Disseminated histoplasmosis
Updates to Article Attributes
Disseminated histoplasmosis, also known as progressive disseminated histoplasmosis, is a severe form of histoplasmosis infection typically seen in immunosuppressed patients, especially in the setting of HIV infection. It results from haematogeneous dissemination of the infection, involving multiple organs and systems and associating a high mortality1.
Terminology
Although it is sometimes referred to as extrapulmonary histoplasmosis, lung involvement is one of the commonest manifestations of this condition2.
Epidemiology
Dissemination of the disease is fairly uncommon and may occur in 1 in 2000 acute infections3.
Risk factors
HIV infection, especially with less than 150 cells/μL CD41,4
Immunosuppressive medications4
Biologic agents (infliximab, etanercept)3
Immune disorders (idiopathic CD4 lymphocytopenia, Job's syndrome, common variable immunodeficiency)4
Extremes of ages4
Clinical presentation
Clinical presentation includes systemic symptoms such as fever, weight loss and fatigue and local systems depending on the organs involved, with frequent progressive shortness of breath and diarrhoea1,3.
Physical examination may reveal hepatoesplenomegaly, lymphadenopathy and skin and oral lesions1.
Location
Lungs: parenchymal disease2.
Gastrointestinal: any site of the digestive tube can be involved, with potential bleeding or perforation1,4.
Liver and spleen: haemophagocytic lymphohistiocytosis with macrophage activation syndrome1.
Kidneys: granulomatous nephritis1.
Adrenal glands: adrenal insuficciency1,3.
Central nervous system: meningeal, brain or spinal cord lesions1.
Skin1
Radiographic features
Although multiple organs and systems can be affected, only pulmonary and gastrointestinal manifestations have been completely described.
Pulmonary
Chest radiographs show pulmonary abnormalities in about 50% of the patients2. Diffuse multiple small nodules are the most common pattern, even though linear and irregular opacities or segmental, lobar or diffuse air-space opacities can also be observed2,5.
CT demonstrates diffuse miliary nodules measuring 1-3 mm3. Pleural effusions and thoracic lymphadenopathy are uncommon2,3. Calcified lymph nodes or granulomas are described in about one third of patients, indicating previous contact with the microorganism2.
Gastrointestinal
Others
-<p><strong>Disseminated histoplasmosis</strong>, also known as<strong> progressive disseminated histoplasmosis</strong>, is a severe form of <a href="/articles/histoplasmosis" title="Histoplasmosis">histoplasmosis infection</a> typically seen in immunosuppressed patients, especially in the setting of <a href="/articles/hivaids" title="HIV">HIV infection</a>. It results from haematogeneous dissemination of the infection, involving multiple organs and systems and associating a high mortality<sup>1</sup>.</p><h4>Terminology</h4><p>Although it is sometimes referred to as extrapulmonary histoplasmosis, lung involvement is one of the commonest manifestations of this condition<sup>2</sup>.</p><h4>Epidemiology</h4><p>Dissemination of the disease is fairly uncommon and may occur in 1 in 2000 acute infections<sup>3</sup>.</p><h5>Risk factors</h5><ul>-<li><p><a href="/articles/hivaids" title="HIV AIDS">HIV infection</a>, especially with less than 150 cells/μL CD4<sup>1,4</sup></p></li>-<li><p>Immunosuppressive medications<sup>4</sup></p></li>-<li><p>Biologic agents (infliximab, etanercept)<sup>3</sup></p></li>-<li><p>Immune disorders (idiopathic CD4 lymphocytopenia, <a href="/articles/hyperimmunoglobulin-ige-syndrome" title="Job's syndrome ">Job's syndrome</a>, <a href="/articles/common-variable-immunodeficiency" title="Common variable immunodeficiency">common variable immunodeficiency</a>)<sup>4</sup></p></li>-<li><p>Extremes of ages<sup>4</sup></p></li>-</ul><h4>Clinical presentation</h4><p>Clinical presentation includes systemic symptoms such as fever, weight loss and fatigue and local systems depending on the organs involved, with frequent progressive shortness of breath and diarrhoea<sup>1,3</sup>.</p><p>Physical examination may reveal hepatoesplenomegaly, lymphadenopathy and skin and oral lesions<sup>1</sup>.</p><h4>Location</h4><ul>-<li><p>Lungs: parenchymal disease<sup>2</sup>.</p></li>-<li><p>Gastrointestinal: any site of the digestive tube can be involved, with potential bleeding or perforation<sup>1,4</sup>.</p></li>-<li><p>Liver and spleen: haemophagocytic lymphohistiocytosis with macrophage activation syndrome<sup>1</sup>.</p></li>-<li><p>Kidneys: granulomatous nephritis<sup>1</sup>.</p></li>-<li><p>Adrenal glands: adrenal insuficciency<sup>1,3</sup>.</p></li>-<li><p>Central nervous system: meningeal, brain or spinal cord lesions<sup>1</sup>.</p></li>-<li><p>Skin<sup>1</sup></p></li>- +<p><strong>Disseminated histoplasmosis</strong>, also known as<strong> progressive disseminated histoplasmosis</strong>, is a severe form of <a href="/articles/histoplasmosis" title="Histoplasmosis">histoplasmosis infection</a> typically seen in immunosuppressed patients, especially in the setting of <a href="/articles/hivaids" title="HIV">HIV infection</a>. It results from haematogeneous dissemination of the infection, involving multiple organs and systems and associating a high mortality<sup>1</sup>.</p><h4>Terminology</h4><p>Although it is sometimes referred to as extrapulmonary histoplasmosis, lung involvement is one of the commonest manifestations of this condition<sup>2</sup>.</p><h4>Epidemiology</h4><p>Dissemination of the disease is fairly uncommon and may occur in 1 in 2000 acute infections<sup>3</sup>.</p><h5>Risk factors</h5><ul>
- +<li><p><a href="/articles/hivaids" title="HIV AIDS">HIV infection</a>, especially with less than 150 cells/μL CD4<sup>1,4</sup></p></li>
- +<li><p>Immunosuppressive medications<sup>4</sup></p></li>
- +<li><p>Biologic agents (infliximab, etanercept)<sup>3</sup></p></li>
- +<li><p>Immune disorders (idiopathic CD4 lymphocytopenia, <a href="/articles/hyperimmunoglobulin-ige-syndrome" title="Job's syndrome ">Job's syndrome</a>, <a href="/articles/common-variable-immunodeficiency" title="Common variable immunodeficiency">common variable immunodeficiency</a>)<sup>4</sup></p></li>
- +<li><p>Extremes of ages<sup>4</sup></p></li>
- +</ul><h4>Clinical presentation</h4><p>Clinical presentation includes systemic symptoms such as fever, weight loss and fatigue and local systems depending on the organs involved, with frequent progressive shortness of breath and diarrhoea<sup>1,3</sup>.</p><p>Physical examination may reveal hepatoesplenomegaly, lymphadenopathy and skin and oral lesions<sup>1</sup>.</p><h4>Location</h4><ul>
- +<li><p>Lungs: parenchymal disease<sup>2</sup>.</p></li>
- +<li><p>Gastrointestinal: any site of the digestive tube can be involved, with potential bleeding or perforation<sup>1,4</sup>.</p></li>
- +<li><p>Liver and spleen: haemophagocytic lymphohistiocytosis with macrophage activation syndrome<sup>1</sup>.</p></li>
- +<li><p>Kidneys: granulomatous nephritis<sup>1</sup>.</p></li>
- +<li><p>Adrenal glands: adrenal insuficciency<sup>1,3</sup>.</p></li>
- +<li><p>Central nervous system: meningeal, brain or spinal cord lesions<sup>1</sup>.</p></li>
- +<li><p>Skin<sup>1</sup></p></li>