Sarcoidosis beyond pulmonary involvement: A case series of unusual presentations

Basma M Medhat; Mervat E Behiry; Mohamed Fateen; Nehal El-Ghobashy; Raghda Fouda; Aya Embaby; Esraa M Seif; Marwa Magdy Taha; Mohammed Kamal Hasswa; Dina Sobhy; Christina Samir Ragheb; Mohamed Abdelkader Morad

doi:10.1016/j.rmcr.2021.101495

Sarcoidosis beyond pulmonary involvement: A case series of unusual presentations

Respir Med Case Rep. 2021 Aug 21:34:101495. doi: 10.1016/j.rmcr.2021.101495. eCollection 2021.

Authors

Affiliations

¹ Rheumatology and Rehabilitation Department, Kasr Alainy Faculty of Medicine, Cairo University, Cairo, Egypt.
² Rheumatology Unit. Internal Medicine Department, Kasr Alainy Faculty of Medicine, Cairo University, Cairo, Egypt.
³ Armed Forces College of Medicine (AFCM), Cairo, Egypt.
⁴ Clinical and Chemical Pathology Department, Kasr Alainy Faculty of Medicine, Cairo University, Cairo, Egypt.
⁵ Chest Department, Kasr Alainy Faculty of Medicine, Cairo University, Cairo, Egypt.
⁶ Resident of Internal Medicine. Master's Degree of Internal Medicine, Egypt.
⁷ Clinical Haematology Unit. Internal Medicine Department Kasr Alainy, Faculty of Medicine, Cairo University, Cairo, Egypt.

Abstract

Unusual presentations of sarcoidosis pose a diagnostic challenge and warrant attention. Hematologic associations: Case 1 (37-years-old male): Pancytopenia: myelofibrosis (leading to sepsis and mortality) following a two-year quiescent course of biopsy-proven-sarcoidosis. Case 2 : (38-years-old male): Presentation with thrombocytopenia (5 × 10³/cmm): immune thrombocytopenic purpura (histologically associated with megakaryocytic emperipolesis). Biopsied enlarged lymph nodes demonstrated sarcoidosis. Hematologic sarcoid involvement is usually due to granulomatous bone marrow (3.9%) or splenic infiltration (6-30%); however, the presented manifestations are scarcely reported with a potential significance that is yet to be elucidated. Case 3: Neurologic presentation: 48-years-old female: presentation with bilateral sensorineural hearing loss and facial palsy. Brain magnetic resonance imaging showed leptomeningeal thickening. Biopsied enlarged lymph nodes showed sarcoidosis. Case 4: Neurologic and renal manifestations: 13-years-old male (family history of sarcoidosis): Presenting with acute headache, investigations showed elevated serum creatinine (2.1 mg/dL) and angiotensin converting enzyme, and computed tomography chest and abdominal findings characteristic of sarcoidosis. Associated benign increased intracranial and acute tubulointerstitial nephritis (with eosinophils) were diagnosed upon concordant workup. Of sarcoidosis neurologic affection (5-10%), cranial nerve(s) involvement is among the most common (25-50% of neurosarcoid affection), particularly that of the facial nerve (Case 3). Leptomeningeal enhancement is among the most common neurosarcoid radiologic findings (30-40%). Whereas benign increased intracranial tension (Case 4) is much less reported. Among sarcoidosis renal involvement (35-50%), interstitial nephritis usually presents with granulomatous renal lesions, yet its sole association with sarcoidosis is unusual (Case 4). The portrayed atypical hematologic, neurologic, and renal manifestations further emphasize the masquerading nature of sarcoidosis.

Keywords: Emperipolesis; Juvenile; Myelofibrosis; Neurosarcoidosis; Renal involvement; Sarcoidosis.

Publication types

Case Reports