2 CASE PRESENTATION 1Mrs R, 44 years old lady with no previous medical or surgical historyMain complaint was painful red eye for one year associated with discomfort, photophobia and blurred vision.Seen by ophthalmologist and diagnosed with bilateral chronic uveitisShe was then investigated to determine aetiologyShe has a history of Bells Palsy which resolved spontaneously after one week and a history of hyper-pigmented scaly lesion on forehead.There is no history of cough or dyspnoea.No history suggestive of TBNo history of joint pain.
3 ExaminationOn examination she was a well looking young, not dyspnoeic,BP 129/88PR 93/minuteNo significant lymphadenopathyHyper pigmented lesions on the foreheadChest clearCVS :NADAbdomen no organomegalyMusculoskeletal system: no arthritis and no muscle weaknessCNS: NADNo cranial nerve palsy, no peripheral neuropathy
4 Investigation FBC: Normal U&E: Normal LFT: Normal CALCIUM LEVEL: Slightly raisedESR: 40mm per hourANF: NegativeSACE LEVEL: 111 (Normal less than 52)LUNG FUNCTION TEST: NormalCHEST X RAY (next slide)
6 SARCOIDOSISMultisystem inflammatory disease of unknown etiology that predominantly affects the lungs.Manifested by the presence of non-caseating granulomas (NCGs) that may affect any organ systemThe many forms and presentation of this disease and the lack of a single diagnostic test can make the diagnosis challenging
7 The lungs are involved in more than 90 percent of patients, with sarcoid usually presenting as interstitial disease.Symptoms are dry cough, dyspnea, and chest discomfort.Pulmonary sarcoidosis has an unpredictable course that may result in spontaneous remission or lead to progressive loss of lung function with fibrosis.Airway involvement can occur and may result in airflow limitation, persistent cough and, in severe cases, bronchiectasis.
8 TreatmentThe majority of patients will have spontaneous remission and a generally benign clinical course.Treatment is reserved forpatients with worsening pulmonary function testspatients with worsening pulmonary symptoms (cough, shortness of breath, chest pain or hemoptysis) andpatients with extra pulmonary sarcoidosis including arthritis, neuropathy, cardiac and renal sarcoid, also in,patient with intractable fatigue, weakness or fever.Corticosteroids are the mainstay of therapy.Generally, prednisone given daily and then tapered over a 6-month course is adequate for pulmonary disease.
9 EPIDEMIOLOGY Sarcoidosis affects men and women of all races and ages Usually presents in adult younger than 40 years more frequent between years, and slightly more predominant in women than in men.Course of sarcoidosis is variable, ranging from self limited acute disease to a chronic debilatating disease.Spontaneous remissions occur in nearly two thirds of patients.
11 Case Presentation 263 years old female presented with gradual onset SOB over the last 6 months.She is known to suffer from Rheumatoid arthritis for the last 3 years and has been on treatment with methotrexate for the same.She has previously had an episode of pleural effusion which was drained and investigated but was found to be an exudate with high protein and low glucose.On examination she had dull percussion note and absent air entry in her left lower zone of the chest.Patient’s bloods were within normal limits.Chest x ray confirmed a pleural effusion.
12 Overview Major catagories of pulmonary disease associated with RA: Pleural effusionNodular lung diseaseDiffuse interstitial fibrosisBOOP (bronchiolitis obliterans organizing pneumonia)Pulmonary vasculitisAlveolar hemmorhageObstructive diseaseInfections
13 Rheumatoid Arthritis And The Lung Broad differential for pleuropulmonary disease in those with rheumatologic disorders:Secondary to, or associated with the underlying rheumatic diseaseSecondary to immunosuppression (infection)Secondary to drug therapyCoexistant medical problemsOverlap syndromes
14 Pleural Effusion Most common pulmonary manifestation of RA Often incidental finding on CXRPatients often asymptomatic?Reduced physical activity prevents symptomsMost common symptoms: pleuritic pain, dyspnea, coughPleural effusions can precede or occur simultaneously with joint symptoms in 25%More common in men with high RF titer and active arthritisCan be uni- or bilateral, resolve, recur or persist for monthsPost-mortem studies almost 50% of patients with RA have pleural effusions
15 Pleural Effusion Treatment: Probably best to control the underlying RA None needed if asymptomaticRepeated thoracentesis or pleurodesisNSAIDs, steroidsIntrapleural steroidsProbably best to control the underlying RA
16 Nodular Lung DiseasePulmonary nodules in RA first described by Caplan in 1953Discovered multiple bilateral nodules on CXR of coal miners with RACaplan’s syndrome: Pneumoconiosis in RA patient leading to multiple basilar pulmonary nodules and mild airflow obstructionOnly pulmonary manifestation specific for RACan occur before, with or after the joint manifestations of RAUsually asymptomatic, but can cause coughing and rarely hemoptysis
17 Nodular Lung Disease Usually multiple, bilateral nodules Range from few millimeters to several centimeters in sizeTypically occur just below the pleura or associated with interlobular septaCan lead to bronchopleural fistula, pneumothorax, abcess or cavitionCan remain static, resolve, increase in size or undergo malignant transformationMore common in men, ?association with smoking
18 Nodular Lung Disease Management usually observation suffices Transbronchial biopsy or transthoracic needle aspiration to rule out malignancy or other pathologic process
19 Diffuse Interstitial Fibrosis More common in those with severe RAMost modifiable risk factor: smoking>25 pack-year smoking history significantly more likely to have radiographic evidence of ILDUsually occurs about five years after joint symptoms present, but can predate themOccurs mostly in those with subcutaneous nodules and high RFSymptoms: progressive SOBOE and productive cough most commonAlso: increased RR, clubbing, crepitations at lung bases, pulmonary hypertensionCXR Reticulated pattern with progression to fine nodularity and honeycombing
20 Diffuse Interstitial Fibrosis Prognosis is poorTreatment usually includes corticosteroids, azathioprine or other immunomodulating medications (e.g., cyclophosphamide)?Single lung tranplantationUsually better results in those with RA associated interstitial fibrosisUsually too many comorbidities for transplantation surgery (e.g., osteoporosis, decreased mobility)Newer therapies (e.g., TNF blockers)
21 Infections Persistent problem in those with RA Many confounding factors, especially corticosteroid or immunosuppressive medicationMay mask the signs of infectionLymphocyte abnormalities in RA?Patients with RA have greater occurrence of bronchitis, bronchiectasis and pneumonia than controls with degenerative joint disease
22 Drug Related Pulmonary Disease Methotrexate:Presents with dyspnea, cough and feverUsually subacute50% of cases diagnosed within 32 weeks of initiating MTXRe-challenge with MTX causes high rate of recurrence of lung injury17% of patients who develop lung disease due to MTX will die of this complication