26 Jan 2022

5/6/21: Tracheobronchial Amyloidosis

PGY2 Cho Kim presented a case of a 63 y/o female with history of hypertension, chronic serous otitis and COVID-19 pneumonia presents with voice changes over the past 2 years with new audible wheezing x 2 days and shortness of breath at rest x 2weeks.  Patient states that her symptoms started 1.5 years ago with bilateral hearing loss and tinnitus followed by voice change and increased hoarseness.  Over the past 2 weeks, patient has had increased dyspnea with exertion, with limited exercise capability.  She presented to an outside clinic where she was given an โ€œinhalerโ€ that improved symptoms, but has since run out.  In addition to dyspnea, patient has developed loud wheezing at rest over the past 2 days.  Denies any lower extremity swelling, orthopnea, night time cough, runny nose, sore throat or muscle pain.

Meds: lisinopril 10mg qday, vit D supplements

FH: Sister with breast cancer

SH: Lives with daughter in Los Angeles.  Worked in a kitchen and as a seamstress.  Denies any tobacco/drug/alcohol use.

Allergies: ASA s/p sensitization protocol

SH: Lives with family.  Denies tobacco/drug/alcohol use.

ROS: +25lb unintentional weight loss over 1 year, rhinorrhea, sore throat 1 year prior that self resolved

Home Meds: Metoprolol 25mg qday

FH: Sister with anemia that required transfusion

SH: Lives at home with husband and children.  Denies tobacco, ETOH or drug use

Vitals: T 36.5, HR 87, RR 24, BP 167/97, O2 sat 100% on RA

Physical Exam: +hoarse voice with limited phonation, no saddle nose, b/l external ear, canal and TM without deformity/effusion/erythema, cardiac exam normal, pulm exam with audible wheezing throughout, inspiratory crackles, decreased lung sounds in R posterior region, MSK with PIP nodularity but no synovial effusion/tenderness.

CBC: WBC 7.7>Hb 15.3/Hct 45.7 <Plt 226 (MCV 92.6, RDW 14.2%)

CMP:

Na 141/K 3.9/Cl 103/HCO3 35/BUN 11/Cr 0.7<Glucose 99

ALP 93>T.protein 7.3/Albumin 4.8/AST 30/ALT 22/Tbili 0.4/Dbili <0.2

Ca 8.8

Infectious workup initiated with negative findings of the following: BCx, UA, HIV, COVID-19, and hepatitis panel.  Of note, UA did show large blood but only 3 RBCs per HPF.

ABG: 7.44/39/149/27, FiO2 28%

CXR: mild R basilar opacity, otherwise clear lungs.

CT Sinus: no evidence of acute sinusitis.

CT Thorax: Irregular nodular wall thickening involving the distal trachea with sparing of the posterior membranous wall and mild luminal narrowing.  Irregular wall thickening extends to the carina, R and L mainstem bronchus and proximal lobar bronchi. 

SPEP: No definitive abnormal bands visible.

UPEP: An abnormal band is visible in the gamma region (30% of the total urinary protein).

K/L: 6.32

Rheum workup negative for ANA, ANCA, dsDNA, SSA/SSB, Scl Ab, SM Ab and ACE.

Bronch was done, found exophytic lesion in the LUL with 50% luminal narrowing.

Transbronchial Biopsy: negative for carcinoma.  Positive for congo red, consistent for amyloid.

Treatment: Patient treated with duonebs and standing fluticasone.  Given amyloidosis diagnosis, she was transferred to Keck Hospital and underwent debulking in the OR with cryoprobe, balloon dilation of b/l mainstem bronchi and Kenalog injections in subglottic region.  Patient was discharged with outpatient followup.

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