SciCombinator

Discover the most talked about and latest scientific content & concepts.

Concept: Sarcoidosis

166

BACKGROUND: Sarcoidosis is a systemic disease characterized by the formation of noncaseating granulomas in various tissues. Cutaneous involvement occurs in 20 to 35 percent of the patients and may be the initial manifestation of the disease. Our study was performed to discriminate the clinical, laboratory, and prognostic differences between patients with specific and nonspecific cutaneous involvement. The second aim was to asses the diagnostic usefulness of punch biopsy in sarcoidosis. METHODS: The clinical, laboratory, pathological features, and skin biopsy results of 120 patients with cutaneous sarcoidosis were evaluated. The patients fulfilled clinical, radiologic or both features of sarcoidosis supported by the histopathologic evidence of noncaseating granulomas.Skin involvement was the initial finding in 30% of the patients. Erythema nodosum and lupus pernio were the most common skin lesions. Almost all of the patients with LP were either stage 0 or 1. Respiratory symptoms occurred in 72.2% of the patients with specific skin involvement. BronchoalveolarLavage (BAL) lymphocytosis, high ratio of CD4/CD8 and elevated serum Angiotensin Converting Enzyme (ACE) were more frequent in patients with specific cutaneous lesions. The frequency of progressive disease was significantly higher in this group. Punch skin biopsy was diagnostic in 81.6% of the patients with a complication rate of 4%. CONCLUSIONS: Specific cutaneous lesions along with BAL lymphocytosis, high CD4/CD8 ratio and elevated serum ACE levels may be predictors of progressive disease in sarcoidosis. Punch biopsy is a simple technique with a high diagnostic yield and a low complication rate for cutaneous sarcoidosis.

Concepts: Medical terms, Biopsy, Pathology, Sarcoidosis, Granuloma, Skin biopsy, Erythema nodosum, Lupus pernio

165

Mycobacterium tuberculosis (MTB) infection has been suggested to contribute to the pathogenesis of erythema nodosum (EN) and nodular vasculitis (NV), the classic forms of panniculitis. However, there is little evidence to demonstrate the presence of MTB in the skin lesions. This study is aimed at evaluating the association between MTB infection and the development of EN and NV in a Chinese population.

Concepts: Tuberculosis, The Association, Mycobacterium, Mycobacterium tuberculosis, Sarcoidosis, Erythema nodosum, Behçet's disease, Conditions of the subcutaneous fat

27

Erythema nodosum (EN) is the most common form of panniculitis. It is characterized by erythematous, raised, tender nodules that usually occur bilaterally on the extensor surfaces of the lower extremities. EN is associated with many underlying conditions including infection, sarcoidosis, malignancy, and pregnancy. Its underlying etiology, however, is unknown in up to half of cases. Pregnancy is thought to create an optimal background for EN to develop, although the exact mechanisms are unclear. Immune complexes may play a role in the pathogenesis of EN during pregnancy, or EN may be a hypersensitivity reaction to either estrogens or progesterone. EN is a self-limiting process, and non-pharmacologic means such as bed rest and elastic web bandages may be sufficient to control the symptoms. Potassium iodide, systemic and intralesional corticosteroids, non-steroidal anti-inflammatory drugs, salicylates, tumor necrosis factor-α inhibitors, hydroxychloroquine, colchicine, and dapsone are other treatment options available, but some of these drugs are contraindicated in pregnancy while others are considered safe. Before prescribing one of these treatments to a pregnant patient, the patient’s obstetrician should be consulted, and a careful risk-benefit analysis should be performed.

Concepts: Immune system, Pregnancy, Hypersensitivity, Non-steroidal anti-inflammatory drug, Progesterone, Sarcoidosis, Erythema nodosum, Potassium iodide

27

Takayasu’s arteritis is a rare, systemic vasculitis with varied presentations across multiple medical specialities. Here, we present a young woman who had recurrent episodes of erythema nodosum on the background of a low-grade fever and no vascular manifestations. The presence of a high erythrocyte sedimentation rate generated a high index of suspicion for underlying vasculitis, and radioimaging confirmed the suspicion of Takayasu’s arteritis. The patient was found to have type III diseases in the vasculitic stage and was managed with systemic corticosteroids.

Concepts: Cardiology, Rheumatoid arthritis, Vasculitis, Hematology, Hodgkin's lymphoma, Sarcoidosis, Takayasu's arteritis, Erythema nodosum

27

PurposeTo analyze the incidence and clinical course of patients developing progressive ocular inflammation following anti-tubercular therapy (ATT) for presumed ocular tuberculosis (TB).MethodsRetrospective analysis of medical records of patients who received ATT for presumed ocular TB and completed at least 12 months follow-up after initiation of ATT. The diagnosis of presumed ocular TB was based on presence of ocular signs suggestive of TB, evidence of past tubercular infection, and exclusion of mimicking clinical entities. All patients received a combination of ATT and corticosteroid therapy. Primary outcome measure was progression (worsening) of ocular inflammation, defined as a two-step increase in level of inflammation (anterior chamber/ vitreous) or the appearance of new lesions following initiation of ATT.ResultsA total of 106 patients (64 male, 42 female) received ATT for presumed ocular TB. Twenty-six (24.5%) patients developed progressive intraocular inflammation following ATT. Primary diagnoses in these patients were: anterior uveitis (n=1), intermediate uveitis (n=9), retinal vasculitis (n=3), serpiginous-like choroiditis (n=7), multifocal choroiditis (n=2), and pan-uveitis (n=4). Following progressive inflammation, diagnosis was revised in two patients (7.7%)-both responded to alternative therapy. Of the rest, majority (n=16; 61.5%) resolved with escalation of corticosteroid therapy. Five patients (19.2%)-all having intermediate uveitis-required therapeutic vitrectomy for resolution. Three patients (11.5%) had persistent inflammation at end of follow-up period.ConclusionProgressive inflammation following ATT for presumed ocular TB is common. It generally resolves on escalation of corticosteroid therapy. Cases not responding to increased immunosuppression need to be re-investigated to rule out a nontubercular cause.Eye advance online publication, 1 March 2013; doi:10.1038/eye.2013.5.

Concepts: Vasculitis, Corticosteroid, Retina, Ophthalmology, Progressive lens, Sarcoidosis, Uveitis, Escalation

25

Permenant make-up is a kind of cosmetic tattoo in which the colorants (pigments) are deposited in dermis after piercing the skin by tiny solid needles.It may cause some adverse effects such as local inflammation, infection and allergic reactions on the skin and even systemic adverse effects such as sarcoidal reactions. Here is described a 34-year-old woman who has some yellowish hard shiny papules on her eyebrows after having a permanent make-up. The histopathological examinations of the papules are diagnosed as sarcoidal foreign body reactions. All the laboratory investigations were in normal limits except a mild elevetion of ACE level. The lesions mostly improved after topical corticosteroid treatment. Sarcoidal foreign body reaction due to permenant make-up is discussed with this presentation.

Concepts: Immune system, Asthma, Immunology, Corticosteroid, Skin, Sarcoidosis, Permanent makeup, Foreign body reaction

10

Granulomatous lung diseases are a heterogeneous group of disorders that have a wide spectrum of pathologies with variable clinical manifestations and outcomes. Precise clinical evaluation, laboratory testing, pulmonary function testing, radiological imaging including high-resolution computed tomography and often histopathological assessment contribute to make a confident diagnosis of granulomatous lung diseases. Differential diagnosis is challenging, and includes both infectious (mycobacteria and fungi) and noninfectious lung diseases (sarcoidosis, necrotising sarcoid granulomatosis, hypersensitivity pneumonitis, hot tub lung, berylliosis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, rheumatoid nodules, talc granulomatosis, Langerhans cell histiocytosis and bronchocentric granulomatosis). Bronchoalveolar lavage, endobronchial ultrasound-guided transbronchial needle aspiration, transbronchial cryobiopsy, positron emission tomography and genetic evaluation are potential candidates to improve the diagnostic accuracy for granulomatous lung diseases. As granuloma alone is a nonspecific histopathological finding, the multidisciplinary approach is important for a confident diagnosis.

Concepts: Pulmonology, Pathology, Positron emission tomography, Anatomical pathology, Tuberculosis, Medical diagnosis, Sarcoidosis, Granuloma

5

This retrospective study investigates the relationship between cardiac and extra-thoracic sarcoid findings on FDG PET-CT using a 72-hour pretest high-fat, high-protein, and very low-carbohydrate (HFHPVLC) diet.

Concepts: Sarcoidosis

4

Primitive lung cancers developed on lung fibroses are both diagnostic and therapeutic challenges. Their incidence may increase with new more efficient lung fibrosis treatments. Our aim was to describe a cohort of lung cancers associated with idiopathic pulmonary fibrosis (IPF) and other lung fibrotic disorders (non-IPF), and to characterize their molecular alterations using immunohistochemistry and next-generation sequencing (NGS).

Concepts: Epidemiology, Pulmonology, Lung cancer, Fibrosis, Idiopathic pulmonary fibrosis, Pulmonary fibrosis, Sarcoidosis, Interstitial lung disease

4

There is currently no effective treatment for acute exacerbation of idiopathic pulmonary fibrosis (IPF). We herein report the case of a patient with acute exacerbation of IPF which was treated with nintedanib, an intracellular inhibitor of tyrosine kinases, and showed improvement of the condition. An 84-year-old man with IPF was admitted to our hospital because of dry cough and worsening of dyspnoea within last 1 month. He presented with hypoxemia, and chest high-resolution computed tomography (HRCT) revealed new, bilateral multifocal ground-glass opacities superimposed on the background of lung fibrosis. After exclusion of alternative causes, acute exacerbation of IPF was diagnosed and we started treatment with nintedanib of 300 mg/day. This resulted in the gradual improvement of his condition and HRCT findings without administering antibiotics or corticosteroids. Serum Krebs von den Lungen-6 and surfactant protein D levels increased at acute exacerbation and subsequently decreased. This case suggests that nintedanib therapy may have possible benefits in acute exacerbation of IPF.

Concepts: Signal transduction, Asthma, Pneumonia, Fibrosis, Idiopathic pulmonary fibrosis, Pulmonary fibrosis, Bronchitis, Sarcoidosis