ILD's! Flashcards by Jade Park

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

Youth: Sarcoidosis & Collagen vascular diseases

Older: smoking-related ILD’s/IPF

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How do PFT’s look w/ ILD’s?

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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What is Raynaud’s Phenomenon?

Exaggerated vascular response to cold T & emotional stress

Px w/ sharply demarcated color changes of skin/digits

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

Pulmonary HTN

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

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

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What lab work is req if Scleroderma is suspected?

CBC

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

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Is a biopsy necessary for autoimmune dx like Scleroderma?

No bc often clinical picture, radiology & lab testing suffice

Biopsy may hurt more than help, especially in impaired healing

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How does Systemic Sclerosis differ from Limited Scleroderma?

Systemic: ILD
Limited: Pulmonary HTN

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What is Systemic Sclerosis (Scleroderma)?

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

Pulmonary inv in >80%

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What other systems might be affected by Scleroderma?

GI/Renal

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What is a pathognomnic CXR finding for Pulmonary Alveolar Proteinosis?

Crazy Paving

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What is the difference between honey combing & crazy paving?

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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What is Pulmonary Alveolar Proteinosis?

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

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What is a unique ft of Pulmonary Alveolar Proteinosis?

Little to no lung inflammation, underlying lung architecture preserved

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What is the lipoproteinaceous material in PAP made of?

Surfactant phospholipid and apoproteins

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What signaling and players are involved in preventing PAP?

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

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

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

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What is congenital PAP?

Disorders of surfactant production

What is secondary PAP & what are its 4 causes?

(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

What are complications of PAP?

Hypoxemia, Dyspnea, & RF

How does pulmonary alveolar proteinosis present histologically?

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

How is PAP treated?

Treat underlying cause!

If GMCSF is 1⁰problem, replace it via inhalation

Serial bronchoalveolar lavage: wash out lipoproteinaceous products

How might cystic lung disease present acutely?

Pneumothorax

How does Lymphangioleiomyomatosis (LAM) appear on CT?

Diffuse cysts of uniform shape

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

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

What is the primary histopathological abnormality in LAM?

Proliferation of atypical SM (LAM) cells

What factor plays a key role in LAM pathogenesis?

Hormonal fluxes (ie: pregnancy, exogenous estrogens)

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

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

What are the Tx's of LAM?

Supportive measures Avoid smoking Immunosupression

What immunosuppressive can stabilize LAM?

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

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

Cysts in gross specimen

How does LAM present histologically?

Abnormal SM proliferation & spindle shaped SM cells all over lung

What does LAM stain w/?

HMB45: melanoma stain Brown= abnormal LAM/SM cells

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

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

What is Langerhans Cell Histiocytosis (LCH)?

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

What systems might LCH affect?

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

What systems does LCH spare?

Heart & kidneys

What is the origin of pathologic Langerhans cells?

Myeloid dendritic cells

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

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

How does LCH present histologically?

Langerhans cells w/ bean shaped nuclei Another name- "Eosinophilic Granuloma"

What is the Tx for smoking related ILD's?

Smoking cessation

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

Steroids may be useful but treat underlying dis & avoid exposure

What are Tx's for Pneumoconioses?

Trick qt- N/A!

What are Tx's for IPF?

Smoking cessation, newer agents (antifibrotics), transplant