Sarcoidosis

Sarcoidosis is a systemic disease characterized by the presence of noncaseating granulomas, which accumulate in affected organs. The incidence, organ involvement, and disease severity depend on environmental exposures and host factors. The cause of sarcoidosis remains unknown. Any organ can be affected; however, involvement of the lung, heart, and nervous system contributes most to morbidity and mortality. This review discusses the epidemiology, etiology, genetics, pathogenesis, diagnosis (including clinical manifestations), differential diagnosis, management, complications, and prognosis of sarcoidosis. Figures depict the pathogenesis of sarcoidosis, radiographic stages of sarcoidosis, contrast-enhanced magnetic resonance imaging of a patient with neurosarcoidosis, noncaseating granuloma from a tissue biopsy of a patient with sarcoidosis, various manifestations of sarcoidosis, approach to the use of anti-inflammatory therapy for sarcoidosis, and an algorithm for symptomatic disease. This review contains 7 highly rendered figures, 7 tables, and 127 references Keywords: cardiac sarcoidosis, hilar lymphadenopathy, Löfgren syndrome, neurosarcoidosis, noncaseating granuloma, sarcoidosis, uveitis

Sarcoidosis

Sarcoidosis is a systemic disease characterized by the presence of noncaseating granulomas, which accumulate in affected organs. The incidence, organ involvement, and disease severity depend on environmental exposures and host factors. The cause of sarcoidosis remains unknown. Any organ can be affected; however, involvement of the lung, heart, and nervous system contributes most to morbidity and mortality. This review discusses the epidemiology, etiology, genetics, pathogenesis, diagnosis (including clinical manifestations), differential diagnosis, management, complications, and prognosis of sarcoidosis. Figures depict the pathogenesis of sarcoidosis, radiographic stages of sarcoidosis, contrast-enhanced magnetic resonance imaging of a patient with neurosarcoidosis, noncaseating granuloma from a tissue biopsy of a patient with sarcoidosis, various manifestations of sarcoidosis, approach to the use of anti-inflammatory therapy for sarcoidosis, and an algorithm for symptomatic disease. This review contains 7 highly rendered figures, 3 tables, and 126 references Keywords: cardiac sarcoidosis, hilar lymphadenopathy, Löfgren syndrome, neurosarcoidosis, noncaseating granuloma, sarcoidosis, uveitis

Cutaneous Sarcoidosis- A Report of Two Intriguing Cases

Sarcoidosis is an inflammatory disease which leads to formation of noncaseating granuloma in the affected organs like lungs, lymph nodes and less commonly the skin. Cutaneous sarcoidosis occurs in about 20-35% of individuals with systemic sarcoidosis. Skin involvement serves as an early marker and may be the presenting sign of systemic sarcoidosis. Wide variety of morphological forms of cutaneous sarcoidosis is known to occur simulating several other dermatoses. Isolated skin involvement is very rare. Histopathological examination and special stains plays an important role in ruling out other granulomatous conditions like cutaneous tuberculosis, leprosy, foreign body granuloma and granuloma annulare from sarcoidosis. Corticosteroids remain the main cornerstone in the treatment of cutaneous sarcoidosis. Here, the authors reported two interesting cases of cutaneous sarcoidosis without systemic involvement, of which one presented as micropapular sarcoidosis and the other was mimicking lupus vulgaris. Both of them responded well to corticosteroid treatment and are in regular monitoring for development of systemic symptoms. Informed consent was obtained was obtained from both the patients.

Scar sarcoidosis: additional involvement of de novo sites and update on immunopathogenesis

Cutaneous sarcoidosis is an immune-mediated multisystem granulomatous inflammatory disease of unknown etiology. Noncaseating granuloma in pre-existing scars is an uncommon but specific cutaneous manifestation of sarcoidosis. This great imitator may be an under-diagnosed disease in developing countries like India probably because of the diverse range of clinical presentations. Herein we present a 45-year old female with cutaneous sarcoidosis involving scars in addition to de-novo sites.

Sarcoidosis of the pleura: A case report

Introduction. Pleural involvement is an uncommon manifestation of sarcoidosis. It may manifest as pleural effusion, pneumothorax, pleural thickening and nodules, hydropneumothorax, trapped lung, hemothorax, or chylothorax. The incidence of pleural effusion with sarcoidosis ranges from 0% to 5% but has been reported to be as high as 7.5%. Pleural effusions complicate sarcoidosis in < 3% of patients. Case report. We reported a 64-year-old male patient with chronic multiorgan sarcoidosis. This patient developed pleural sarcoidosis with massive pleural effusion several years after the diagnosis of sarcoidosis. A definitive diagnosis of a sarcoid pleural effusion was based on a biopsy demonstrating noncaseating granuloma. The patient responded well to the treatment (methotrexate and methylprednisolone) with a complete withdrawal of pleural effusion following five weeks of the treatment beginning. Conclusion. The presented patient is a rare case of pleural involvement of sarcoidosis with massive effusion, who responded well to the treatment.

Aspergilloma formation in cavitary sarcoidosis

Pulmonary cavitation is rather uncommon in patients with sarcoidosis, and aspergilloma is even more uncommon in such cases. Here, we present the case of a 63-year-old female patient with cavitary lung disease who had been under treatment for TB for 9 months. A diagnosis of pulmonary sarcoidosis was established based on the fiberoptic bronchoscopy finding of noncaseating granuloma. Treatment with corticosteroids led to a dramatic improvement in symptoms. While under treatment for sarcoidosis, the patient developed an aspergilloma. She presented immediate skin test reactivity to Aspergillus fumigatus, as well as positivity for A. fumigatus serum precipitins. This is the first reported case of aspergilloma formation in a patient with cavitary sarcoidosis in India.