Chest at a Glance (1/2569): A man with lung cysts

A 68-year-old man, underlying disease: Atrial fibrillation

CC: Worsened dyspnea for 1 month

Key clinical findings

• Progressive dyspnea with abdominal fullness for 1 year
• Worsening dyspnea with bilateral pedal edema and jaundice for 1 month
  
• Desaturation (SpO2 92% on room air) with mild respiratory distress
• CVS: total irregular pulse, PSM grade 2 at LLPSA, presence of Carvallo’s sign

Key investigations

• Hb 11.3 g/dL, Hct 35.5%, WBC 3730/uL (PMN 60%, Lym 30%), plt 197,000 /uL
• BUN 34.7 mg/dL, Cr 1.64 mg/dL, NT-proBNP 4335 pg/mL
• AST 45 U/L, ALT 40 U/L, ALP 352 U/L, TB 3.2 mg/dL, DB 2.6 mg/dL
• TTE: RV dilate, severe TR, TRVmax 4.06, EF 55%

Provisional diagnosis

Pulmonary hypertension with right-sided heart failure

คำถามสำคัญต่อมา คือ “What is (are) the cause (s) of PH?“

Further investigation

Aims: To confirm PH and determine its causes

…………..

• RHC: PAP 72/18 mmHg, mPAP 40 mmHg, PCWP 23 mmHg, PVR 5.4 WU, CO 3.15 L/min, CI 1.96 L/min/m2
• PA angiography: no filling defect
• PFT: FEV1/FVC 0.59, FEV1 1.47 L (66%), FVC 2.51 L (77%), TLC 4.50 L (74%), RV/TLC 43%, DLCO 64%

ลองแปลผลและให้การวินิจฉัยแยกโรคกันครับ แล้วคลิ๊กไปดูเฉลยกันที่ Tab ด้านบนต่อไปได้เลย

(ต่อ)

Key clinical clues

• PH with right-sided heart failure
• Combined pre- and post-capillary PH (mPAP > 20 mmHg, PCWP > 15 mmHg, PVR > 2 WU)
• Diffuse cystic lung disease*
• CTEPH is excluded
• Anemia and leukopenia
• Azotemia

Diffuse cystic lung disease

• Diffuse thin-walled cyst
• Ground-glass opacities and fine reticulation in lower lung zone
• No nodule or calcified nodule

จาก chest CT จะคิดถึงโรคต่อไปนี้

• Lymphoid interstitial pneumonia (LIP)
• Amyloidosis
• Light chain deposition disease (LCDD)

จึงส่งตรวจ bone marrow study ต่อ เนื่องจากมี anemia และ leukopenia ร่วมด้วย

Plasma cell neoplasm with lambda light chain restriction, negative Congo red stain

Definite diagnosis

Pulmonary light chain deposition disease (LCDD) with pulmonary hypertension

Clinical pearls