TRENTO MEETING ON LUNG PATHOLOGY THOMAS V. COLBY …
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Transcript TRENTO MEETING ON LUNG PATHOLOGY THOMAS V. COLBY …
CASE PRESENTATION
Andrea Tironi, Franco Franceschini
58 year-old woman, non smoker
persistent abdominal pain and fever
RELEVANT MEDICAL HISTORY
- post-autoimmune thyroiditis ipothyroidism
(EUTHYROX 100 mcg/d)
ESOPHAGO-GASTRIC-DUODENAL-COLONSCOPY
- chronic gastritis HP+
- colitis most consistent with infective etiology
CT THORAX AND ABDOMEN
- pulmonary alterations consistent with…
HRCT - lung
CT THORAX AND ABDOMEN
- pulmonary alterations consistent with
lymphangioleiomyomatosis (LAM)
- mild pericardial effusion
- hypertrophic thymic residue
- 12 mm calcified nodule in the right thyroid lobe
- hepatosplenomegaly
RELEVANT BLOOD-SERUM TESTS
- anti-ANA +
- anti-ENA (SSA/Ro) +
SURGICAL LUNG
BIOPSY
HMB45
HISTOLOGICAL DIAGNOSIS
LAM associated with lymphoid aggregates and granulomas
See comment
COMMENT
- probably unrelated diseases
- non-LAM features to be related to CVD, colitis, infection
RHEUMATOLOGIC
EVALUATION
Light Raynaud phenomenon for
some years
Light and transitory arthralgia of
small joints of hand and foot
Light xerostomia
Episodic asthenia
Nailfold capillaroscopy:
moderate non specific changes
LABORATORY TESTS
Consistent reduction of C4
ANA and SSA/Ro confirmed
positive
RHEUMATOLOGIST’S
DIAGNOSIS (12.2011)
Undifferentiated Connective
Tissue Disease (UCTD)
LABORATORY TESTS
Anti-phospholipids antibodies: anti-cardiolipin positive for IgM 47.5 MPL
(nv<10 MPL) and anti-Beta2 glycoprotein I (IgG and IgM at high title)
Modest reduction of C3 (78 mg/dl) (nv: 90-180 mg/dl) and consistent
reduction of C4 (4,3 mg/dl) (nv: 10-40 mg/dl)
Rheumatoid factor positivity (IgM 23,7 UI/ml) (nv: <14 UI/ml) with
negativity of anti-cyclic citrullinate peptide (anti-CCP) IgA and IgG
ANCA negative
Absence of cryoglobulines
Anti-thyroglobulin 374 UI/ml (nv: <4,1UI/ml) and anti-TPO 4 UI/ml (nv:
<5.6 UI/ml)
ANA and SSA/Ro confirmed positive
TREATMENT
UCTD:
- Hydroxichloroquin 5mg/kg/d
- Cardioaspirin 100mg/d
LAM:
- followed-up
Last follow-up: patient is well
Pulmonary lymphangioleiomyomatosis
associated with pulmonary parenchimal, hilar,
and mediastinal noncaseating granulomas
Huml J.P. et al. Chest 1991; 100:1726-28
37 year old woman, non-smoker, presented with chest disconfort and
severe dyspnea on exertion of two months duration
Surgical lung biopsy: LAM with non caseating granulomas
Death after acute tension pneumothorax
Pertinent autopsy findings: non caseating granulomas in lung and
hilar and mediastinal lymph nodes, lymphangioma of liver, uterine
leiomyoma
Infection, cancer, foreign bodies, autoimmune reactions were excluded
DISCUSSION POINTS
§
LAM as incidental CT finding
might be clinically irrelevant.
§
LAM associated with non-PEC
systemic disease involving lung
§
Straightforward HRCT pattern
might not explain sufficiently the
clinical scenario