Pulmonary subspeciality rounds

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Pulmonary subspeciality rounds

• Dr.Krock pulmonology
• Dr.Poddutoori PGY3, I.M

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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
• Chest pain Middle right chest pain for the
  past 2 months, radiating intermittently to right
  arm, increasing in 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.

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PMH

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

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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.

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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.

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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 oxycodoneacetaminophen for pain.

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Case presentation

• VitalsB.P- 116/71, HR 74, RR 18, temp 36.3C,
  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.

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Labs

• CBCWBC-15.4, Hb-12.5, Pl-600, N-71, MCV-79
• BMPWNL, 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.

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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.

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Transthoracic and Transesophageal Echocardiograms
Merkel P et al. N Engl J Med 20083591603-1614
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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.

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Radiologic Images
Merkel P et al. N Engl J Med 20083591603-1614
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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 infectionssyphilis, sarcoidosis,
  vasculitis, lymphoma, SLE varaint lupus sclerosis

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Hospital course

• During 1st 3 hospital days, night sweats occurred
  and Tmax was 38.1C. 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.

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Diagnostic procedure

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

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Biopsy Specimens of the Mediastinal Mass
(Hematoxylin and Eosin)
Merkel P et al. N Engl J Med 20083591603-1614
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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

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Final Diagnosis

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

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Features of Wegener's Granulomatosis
Merkel P et al. N Engl J Med 20083591603-1614
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Wegeners granulomatosis

• Definition Clinicopathologic entity ch by
  granulomatous vasculitis of upper95 and lower
  respiratory tracts85-90 with GN.
• Variable degrees of disseminated vasculitis of
  small arteries and veins may occur.
• Prevalence 3/100,000, rare in blacks
• MF 11, any age, 15lt19, mean age-40
• Pathology hallmarks-necrotizing vasculitis of
  small arteries and veins with granuloma
  formation-intravascular or extravascular.
• Increased incidence of venous thrombotic events.

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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-vasculiti
  s or granuloma.
• Cardiac 8 pericarditis, coronary vasculitis,
  cardiomyopathy
• Nervous system 23- cranial neuritis,
  mononeuritis multiplex, cerebral vasculitis or
  granuloma
• Renal disease77- most of the mortality
• Non-specific symptoms- malaise, weakness,
  arthralgias, anorexia, weight loss

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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 CD20Rituximab

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Diagnosis

• Elevated ESR, mild anemia and leukocytosis, mild
  hypergammaglobulinemiaIgA, mildly elevated RA
  factor.
• 90 pts with active disease have a
  antiproteinase-3 ANCA, inactive disease-60-70

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C-ANCA

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