CME INDIA Case Presentation by Dr. V. Nagarjuna Maturu, Head, Dept of Pulmonology, Yashoda hospitals Hitec city, Hyderabad.

CME INDIA Case Study

How Presented?

• A 35-year-old lady, nurse by occupation who recently completed treatment for MDR cervical node tuberculosis using an all-oral bedaquiline based regimen, presented with 6 months history of streaky hemoptysis.
• There were no other constitutional symptoms.

Relevant Investigations

• A CT thoracic angiography did not reveal any abnormal vessels, and the lung parenchyma was normal. There were a few mediastinal nodes.

• Bronchoscopy done before referral to our centre was reported to be normal.
• At presentation to our centre, she was comfortable but with ongoing cough and streaks of hemoptysis.
• A repeat bronchoscopy was performed.
• It showed red flecks all through the bronchial tree.

• A BAL was performed, which gave a reddish-purple return. On centrifugation, there was a deposit of powdery purple substance. Microbiological workup showed only xpert MTB to be positive(consistent with healed tuberculosis).

Provisional Diagnosis at this Point

• After a deep discussion, a possibility of clofazimine deposition was considered and was evaluated further.

What further evaluation showed?

Index Case

Take Home Messages

• When routine causes are ruled out, it sometimes helps to think out of the box.
• All hemoptysis is not true hemoptysis – consider spurius hemoptysis, pseudo hemoptysis or factitious hemoptysis when the cause is not obvious.
• Clofazimine deposition in alveolar macrophage is extremely uncommon with only a few reported cases earlier Serum and BAL clofazimine levels were performed using HPLC and it was seen that BAL levels were markedly elevated.
• Microscopic examination of BAL fluid showed alveolar macrophage to be filed with reddish crystals of clofazimine.
• She also had diffuse hyperpigmentation of skin.
• A diagnosis of Sysyemic Clofazimine Crystal Deposition Disorder was thus confirmed.

Final Diagnosis

• Clofazimine Crystal Deposition Syndrome leading to Pseudo Hemoptysis.

CME INDIA Learning Points

• Diverse drugs and substances carry the potential to harm the respiratory system through various mechanisms. Clofazimine, an anti-leprosy medication typically prescribed for a limited duration, possesses an extended half-life. Documentation indicates the crystalline deposition of the drug in various tissues, emphasizing the need for cautious use due to its prolonged presence in the body.
• The infrequent occurrence of drug crystal deposition in pulmonary parenchyma represents a rare mechanism leading to drug-induced pulmonary diseases. Prolonged, non-approved administration of clofazimine poses a significant risk, potentially resulting in severe pulmonary damage and fatal outcomes.
• Crystal-storing histiocytosis induced by clofazimine is a seldom encountered yet well-documented condition in the literature. Alongside the typical reddish discoloration of the skin, clofazimine triggers gastrointestinal disturbances, occasionally leading to severe abdominal pain.

References:

1. Wu Q, Ganzert C, Lücke E, Bittmann I, Schreiber J. Fatal course of clofazimine-induced pulmonary crystal deposition in a patient with Melkersson-Rosenthal syndrome. Int J Clin Pharmacol Ther. 2020 Sep;58(9):518-522. doi: 10.5414/CP203784. PMID: 32589131.
2. Singh H, Azad K, Kaur K. Clofazimine-induced enteropathy in a patient of leprosy. Indian J Pharmacol. 2013 Mar-Apr;45(2):197-8. doi: 10.4103/0253-7613.108323. PMID: 23716903; PMCID: PMC3660939.
3. Tomsula, Jessica & Meis, Jeanne & Koy, Robert & Monheit, Jacqueline & Zieske, Arthur & Ro, Jae & Ayala, Alberto. (2019). Crystal storing histiocytosis: Unusual clinical presentations in two patients. Annals of Diagnostic Pathology. 40. 10.1016/j.anndiagpath.2019.01.003.

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