18 Jun 2021

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 “inhaler” 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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