DLCO (non-SP)

Exercise-induced hypoxia

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3
Q

What is Raynaud’s Phenomenon?

A

Exaggerated vascular response to cold T & emotional stress

Px w/ sharply demarcated color changes of skin/digits

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4
Q

What condition is Raynaud’s Phenomenon closely associated w/?

A

Pulmonary HTN

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5
Q

What Tx may be given for Raynaud’s Phenomenon but why might this require monitoring?

A

Prostaglandin infusions may be req to restore flow but may req monitoring for hypotension

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6
Q

What lab work is req if Scleroderma is suspected?

A

CBC

Antiscleroderma ab’s: Anti Scl 70 & Anticentromere ab
Anti-nuclear ab’s

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7
Q

Is a biopsy necessary for autoimmune dx like Scleroderma?

A

No bc often clinical picture, radiology & lab testing suffice

Biopsy may hurt more than help, especially in impaired healing

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8
Q

How does Systemic Sclerosis differ from Limited Scleroderma?

A

Systemic: ILD
Limited: Pulmonary HTN

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9
Q

What is Systemic Sclerosis (Scleroderma)?

A

Autoimmune disease; heterogeneous group of conditions linked to thickened, sclerotic skin lesions

Pulmonary inv in >80%

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10
Q

What other systems might be affected by Scleroderma?

A

GI/Renal

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11
Q

What is a pathognomnic CXR finding for Pulmonary Alveolar Proteinosis?

A

Crazy Paving

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12
Q

What is the difference between honey combing & crazy paving?

A

Honey-combing is more basal & peripheral involving stacks of cells; larger than alveolar spaces bc lungs are destroyed; ground glass appearance

Crazy-paving: more central; CXR also w/ hyper-dense opacification

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13
Q

What is Pulmonary Alveolar Proteinosis?

A

Diffuse lung dis w/ accumulation of amorphous periodic acid Schiff (+) lipoproteinaceous material in distal air spaces

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14
Q

What is a unique ft of Pulmonary Alveolar Proteinosis?

A

Little to no lung inflammation, underlying lung architecture preserved

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15
Q

What is the lipoproteinaceous material in PAP made of?

A

Surfactant phospholipid and apoproteins

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16
Q

What signaling and players are involved in preventing PAP?

A

Macrophages aided by Granulocyte Macrophage Colony Stimulating Factor (GMCSF) to mop up surfactant and avoid excess

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17
Q

What is the infectious agent most commonly associated w/ PAP?

A

Nocardia: Gram (+), catalase (+) rod shaped bacteria- risk of infection!

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18
Q

What is congenital PAP?

A

Disorders of surfactant production

19
Q

What is secondary PAP & what are its 4 causes?

A

(More Common)
1) High dust exposure (ie: silica, Al, Ti)

2) Hematologic dyscrasia (ie: myelodysplastic syndrome, malignancy)
3) After allogeneic hemtaopoietic cell transplantation for myeloid malignancies
4) Rel deficiency of GM-CSF and related macrophage dysfunction

20
Q

What are complications of PAP?

A

Hypoxemia, Dyspnea, & RF

21
Q

How does pulmonary alveolar proteinosis present histologically?

A

Alveolar spaces filled w/ pink lipoproteinaceous surfactant, w/o inflammation and architecture preserved

22
Q

How is PAP treated?

A

Treat underlying cause!

If GMCSF is 10 problem, replace it via inhalation

Serial bronchoalveolar lavage: wash out lipoproteinaceous products

23
Q

How might cystic lung disease present acutely?

A

Pneumothorax

24
Q

How does Lymphangioleiomyomatosis (LAM) appear on CT?

A

Diffuse cysts of uniform shape

25
Q

What is Lymphangioleiomyomatosis (LAM) & who does it affectly primarily?

A

Rare multisystem disorder that affects lungs w/ characteristic cysts (but also kidneys); primarily of young women

26
Q

What is the primary histopathological abnormality in LAM?

A

Proliferation of atypical SM (LAM) cells

27
Q

What factor plays a key role in LAM pathogenesis?

A

Hormonal fluxes (ie: pregnancy, exogenous estrogens)

28
Q

What role does genetics play in LAM pathogenesis? What might these genes induce?

A

Excessive proliferation of LAM cells due to mutations in tuberous sclerosis complex (TSC) genes, esp TSC2

TSC can form multiple benign tumors in many systems->seizure, epilepsy

29
Q

What are the Tx’s of LAM?

A

Supportive measures

Avoid smoking

Immunosupression

30
Q

What immunosuppressive can stabilize LAM?

A

Sirolimus (mTOR inhibitor that blocks T cell activation and B cell differentiating by preventing IL-2 response)

31
Q

What might a LAM lung’s gross appearance look like?

A

Cysts in gross specimen

32
Q

How does LAM present histologically?

A

Abnormal SM proliferation & spindle shaped SM cells all over lung

33
Q

What does LAM stain w/?

A

HMB45: melanoma stain

Brown= abnormal LAM/SM cells

34
Q

What does Langerhans Cell Histiocytosis (LCH) look like on CT?

A

w/ bizarre shaped cysts & nodules, less regular than LAM

35
Q

What is Langerhans Cell Histiocytosis (LCH)?

A

Rare histiocytic disorder most commonly characterized by single or multiple osteolytic bone lesions w/ infiltration w/ histiocytes having bean-shaped nuclei on biopsy

36
Q

What systems might LCH affect?

A

Histiocytes, lymphocytes, macrophages & eosinophils may infiltrate every organ (skin, lymph nodes, lungs, thymus, liver, spleen, BM, CNS)

37
Q

What systems does LCH spare?

A

Heart & kidneys

38
Q

What is the origin of pathologic Langerhans cells?

A

Myeloid dendritic cells

39
Q

What features lead to a characteristic Dx of LCH that a biopsy may not even be req?

A

High resolution CT w/ multiple cysts & nodules w/ mid-upper zone predominance and intersitial thickening in a young smoker

40
Q

How does LCH present histologically?

A

Langerhans cells w/ bean shaped nuclei

Another name- “Eosinophilic Granuloma”

41
Q

What is the Tx for smoking related ILD’s?

A

Smoking cessation

42
Q

What are the Tx’s for ILD’s associated w/ CT disorders, Sarcoidosis, Radiation/Drug Exposure, HP, NSIP pattern?

A

Steroids may be useful but treat underlying dis & avoid exposure

43
Q

What are Tx’s for Pneumoconioses?

A

Trick qt- N/A!

44
Q

What are Tx’s for IPF?

A

Smoking cessation, newer agents (antifibrotics), transplant

Block 3b1 PHM (5 decks)

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