Case Reports in Medicine / 2020 / Article / Tab 3

Case Report

Necrotizing Sarcoid Granulomatosis: A Disease Not to be Forgotten

Table 3

Main differential diagnosis of NGS and their typical characteristics. GPA: granulomatosis with polyangiitis; FSGS: focal segmental glomerulosclerosis; EGPA: eosinophilic granulomatosis with polyangiitis; TB: tuberculosis; NTM: nontuberculous mycobacteria; BAL: bronchoalveolar lavage; ANCA: antineutrophil cytoplasmic antibodies; CRP: C-reactive protein; SIADH: syndrome of inappropriate antidiuretic hormone secretion; TC: computed tomography; and AFB: acid-fast bacilli.

GPAEGPATBNTM

EpydemiologyMean age at diagnosis: 40–60 years
No gender predominance
Mean age at diagnosis: 40 years
No gender predominance
100 per 100,000 or higher: Sub-Saharan Africa, India, and the islands of Southeast Asia and Micronesia
26 to 100 cases per 100,000: China, central and South America, Eastern Europe, and northern Africa
Less than 25 cases per 100,000: United States, Western Europe, Canada, Japan, and Australia
Environmental contaminants in soil and water, having been isolated from the domestic water distribution network, hot tubs, swimming pools, and workplaces
HistologyGranulomatous inflammation, vasculitis, and necrosisEosinophilic infiltration
Areas of necrosis
Interstitial and perivascular necrotizing granulomas
An eosinophilic, giant cell vasculitis, especially of the small arteries and veins
Granulomas caseating which contain epithelioid macrophages, Langhans giant cells, and lymphocytesGranulomatous inflammation
Clinical presentationConstitutional symptoms (fever, malaise, anorexia, and weight loss)
Ear, nose, and throat manifestations (nasal crusting, sinusitis, otitis media, earache, otorrhea, persistent rhinorrhea, purulent/bloody nasal discharge, oral and/or nasal ulcers, and polychondritis)
Tracheal and pulmonary disease (nodules cavitary, and pulmonary opacities)
Renal manifestations (FSGS)
Poorly controlled asthma and lung disease (migratory infiltrates, pleural effusion, nodules rarely cavitary, and alveolar hemorrhage)
Upper airway and ear disease
Skin involvement
Peripheral neuropathy (mononeuritis multiplex)
Constitutional symptoms (fever, malaise, and weight loss)
Primary disease: pleuritic chest pain, fatigue, cough, arthralgia, and pharyngitis
Persistent fever, night sweats, weight loss, fatigue, malaise, and anorexia
Pulmonary disease
Superficial lymphadenitis
Skin and soft tissue infection
Laboratory testsANCA positive
Urinary sediment disorder
Peripheral blood eosinophilia
ANCA positive
BAL: high percentage of eosinophils in the lavage fluid
CRP elevated. Leukocytosis Hyponatremia, may be associated with the SIADHElevated acute phase reactants
DiagnosisBiopsy of a site of suspected active diseaseSurgical lung biopsyRadiographic imaging (radiography and TC) and microbiologic testing (sputum AFB smear, mycobacterial culture, and molecular tests)Recurrent isolation of mycobacteria from sputum or isolated from at least one bronchial wash in a symptomatic patient
Culture of blood for mycobacteria