18 Sep 2020

7/23/20 Caplan Syndrome

Today in Goldstein Morning Report, PGY3 Ammar Dahodowala presented the case of a 39 yo M with controlled DM who presented with a 6 month history of dry cough and a 1 day history of hemoptysis. The cough was accompanied by unintentional 40 lb weight loss, intermittent night sweats and chest pain. Additionally, the patient has a 2 month history of joint pain in the shoulders, hips and knees that is worse in the morning.

Physical Exam:
Lung exam: unremarkable
Extremities: bogginess and TTP of bilateral MCP/PIP/shoulders/knees/ankles/feet but all without erythema. Right elbow – boggy and TTP with a nodule on the right lateral aspect of elbow

Labs:
CBC: WBC 8.5, Hgb 8.7 (MCV 77, RDW 18%), Platelets 1095K
CMP: Na 139, K 4.4, Cl 99, HCO3 24, BUN 20, Cr 1.08; Alk Phos 222, Pr 7.9, Alb 3.5, AST 24, ALT 24, Bili 0.3, Dbili <0.2
UA: negative, FeNA 3.6%

BCx negative

CXR:

CT Thorax:
ct1

Infectious Labs:
AFB x 3: negative
MTB PCR x 2: negative
HIV: negative
Fungal Blood culture: negative
Histo Ag: Negative
Histo Ab: Negative
Cocci Ab: Negative
Blasto Ab: Negative
Crypto Ag: Negative
Aspergillus: Negative
Beta-D-glucan: Negative
PCP DFA: Negative

Autoimmune Labs:
ANA: positive 1:160, homogenous
RF: 714 IU/L
CCP: 135 U/L
ANCA: negative
SSA/SSB: negative
C3/C4: WNL

Transbronchial Biopsy:
Final Pathologic Diagnosis:

  • Multifocal dust macules consisting of early cellular silicosis and with interalveolar aggregates of macrophages
  • Special stains are negative for fungus (GMS stain) and acid fast bacilli (Kinyoun AFB stain)

Biopsy x 3 confirmed same diagnosis.
biopsy1

Diagnosis:
Pulmonary Silicosis and Rheumatoid Arthritis

Take Home Messages:
-Pulmonary silicosis is caused by occupational exposure to silicon dioxide and is commonly found in hard rock miners (although incidence is now decreasing given workplace regulations)
-Caplan Syndrome is the manifestation of inflammatory pulmonary findings in patients with rheumatoid arthritis (RA) who work as coal miners after exposure to coal, asbestos, and silica
-Treatment entails addressing the extra-pulmonary manifestations of the RA

Sentinel Article:
Silica, Silicosis, and Autoimmunity

 

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