Histology of Cardiac Sarcoidosis with Novel Considerations Arranged upon a Pathologic Basis

Sarcoidosis is a rare disease of isolated or diffuse granulomatous inflammation. Although any organs can be affected by sarcoidosis, cardiac sarcoidosis is a fatal disorder, and it is crucial to accurately diagnose it to prevent sudden death due to dysrhythmia. Although endomyocardial biopsy is invasive and has limited sensitivity for identifying granulomas, it is the only modality that yields a definitive diagnosis of cardiac sarcoidosis. It is imperative to develop novel pathological approaches for the precise diagnosis of cardiac sarcoidosis. Here, we aimed to discuss commonly used diagnostic criteria for cardiac sarcoidosis and to summarize useful and novel histopathologic criteria of cardiac sarcoidosis. While classical histologic observations including noncaseating granulomas and multinucleated giant cells (typically Langhans type) are the most important findings, others such as microgranulomas, CD68+ CD163− pro-inflammatory (M1) macrophage accumulation, CD4/CD8 T-cell ratio, Cutibacterium acnes components, lymphangiogenesis, confluent fibrosis, and fatty infiltration may help to improve the sensitivity of endomyocardial biopsy for detecting cardiac sarcoidosis. These novel histologic findings are based on the pathology of cardiac sarcoidosis. We also discussed the principal histologic differential diagnoses of cardiac sarcoidosis, such as tuberculosis myocarditis, fungal myocarditis, giant cell myocarditis, and dilated cardiomyopathy.

A Rare Case of Systemic Sarcoidosis in a Middle-Aged Female with a Solitary Thyroid Nodule

Sarcoidosis is a multisystem inflammatory disease characterized by the presence of noncaseating granulomas. Sarcoidosis can affect any organ of the body, the lung being the most affected. Sarcoidosis rarely affects the thyroid gland, and only a few cases of thyroid-related sarcoidosis have been reported in the literature. Herein, we report a case of systemic sarcoidosis with multiple organ involvement in a patient with a solitary left thyroid nodule and benign Fine Needle Aspiration (FNA) findings. The patient was eventually diagnosed with thyroid sarcoidosis using a core needle biopsy of the thyroid nodule. With this case report, we highlight the limitations of the FNA. This case report has the objective to encourage clinicians to include thyroid sarcoidosis in the differential diagnosis of thyroid nodules in patients with systemic sarcoidosis even with unremarkable FNA findings.

PET Imaging in Cardiac Sarcoidosis: A Narrative Review with Focus on Novel PET Tracers

Sarcoidosis is a multi-system inflammatory disease characterized by the development of inflammation and noncaseating granulomas that can involve nearly every organ system, with a predilection for the pulmonary system. Cardiac involvement of sarcoidosis (CS) occurs in up to 70% of cases, and accounts for a significant share of sarcoid-related mortality. The clinical presentation of CS can range from absence of symptoms to conduction abnormalities, heart failure, arrhythmias, valvular disease, and sudden cardiac death. Given the significant morbidity and mortality associated with CS, timely diagnosis is important. Traditional imaging modalities and histologic evaluation by endomyocardial biopsy often provide a low diagnostic yield. Cardiac positron emission tomography (PET) has emerged as a leading advanced imaging modality for the diagnosis and management of CS. This review article will summarize several aspects of the current use of PET in CS, including indications for use, patient preparation, image acquisition and interpretation, diagnostic and prognostic performance, and evaluation of treatment response. Additionally, this review will discuss novel PET radiotracers currently under study or of potential interest in CS.

A Woman With Subacute Numbness of the Trunk, Arms, and Legs

A 39-year-old woman had development of new-onset numbness in her left arm. This progressed over 2 to 3 weeks to involve the left axilla, trunk, lower extremities, and genital region. She had mild imbalance and left-sided weakness but remained ambulating without a gait aid. Repeated magnetic resonance imaging of the cervical spine showed the longitudinally extensive T2 lesion in a central location on axial sequences, with linear, dorsal, subpial gadolinium enhancement extending more than 2 vertebral segments. The magnetic resonance image findings were most suggestive of spinal cord neurosarcoidosis. Computed tomography of the chest showed bilateral hilar adenopathy. Serum levels of angiotensin-converting enzyme were normal. Transbronchial lung biopsy showed noncaseating granulomas. Noncaseating granulomas were confirmatory of pulmonary sarcoidosis, which led to a diagnosis of spinal cord neurosarcoidosis. Treatment with intravenous methylprednisolone for 5 days was repeated, followed by oral prednisone for 2 months. Her neurologic symptoms improved, and repeated magnetic resonance image showed a marked decrease in T2 hyperintensity and gadolinium enhancement consistent with interval response to treatment. A slow prednisone taper over 9 months was initiated. Spinal cord neurosarcoidosis often presents with an isolated myelopathy without symptoms of pulmonary sarcoidosis (eg, cough, dyspnea). The presentation can range from a subacute onset mimicking transverse myelitis (as in this case patient) to a more insidious progressive myelopathy over months to years.

Diplopia, Orbital Pain, and Vision Loss in a Middle-Aged Woman

A 59-year-old woman with type 2 diabetes had development of fluctuating, binocular, painless diplopia. She experienced headache, orbital pain, facial numbness, and progressive vision loss in the left eye. Her left eye vision worsened. Magnetic resonance imaging of the brain and orbit showed bilateral optic nerve enhancement. She started treatment with empiric intravenous corticosteroids for presumed optic neuritis, which resulted in transient improvement. Subsequently, her vision worsened to no light perception in the left eye, and she had fluctuating vision loss in the right eye. Cerebrospinal fluid evaluation was performed because the patient’s fluctuating diplopia and facial numbness suggested involvement of multiple cranial nerves. The cerebrospinal fluid showed white blood cells with lymphocytes, increased protein concentration, and negative oligoclonal bands and cytologic findings. Repeated brain magnetic resonance imaging 1 year after symptom onset showed persistent bilateral (left > right) optic nerve enhancement along with oculomotor nerve and left midbrain enhancement. We recommended left optic nerve biopsy to obtain the diagnosis and attempt to preserve vision in the threatened right eye. Because the patient had no light perception in the left eye for 5 months, with significant pallor of the optic nerve, it was unlikely that she had salvageable left eye vision. Furthermore, there was concern for lymphomatous infiltration of the optic nerve. Confirmation of a lymphoma diagnosis would be critical for initiation of specific chemotherapy. The pathologic analysis identified noncaseating granulomas. The finding of noncaseating granulomas was consistent with neurosarcoidosis infiltrating the left optic nerve. The patient was treated with intravenous methylprednisolone followed by prolonged, high-dose, oral corticosteroids along with corticosteroid prophylaxis of calcium, vitamin D, a proton-pump inhibitor, and dapsone for Pneumocystis prophylaxis Sarcoidosis is a systemic disease of unknown cause that can occur anywhere in the body but most commonly involves the lungs. The pathologic hallmark of sarcoidosis is noncaseating granulomas.

A Case Of Disseminated Histoplasmosis In A Patient With Sarcoidosis

Introduction/Objective Sarcoidosis is a syndrome of unknown cause that may manifest with clinical, radiographic and pathological findings similar to those seen with histoplasmosis. We present a case of disseminated histoplasmosis in an immunocompetent patient previously diagnosed with sarcoidosis. Methods/Case Report A 69-year-old obese male with a history of hypertension, diabetes mellitus and long-standing sarcoidosis was admitted to the hospital for several months of intermittent fevers and pancytopenia. His sarcoidosis was diagnosed 21 years prior, initially involving the lungs and eventually showing cardiac involvement, requiring a pacemaker. He had been treated with methotrexate and prednisone. His recent medical history was also significant for COVID-19 infection, diagnosed 3 months before admission. His fevers were initially attributed to sarcoidosis and his pancytopenia to methotrexate. However, his symptoms continued despite discontinuation of his medications, and further workup was initiated. Computed tomography showed hepatomegaly, splenomegaly, and lymphadenopathy, concerning for a lymphoproliferative disorder. The patient underwent a bone marrow biopsy that showed noncaseating granulomas and microorganisms consistent with histoplasmosis on fungal stain. Bone marrow cultures were not possible as the marrow was inaspirable. The patient subsequently underwent a lymph node biopsy with both morphology and culture identifying histoplasmosis. Urine and serum histoplasma antigen also returned positive. The patient’s overall clinical picture was consistent with disseminated histoplasmosis and he was administered intravenous Amphotericin B for 3 weeks followed by oral itraconazole for 1 year. One month follow-up after discharge showed significant improvement in the patient’s condition. Results (if a Case Study enter NA) N/A Conclusion Sarcoidosis reduces T-cell activity, and treatment with steroids causes further immunosuppression and vulnerability for development of a disseminated infection. COVID-19 also presumably increases the predisposition to acquire bacterial or fungal co-infections. Clinicians and pathologists should be aware of the overlap in clinical, radiologic and pathological presentations of sarcoidosis and histoplasmosis to make the correct diagnosis and administer the appropriate treatment.

Tubulointerstitial nephritis and uveitis syndrome in an adolescent female: a case report

Background Tubulointerstitial nephritis with uveitis syndrome is a rare disease affecting mainly children and young women. Tubulointerstitial nephritis with uveitis is a diagnosis of exclusion, requiring a high degree of clinical suspicion. Studies report recent infections or certain drugs as precipitating factors of a lymphocytic oculorenal immune response. The prognosis is usually favorable with topical and systemic corticosteroid therapy. Case presentation We report a literature review and the case of a 14-year-old white girl, who presented to the ophthalmology department with features of one-sided uveitis. Upon transfer of patient to nephrological care, diagnostic work-up revealed renal involvement. Renal biopsy showed a mixed-cell and granulomatous tubulointerstitial nephritis with some noncaseating granulomas, leading to a diagnosis of tubulointerstitial nephritis with uveitis syndrome. With topical ocular and systemic corticosteroid therapy, the patients’ condition improved over several weeks. Conclusions Our case highlights the importance of early recognition and treatment of this syndrome, where cross-specialty care typically leads to a favorable outcome.

Sarcoidosis presenting as bilateral lacrimal gland swelling: a pediatric case report

Background To describe a case of pediatric sarcoidosis which initially presented as papillary conjunctivitis before manifesting as bilateral lacrimal gland swelling without other known systemic involvement. Case presentation A 10-year-old female presented to the pediatric ophthalmology clinic with complaints of bilateral eyelid swelling, tearing and itching for approximately 1 month. Her history and exam were most consistent with allergic conjunctivitis, for which she was started on a standard topical regimen. Despite initial improvement, she re-presented with significantly worsened eyelid swelling and minimal allergic symptoms. Enlargement of the lacrimal glands were palpable at this time. Lacrimal gland biopsy was obtained which demonstrated noncaseating granulomas. Systemic workup did not reveal evidence of disease involvement elsewhere. Conclusions Sarcoidosis in the pediatric population may present in a myriad of ways and is well-known to mimic other disease entities. We present a case of pediatric sarcoidosis which presented initially as papillary conjunctivitis before manifesting as bilateral lacrimal gland swelling without systemic involvement.

A CLINICAL CASE OF PROGRESSIVE COURSE OF SARCOIDOSIS OF THE RESPIRATORY ORGANS

Sarcoidosis is a systemic inflammatory disease of unknown etiology, characterized by the formation of noncaseating granulomas and multiple system organ damage. Sarcoidosis therapy is aimed at prevention and reducing inflammation as well as improving patient’s symptoms and quality of life. Active observation is preferred for patients with morphologically confirmed diagnosis in the absence of a lifethreatening condition, organ and system dysfunction as well as disease progression. Alternative therapy may include alphatocopherol and/or pentoxifylline. Treatment with glucocorticosteroids is used for a long period of time (12-24 months) only when clinically indicated. The presented clinical case of progressive course of respiratory organ sarcoidosis is intended to draw the attention of doctors to the peculiarities of examination, treatment and observation of such patients. The case is discussed from the perspective of personalized medicine and modern clinical guidelines.

Sarcoidosis vs. Colon Cancer Metastasis: Diagnostic Dilemma and the Role of PET Scan in Monitoring Disease Activity

Sarcoidosis is a systemic inflammatory disorder characterized by “noncaseating granulomas.” It primarily affects the lungs, but multiple other organs can be involved. Sarcoidosis has been increasingly reported in association with cancer. It can precede, follow or occur at the same time as the diagnosis of cancer. We report a case of sarcoidosis that was diagnosed concomitantly with colon cancer, highlighting the diagnostic dilemma of sarcoidosis vs. cancer metastasis, the relationship between the two, and the value of PET scan in follow-up and monitoring of disease activity.