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Pulmonary subspeciality
rounds
Dr.Krock [pulmonology]
Dr.Poddutoori [PGY3, I.M]
Case presentation
Chief complaint:
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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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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.
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.
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.
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.
Case presentation
Allergies: penicillin.
Social history:
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Ex-smoker, no alcohol or illicit drug use.
Divorced, was sexually active, and worked as
a machinist.
Family history:
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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.
Case presentation
Vitals:B.P- 116/71, HR 74, RR 18, temp 36.3°C,
Spo2 99% on RA.
On exam:
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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.
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, antiRNP, 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, complementsWNL
Blood and urine cultures were negative.
Investigations
EKG: PR interval-256 msec and 1st degree AV
block
Echo:
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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.
Transthoracic and Transesophageal Echocardiograms
Merkel P et al. N Engl J Med 2008;359:1603-1614
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.
Radiologic Images
Merkel P et al. N Engl J Med 2008;359:1603-1614
Differential diagnosis
Mediastinal mass:
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Tumors: lung, esophageal, vascular, thymic, or
thyroid cancers
Infections: TB, non-TB mycobacteria,
actinimycosis and nocardiosis, histoplasmosis
Systemic inflammatory diseases: Sarcoidosis,
vasculitis
Arthritis:
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Rheumatoid arthritis, SLE, Sarcoidosis, vasculitis,
solid organ cancers like leukemia and lymphoma
associated paraneoplastic arthritis, infections.
Optic neuritis:
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TB, other infections[syphilis], sarcoidosis,
vasculitis, lymphoma, SLE varaint lupus sclerosis
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.
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.
Biopsy Specimens of the Mediastinal Mass (Hematoxylin and Eosin)
Merkel P et al. N Engl J Med 2008;359:1603-1614
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
Final Diagnosis
Wegener's granulomatosis involving the
mediastinum, heart, and pulmonary
artery.
Features of Wegener's Granulomatosis
Merkel P et al. N Engl J Med 2008;359:1603-1614
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.
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
Treatment
Cyclophosphamide induction for severe
disease:
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2mg/kg/day po with glucocorticoids [prednisone1mg/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:
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Etanercept-no sustained remission.
Anti CD20[Rituximab]
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%
C-ANCA
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