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