27 Nov 2020

10/29/20: Sarcoidosis

PGY3 Argin Baghramian presented a case of a 42 year old male with no past medical history who presents with one week of chest pain.  He characterized the chest pain as deep, sharp, worse on the R side, radiating to the back, exacerbated by exertion and with respirations, improved by rest.  He has had no prior history of similar pain.  Denies recent fever, cough, hemoptysis, PND, or edema.  Denies recent travel history, surgery or sick contacts.  ROS further elucidated a 15 pound weight loss and testicular swelling in the last 2 months along with a nonpruritic rash in the back and abdomen for the last week.  He does not take any home medications.  Social history revealed that the patient was born in Mexico and immigrated oto the US in 1992.  He works as a custodial service in the hospital.  Has a small dog at home.  Denies any tobacco or illicit drug use.  Rare alcohol use.

Vitals: T 37.1, HR 72, RR 18, BP 154/94, O2 sat 97% on RA

Physical Exam significant for normal cardiac and respiratory exam, +splenomegaly, multiple hard nontender nodules on each testicle R>L and multiple violaceous, erythematous and skin colored indurated 0.5-1cm papules and nodules scattered on back, upper arms and chest.  Non-scaly, non-tender and non-pruritic.

CBC: WBC 6.4> Hb 12.3/Hct 36.4 <Plt 227(MCV 80.5, RDW 15.3%)

CMP:

Na 135/K 4.1/Cl 100/HCO3 26/BUN 19/Cr 1.92 <Glucose 92 (baseline Cr 1.09)

ALP 96>T.protein 6.8/Albumin 3.6/AST 20/ALT 14/Tbili 0.5/Dbili 0.2

Ca 13.1, Mg 2.2, Phos 3.2

Coags: PT 14.3, INR 1.3, PTT 31

CXR showed bilateral hilar prominence.

Scrotal US showed multiple hypoechoic masses are seen in the testicles bilaterally, R>L with no evidence of enhancement or internal vascularity.

Because of the scrotal US findings, cancer markers sent including AFP, HCG, LDH which all resulted negative.

Infectious workup also initiated with negative BCx, UCx, HIV, RPR, TB and fungal serologies including histoplasmosis.

Hypercalcemia workup obtained with the following results:

  • PTH: 5 pg/ml (normal 15-65)
  • Vit D 25-OH: 11 ng/mL (normal 30-100)
  • Vit D 1,25 OH: 104 pg/mL (normal 18-72)

CT Chest was obtained which showed bilateral diffuse upper lobe predominant reticulonodular opacities with mediastinal and bilateral hilar lymphadenopathy.

Skin biopsy and EBUS biopsy both resulted with noncaseating granulomatous inflammation, consistent with sarcoidosis.

Treatment: Patient was started on NS 125cc/hr for hypercalcemia pending further workup.  Once the hypercalcemia workup was indicative of possible granulomatous disease such as sarcoid, pulmonology was consulted.  The patient was started on prednisone for management of sarcoidosis.

Teaching points:

  1. Differential diagnosis for splenomegaly: After portal hypertension is ruled out, your three top causes: Lymphoma, Infection and inflammatory process.
  2. Sarcoidosis is a multisystem granulomatous disorder characterized pathologically by noncaseating granulomas in involved organs including lungs, eyes (25%), skin (25%) and heart (25-70% with heart block, arrhythmias, HF, valvular dysfunction).
    1. Lofgren syndrome: constellation of skin findings (erythema nodosum) + pulm (bilateral hilar lymphadenopathy) + fever + polyarthritis
  3. 3 major criteria for diagnosis: compatible clinical presentation, non-necrotizing granulomas and the exclusion of alternative causes of granulomatous disease.
  4. Will need the following: baseline eye exam, baseline ECG, baseline labs, TB testing, CXR, HRCT and ACE.
  5. Treatment is prednisone with taper over several weeks.  Treat for at least a year.  Relapses are frequent with some people requiring lifelong maintenance therapy.

Relevant articles:

Also, a USC fun fact! Dr. John S. Adams from Division of Endocrinology dedicated his work to understanding the connection between hypercalcemia and sarcoidosis here at USC!

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