ISCEE ISCEE Respiratory Respiratory

SystemSystem2323rdrd Nov 2010 Nov 2010

CASE PRESENTATION 1CASE PRESENTATION 1 Mrs R, 44 years old lady with no previous medical Mrs R, 44 years old lady with no previous medical

or surgical historyor surgical history Main complaint was painful red eye for one year Main complaint was painful red eye for one year

associated with discomfort, photophobia and associated with discomfort, photophobia and blurred vision.blurred vision.

Seen by ophthalmologist and diagnosed with Seen by ophthalmologist and diagnosed with bilateral chronic uveitisbilateral chronic uveitis

She was then investigated to determine aetiologyShe was then investigated to determine aetiology She has a history of Bells Palsy which resolved She has a history of Bells Palsy which resolved

spontaneously after one week and a history of spontaneously after one week and a history of hyper-pigmented scaly lesion on forehead.hyper-pigmented scaly lesion on forehead.

There is no history of cough or dyspnoea.There is no history of cough or dyspnoea. No history suggestive of TBNo history suggestive of TB No history of joint pain.No history of joint pain.

ExaminationExamination On examination she was a well looking young, not On examination she was a well looking young, not

dyspnoeic,dyspnoeic, BP 129/88BP 129/88 PR 93/minutePR 93/minute No significant lymphadenopathyNo significant lymphadenopathy Hyper pigmented lesions on the foreheadHyper pigmented lesions on the forehead Chest clearChest clear CVS :NADCVS :NAD Abdomen no organomegalyAbdomen no organomegaly Musculoskeletal system: no arthritis and no muscle weaknessMusculoskeletal system: no arthritis and no muscle weakness CNS: NADCNS: NAD No cranial nerve palsy, no peripheral neuropathyNo cranial nerve palsy, no peripheral neuropathy

InvestigationInvestigation

FBC: NormalFBC: Normal U&E: NormalU&E: Normal LFT: NormalLFT: Normal CALCIUM LEVEL: Slightly raisedCALCIUM LEVEL: Slightly raised ESR: 40mm per hourESR: 40mm per hour ANF: NegativeANF: Negative SACE LEVEL: 111 (Normal less than 52)SACE LEVEL: 111 (Normal less than 52) LUNG FUNCTION TEST: NormalLUNG FUNCTION TEST: Normal CHEST X RAY (next slide)CHEST X RAY (next slide)

SARCOIDOSISSARCOIDOSIS

Multisystem inflammatory disease of Multisystem inflammatory disease of unknown etiology that predominantly unknown etiology that predominantly affects the lungs.affects the lungs.

Manifested by the presence of non-Manifested by the presence of non-caseating granulomas (NCGs) that may caseating granulomas (NCGs) that may affect any organ systemaffect any organ system

The many forms and presentation of this The many forms and presentation of this disease and the lack of a single disease and the lack of a single diagnostic test can make the diagnosis diagnostic test can make the diagnosis challengingchallenging

The lungs are involved in more than 90 The lungs are involved in more than 90 percent of patients, with sarcoid usually percent of patients, with sarcoid usually presenting as interstitial disease.presenting as interstitial disease.

Symptoms are dry cough, dyspnea, and Symptoms are dry cough, dyspnea, and chest discomfort.chest discomfort.

Pulmonary sarcoidosis has an Pulmonary sarcoidosis has an unpredictable course that may result in unpredictable course that may result in spontaneous remission or lead to spontaneous remission or lead to progressive loss of lung function with progressive loss of lung function with fibrosis.fibrosis.

Airway involvement can occur and may Airway involvement can occur and may result in airflow limitation, persistent result in airflow limitation, persistent cough and, in severe cases, bronchiectasis.cough and, in severe cases, bronchiectasis.

TreatmentTreatment The majority of patients will have spontaneous remission The majority of patients will have spontaneous remission

and a generally benign clinical course.and a generally benign clinical course. Treatment is reserved forTreatment is reserved for

patients with worsening pulmonary function testspatients with worsening pulmonary function tests patients with worsening pulmonary symptoms (cough, patients with worsening pulmonary symptoms (cough,

shortness of breath, chest pain or hemoptysis) andshortness of breath, chest pain or hemoptysis) and patients with extra pulmonary sarcoidosis including patients with extra pulmonary sarcoidosis including

arthritis, neuropathy, cardiac and renal sarcoid, also in,arthritis, neuropathy, cardiac and renal sarcoid, also in, patient with intractable fatigue, weakness or fever.patient with intractable fatigue, weakness or fever.

Corticosteroids are the mainstay of therapy.Corticosteroids are the mainstay of therapy. Generally, prednisone given daily and then tapered Generally, prednisone given daily and then tapered

over a 6-month course is adequate for pulmonary over a 6-month course is adequate for pulmonary disease.disease.

EPIDEMIOLOGYEPIDEMIOLOGY

Sarcoidosis affects men and women of all races Sarcoidosis affects men and women of all races and agesand ages

Usually presents in adult younger than 40 years Usually presents in adult younger than 40 years more frequent between 20 - 29 years, and more frequent between 20 - 29 years, and slightly more predominant in women than in slightly more predominant in women than in men.men.

Course of sarcoidosis is variable, ranging from Course of sarcoidosis is variable, ranging from self limited acute disease to a chronic self limited acute disease to a chronic debilatating disease.debilatating disease.

Spontaneous remissions occur in nearly two Spontaneous remissions occur in nearly two thirds of patients.thirds of patients.

CASE 2CASE 2

Case Presentation 2Case Presentation 2

63 years old female presented with gradual onset 63 years old female presented with gradual onset SOB over the last 6 months. SOB over the last 6 months.

She is known to suffer from Rheumatoid arthritis for She is known to suffer from Rheumatoid arthritis for the last 3 years and has been on treatment with the last 3 years and has been on treatment with methotrexate for the same. methotrexate for the same.

She has previously had an episode of pleural effusion She has previously had an episode of pleural effusion which was drained and investigated but was found to which was drained and investigated but was found to be an exudate with high protein and low glucose. be an exudate with high protein and low glucose.

On examination she had dull percussion note and On examination she had dull percussion note and absent air entry in her left lower zone of the chest. absent air entry in her left lower zone of the chest.

Patient’s bloods were within normal limits.Patient’s bloods were within normal limits. Chest x ray confirmed a pleural effusion. Chest x ray confirmed a pleural effusion. 

OverviewOverview Major catagories of pulmonary disease Major catagories of pulmonary disease

associated with RA:associated with RA: Pleural effusionPleural effusion Nodular lung diseaseNodular lung disease Diffuse interstitial fibrosisDiffuse interstitial fibrosis BOOP (bronchiolitis obliterans organizing BOOP (bronchiolitis obliterans organizing

pneumonia)pneumonia) Pulmonary vasculitisPulmonary vasculitis Alveolar hemmorhageAlveolar hemmorhage Obstructive diseaseObstructive disease InfectionsInfections

Rheumatoid Arthritis Rheumatoid Arthritis And The LungAnd The Lung

Broad differential for pleuropulmonary Broad differential for pleuropulmonary disease in those with rheumatologic disease in those with rheumatologic disorders:disorders: Secondary to, or associated with the underlying Secondary to, or associated with the underlying

rheumatic diseaserheumatic disease Secondary to immunosuppression (infection)Secondary to immunosuppression (infection) Secondary to drug therapySecondary to drug therapy Coexistant medical problemsCoexistant medical problems Overlap syndromesOverlap syndromes

Pleural EffusionPleural Effusion Most common pulmonary manifestation of RAMost common pulmonary manifestation of RA Often incidental finding on CXROften incidental finding on CXR Patients often asymptomatic Patients often asymptomatic

?Reduced physical activity prevents symptoms?Reduced physical activity prevents symptoms Most common symptoms: pleuritic pain, dyspnea, coughMost common symptoms: pleuritic pain, dyspnea, cough Pleural effusions can precede or occur simultaneously with Pleural effusions can precede or occur simultaneously with

joint symptoms in 25%joint symptoms in 25% More common in men with high RF titer and active arthritisMore common in men with high RF titer and active arthritis Can be uni- or bilateral, resolve, recur or persist for monthsCan be uni- or bilateral, resolve, recur or persist for months Post-mortem studies Post-mortem studies almost 50% of patients with RA almost 50% of patients with RA

have pleural effusionshave pleural effusions

Pleural EffusionPleural Effusion

Treatment:Treatment: None needed if asymptomaticNone needed if asymptomatic Repeated thoracentesis or pleurodesisRepeated thoracentesis or pleurodesis NSAIDs, steroidsNSAIDs, steroids Intrapleural steroidsIntrapleural steroids

Probably best to control the Probably best to control the underlying RAunderlying RA

Nodular Lung DiseaseNodular Lung Disease Pulmonary nodules in RA first described by Pulmonary nodules in RA first described by

Caplan in 1953Caplan in 1953 Discovered multiple bilateral nodules on CXR of

coal miners with RA Caplan’s syndrome: Pneumoconiosis in RA

patient leading to multiple basilar pulmonary nodules and mild airflow obstruction

Only pulmonary manifestation specific for Only pulmonary manifestation specific for RARA

Can occur before, with or after the joint Can occur before, with or after the joint manifestations of RAmanifestations of RA

Usually asymptomatic, but can cause Usually asymptomatic, but can cause coughing and rarely hemoptysiscoughing and rarely hemoptysis

Nodular Lung DiseaseNodular Lung Disease Usually multiple, bilateral nodulesUsually multiple, bilateral nodules Range from few millimeters to several Range from few millimeters to several

centimeters in sizecentimeters in size Typically occur just below the pleura or Typically occur just below the pleura or

associated with interlobular septaassociated with interlobular septa Can lead to bronchopleural fistula, Can lead to bronchopleural fistula,

pneumothorax, abcess or cavitionpneumothorax, abcess or cavition Can remain static, resolve, increase in size Can remain static, resolve, increase in size

or undergo malignant transformationor undergo malignant transformation More common in men, ?association with More common in men, ?association with

smokingsmoking

Nodular Lung DiseaseNodular Lung Disease

Management Management usually observation usually observation sufficessuffices

Transbronchial biopsy or Transbronchial biopsy or transthoracic needle aspiration to rule transthoracic needle aspiration to rule out malignancy or other pathologic out malignancy or other pathologic processprocess

Diffuse Interstitial Diffuse Interstitial FibrosisFibrosis

More common in those with severe RAMore common in those with severe RA Most modifiable risk factor: smokingMost modifiable risk factor: smoking

>25 pack-year smoking history significantly more likely to >25 pack-year smoking history significantly more likely to have radiographic evidence of ILD have radiographic evidence of ILD

Usually occurs about five years after joint symptoms present, Usually occurs about five years after joint symptoms present, but can predate thembut can predate them

Occurs mostly in those with subcutaneous nodules and high Occurs mostly in those with subcutaneous nodules and high RFRF

Symptoms: progressive SOBOE and productive cough most Symptoms: progressive SOBOE and productive cough most commoncommon Also: increased RR, clubbing, crepitations at lung bases, Also: increased RR, clubbing, crepitations at lung bases,

pulmonary hypertensionpulmonary hypertension CXR CXR Reticulated pattern with progression to fine Reticulated pattern with progression to fine

nodularity and honeycombingnodularity and honeycombing

Diffuse Interstitial Diffuse Interstitial FibrosisFibrosis

Prognosis is poorPrognosis is poor Treatment usually includes Treatment usually includes

corticosteroids, azathioprine or other corticosteroids, azathioprine or other immunomodulating medications (e.g., immunomodulating medications (e.g., cyclophosphamide)cyclophosphamide)

?Single lung tranplantation?Single lung tranplantation Usually better results in those with RA Usually better results in those with RA

associated interstitial fibrosisassociated interstitial fibrosis Usually too many comorbidities for Usually too many comorbidities for

transplantation surgery (e.g., osteoporosis, transplantation surgery (e.g., osteoporosis, decreased mobility)decreased mobility)

Newer therapies (e.g., TNF blockers)Newer therapies (e.g., TNF blockers)

InfectionsInfections

Persistent problem in those with RAPersistent problem in those with RA Many confounding factors, especially Many confounding factors, especially

corticosteroid or immunosuppressive corticosteroid or immunosuppressive medicationmedication May mask the signs of infectionMay mask the signs of infection

Lymphocyte abnormalities in RA?Lymphocyte abnormalities in RA? Patients with RA have greater occurrence Patients with RA have greater occurrence

of bronchitis, bronchiectasis and of bronchitis, bronchiectasis and pneumonia than controls with degenerative pneumonia than controls with degenerative joint diseasejoint disease

Drug Related Pulmonary Drug Related Pulmonary DiseaseDisease

Methotrexate:Methotrexate: Presents with dyspnea, cough and feverPresents with dyspnea, cough and fever Usually subacuteUsually subacute 50% of cases diagnosed within 32 50% of cases diagnosed within 32

weeks of initiating MTXweeks of initiating MTX Re-challenge with MTX causes high rate Re-challenge with MTX causes high rate

of recurrence of lung injuryof recurrence of lung injury 17% of patients who develop lung 17% of patients who develop lung

disease due to MTX will die of this disease due to MTX will die of this complicationcomplication