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Pulmonary subspeciality rounds

Pulmonary subspeciality rounds. Dr.Krock [pulmonology] Dr.Poddutoori [PGY3, I.M]. Case presentation. Chief complaint: A 39-year-old man presented with chest pain arthralgias for 2 months and drenching night sweats for 1 wk. HOPI:

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Pulmonary subspeciality rounds

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  1. Pulmonary subspeciality rounds Dr.Krock [pulmonology] Dr.Poddutoori [PGY3, I.M]

  2. Case presentation • Chief complaint: • A 39-year-old man presented with chestpain arthralgias for 2 months and drenching night sweats for 1 wk. • HOPI: • Chest pain: Middle & right chest pain for the past 2 months, radiating intermittently to right arm, increasingin intensity with deep inspiration or postural changes. • Arthralgias: neck, shoulders, hips, knees, elbows, and wrists without swelling for the past several wks,. • Drenching night sweats: 1 wk. • Accompanied by fatigue & mild shortness of breath; no h/o associated wheezing, cough, or sputum.

  3. PMH • 1 yr ago: similar symptoms associated with diffuse arthralgias. • EKG showed diffuse ST-seg elevations. • CT chest: small pericardial effusion, thickened pericardium, severalpericardial LN up to 0.4 cm & small L pleural effusion. • PPD was negative • Diagnosed as pericarditis, ibuprofen prescribed.

  4. PMH • 2 months ago: • CT chest/abd/pelvis: pericardial thickening, which increased compared with the earlier study; the main pulmonary artery was distorted and narrowed to 2.6 cm(3.2 cm 1 yr earlier) at the level of the bifurcation; lymphadenopathy in the mediastinum, right hilar, and gastrohepatic regions was unchanged. There were no pleural or pericardial effusions, and there was no PE. • Seen by cardiologist, cardiac stress test was negative . • Arthralgias: seen by rheumatologist started on Prednisone (7.5 mg QD) for 2 wks.

  5. PMH • 4 yrs ago: H/o renal calculi • 5 yrs ago: h/o vision loss R eye and periorbital headaches • MRI: Enhancement of the frontal meninges and both optic nerves • CT chest: R hilar lymph node(1 cm in diameter) • Path of lung-biopsy showed no malignant tumor cells or granulomas. • Diagnosed with optic neuritis and pseudotumor cerebri • Prednisone [100 mg QD was continued for 3.5 yrs], acetazolamide, and oxcarbazepine were given.

  6. Case presentation • Allergies: penicillin. • Social history: • Ex-smoker, no alcohol or illicit drug use. • Divorced, was sexually active, and worked as a machinist. • Family history: • Father died in MVA. Maternal GF CHF, maternal GM had a brain tumor, mother had fibromyalgia. • Medications: prednisone(15 mg QD), ibuprofen [800 mg TID], and oxycodone–acetaminophen for pain.

  7. Case presentation • Vitals:B.P- 116/71, HR 74, RR 18, temp 36.3°C, Spo2 99% on RA. • On exam: • GEN: patient appeared tired. • HEENT: The conjunctivae and oropharynx were injected. Visual acuity was impaired in the R eye. • Neck: No JVD. No bruits. • Nodes: soft LN(1-2 cm) in the axillae B/L • S1 normal, S2 loud with a prominent split, and an ESM (grade 1-2/6) was loudest at the L upper sternal border. • Ext: trace edema. • MS: mild tenderness in the large muscle groups, with 4+/5 strength diffusely. There was full ROM in all joints and no swelling, redness, or effusions.

  8. Labs • CBC:WBC-15.4, Hb-12.5, Pl-600, N-71, MCV-79 • BMP:WNL, LFTs: WNL, Cardiac enzymes: negative • Serum PTH, 25-hydroxyvitamin D, and thyrotropin, were normal. • ESR-53, IgA - 648 • Tests for antibodies to ANA, ds DNA, anti-Smith, anti-RNP, anti-cyclic citrullinated peptide IgG, anti-La, and anti-Ro were negative. • Hepatitis C and HIV, monospot test, RPR, Lymes serology and HBsAg were negative. • PPD was negative • Ferritin- 313, iron-19, SPEP- WNL, complements- WNL • Blood and urine cultures were negative.

  9. Investigations • EKG: PR interval-256 msec and 1st degree AV block • Echo: • normal EF and MV, mild-to-moderate TR, mild RAE, mildly dilated RV. • Homogeneous echodensities within the main pulmonary artery narrowed lumen to 1 cm; lumen of the right pulmonary artery was 0.8 cm at its origin. Flow velocities in the main pulmonary artery were increased, Velocities at level of pulmonary valve were normal. • RVSP: 46 mm Hg. There was a small pl effusion and no evidence of pericardial constriction.

  10. Transthoracic and Transesophageal Echocardiograms Merkel P et al. N Engl J Med 2008;359:1603-1614

  11. Investigations • MRI chest: mediastinum contained extensive soft tissue, surrounding the ascending thoracic aorta and portions of the R pulmonary artery, with 80% reduction in lumen of the proximal portion of the R pulmonary artery and moderate compression of the L pulmonary artery. • Soft tissue enhanced after the administration of gadolinium.

  12. Radiologic Images Merkel P et al. N Engl J Med 2008;359:1603-1614

  13. Differential diagnosis • Mediastinal mass: • Tumors: lung, esophageal, vascular, thymic, or thyroid cancers • Infections: TB, non-TB mycobacteria, actinimycosis and nocardiosis, histoplasmosis • Systemic inflammatory diseases: Sarcoidosis, vasculitis • Arthritis: • Rheumatoid arthritis, SLE, Sarcoidosis, vasculitis, solid organ cancers like leukemia and lymphoma associated paraneoplastic arthritis, infections. • Optic neuritis: • TB, other infections[syphilis], sarcoidosis, vasculitis, lymphoma, SLE varaint lupus sclerosis

  14. Hospital course • During 1st 3 hospital days, night sweats occurred and Tmax was 38.1°C. Swelling of MCP & PIP joints of both hands developed, with tenderness on palpation and decreased ROM; swelling and limited ROM in both ankles and numbness over dorsum of the feet also developed. • Day 4: MRI of the cervical, thoracic, and lumbar spine disclosed no abnormalities.

  15. Diagnostic procedure • Open biopsy of mediastinal surface, R ventricular masses and core biopsy of the R pulmonary artery • Pathologic exam:granulomatous inflammation of surface and extensive necrosis in the deeper aspects of mass, small vessel neutrophilic vasculitis was noted.

  16. Biopsy Specimens of the Mediastinal Mass (Hematoxylin and Eosin) Merkel P et al. N Engl J Med 2008;359:1603-1614

  17. Hospital course • IV methylprednisone 500 mg daily • cyclophosphamide 300 mg daily • Fever, arthritis and night sweats resolved and was d/c ed on tapering dose of steroids and cyclophosphamide • Rpt echo 1 month later: resolution of abnormality of pulmonary artery. • ANCA titres disappeared 1 yr later • F-up imaging showed slight thickening of walls of aorta and main pulmonary artery, mediastinal mass is gone. • 18 m later cyclophosphamide d/c ed & azathioprine 150 mg + prednisone 10 started

  18. Final Diagnosis • Wegener's granulomatosis involving the mediastinum, heart, and pulmonary artery.

  19. Features of Wegener's Granulomatosis Merkel P et al. N Engl J Med 2008;359:1603-1614

  20. Wegeners granulomatosis • Definition: Clinicopathologic entity ch by granulomatous vasculitis of upper[95%] and lower respiratory tracts[85-90%] with GN. • Variable degrees of disseminated vasculitis of small arteries and veins may occur. • Prevalence: 3/100,000, rare in blacks • M:F – 1:1, any age, 15%<19, mean age-40 • Pathology: hallmarks-necrotizing vasculitis of small arteries and veins with granuloma formation-intravascular or extravascular. • Increased incidence of venous thrombotic events.

  21. Wegeners granulomatosis • Organ system involvement: • Lung involvement: typically multiple , b/l nodular cavitary infiltrates. • Renal involvement: FSGNRPGN • UR involvement: sinuses, nasopharynx-inflmn, necrosis, granuloma formation+/-vasculitis • Eye: 52%-mild conjunctivitis, dacrocystitis, episcleritis, scleritis, retroorbital mass, granulomatous sclerouveitis, ciliary vessel vasculitis • Skin lesions: 46% papules, vesicles, palpable purpura, ulcers, subcutaneous nodules-bx-vasculitis or granuloma. • Cardiac: 8% pericarditis, coronary vasculitis, cardiomyopathy • Nervous system: 23%- cranial neuritis, mononeuritis multiplex, cerebral vasculitis or granuloma • Renal disease:77%- most of the mortality • Non-specific symptoms- malaise, weakness, arthralgias, anorexia, weight loss

  22. Treatment • Cyclophosphamide induction for severe disease: • 2mg/kg/day po with glucocorticoids [prednisone-1mg/kg/dayalternate day and then taper in 6m], monitor leukocyte count. • Remission maintenance with azathioprine or methotrexate. • Methotrexate induction for non severe disease. • Biologic therapies: • Etanercept-no sustained remission. • Anti CD20[Rituximab]

  23. Diagnosis • Elevated ESR, mild anemia and leukocytosis, mild hypergammaglobulinemia[IgA], mildly elevated RA factor. • 90% pts with active disease have a + antiproteinase-3 ANCA, inactive disease-60-70%

  24. C-ANCA

  25. THANK YOU! THANK YOU

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