Neurological | Impact A Medical Profession

Percussion Neurological

Aug 04
2011

Sarcoidosis remission

Despite the fact that sarcoidosis is a disease spread by international experts failed to identify the exact causes of her appearance. The disease is associated with the existence of an abnormal immune response, but it triggers remain unidentified. One of the most important reasons for which cases can be studied is the lack of an animal model for this disease and the inability to detect the antigen that causes immune activation. Although extensive studies have been conducted, it could not determine which is the agent responsible for the appearance of granulomas that characterize the disease.a
Sarcoidosis remission-
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Potential cases were considered on all bacterial infections, drugs, toxic exposures, genetic factors and autoimmunity. Most experts believe that sarcoidosis is the result of complex interactions between environmental factors and increased susceptibility to the host organism. Following this interaction could activate the immune system, triggering the inflammatory events in various organs, which will lead to granulomas.

Because in sarcoidotice lesions (granulomas) in nodules was found genetic material (DNA and RNA) from Mycobacterium tuberculosis, scientists have intensely studied the influence of bacteria on the appearance of the disease. Other suspected bacteria were Propionibacterium acnes, Streptococci species, Borrelia burgdorferi, Mycoplasma species, and Nocardia species. Under analysis are (still) viruses such as Epstein Barr virus, herpes viruses, Coxsackie B virus, cytomegalovirus and retroviruses, and environmental factors (chemicals such as beryllium, aluminum, zirconium, titanium).

Very important are genetic factors, especially when considering simtpomele, disease severity and prognosis.Sarcoidosis is a chronic inflammatory disease that is characterized by an exaggerated immune response to an unknown antigen, but are expected to target the disease found in organs. The hallmark of the disease, sarcoid granuloma, most likely formed in response to a persistent antigenic stimulus (producing a permanent incentive to maintain the abnormal immune response), but can not be eliminated (or at least neutralized) effectively.

The inflammatory cascade is initiated locally, being driven increasingly more cells that release enzymes, cytokines, interleukins, which are nothing more than assault and maintain additional tissue injury. Tissue, especially lung cancer, mechanisms that respond with their own aggression, and secrete growth factors, tumor necrosis factors and cytokines.

As a result of this fight standing, acute inflammation turns from chronic local tissue will change permeability, capillary (vascular) will alter the mechanisms of cell proliferation, changes that will eventually lead to the appearance of granuloma. Persistent antigenic stimulation appears to be a key factor that maintains this inflammation and is responsible for the disease.

Although sarcoidosis can occur at any organ and often affects the lungs, the granulomas can be described in lymph nodes, eyes, ears, skin, liver, joints, nervous system and heart. Inside the granuloma cells secrete growth factors that favor the appearance of local fibrosis. This can sometimes be so severe that insufficient cause visceral.Symptoms of sarcoidosis can vary greatly from one patient to another, depending on the organs affected by disease process. Most patients complain dry cough, fatigue and shortness of breath, but the clinical picture may include the following:
- Chest pain, bone pain, abdominal pain;
- Fever, Malaysia, weight loss;
- erythematous nodules, painful, present in the skin;
- red eye, visual acuity and reducing hiperlacrimatie;
- painful joints, swollen;
- Pneumothorax, pulmonary atelectazii;
- restrictive respiratory syndrome following pleural effusions (pleurisy);
- painful lymph nodes in the neck, armpits, groin;
- nasal congestion or hoarseness;
- Pain in the hands, feet, bone or other region. Pain arising from the formation of cysts in the bones;
- Nephrolithiasis;
- Hepatomegaly;
- heart rhythm disturbances (arrhythmias), pericarditis, or heart failure;
- central nervous system disorders, hearing loss, meningitis, convulsions, headache;
- Psychiatric disorders: dementia, depression, psychosis.

For some patients, symptoms may occur suddenly and are severe from the beginning, while in others even when visceral lesions are present, they do not cause clinical symptoms (lumps appear on the routine radiological examinations, or performed for other reasons ).There is no single diagnostic test for sarcoidosis, the disease is very complex and having pluriorganica damage, will always carry out full investigations. For diagnosis of sarcoidosis is primarily require histological analysis of tissues affected, and the presence of X-rays to certify this granulomas (nodules).

Even if the clinical picture may contain elements of suspicion, the patient must be fully investigated and excluded a number of diseases that may have similar manifestations. Differential diagnosis is based on the patient's clinical symptoms. The doctor should exclude pulmonary infection with the formation of granulomas evolve (such as in particular mycobacterial and fungal) and malignancies (lymphoma that causes lymph nodes). Hypercalcemia in sarcoidosis can mimic metabolic diseases, and sometimes joint events can be interpreted as juvenile rheumatoid arthritis.

General medical history and physical examination remain important because they guide the diagnosis and suspicion based on other tests they will be required. After medical history can determine the patient's symptoms, time of onset and evolution of the time until the moment of presentation, if there were moments when the symptoms have worsened or have improved. Patient's underlying disease (along with their treatment), risk factors and personal history (previous illness) and side-hereditary diseases (close relatives) should also be clarified. Physical examination can be performed concurrently or consecutively with her history.

Following this examination can be seen in the changes occurring:
- Respiratory System: is dullness to percussion, diminished vesicular murmur due to interpunerii pleural fluid or the occurrence of calcifications, or pleural thickening;
- Lymphatic system: one of the most frequently performed Findings on physical examination is the existence of peripheral lymphadenopathy. Approximately 70% of children with sarcoidosis have palpable lymph nodes. They are firm, painless, discreet (do not grow very much) and there are no plans adherents deep nor to the superjacent (superior, such as eg skin, superficial lymph nodes in cases);
- Liver and spleen: hepatomegaly is relatively common, but the liver itself has only rarely affected;
- Eyes: patients may have (in up to 90% of cases) iridocyclitis (or anterior uveitis) and interstitial keratitis, band keratopathy, retinal vasculitis. The patient often describes eye pain, photophobia, and the eye is red, irritated, with hiperlacrimatie;
- Skin: more than 75% of young children with signs of skin sarcoidosis form of violet or yellow-brown papules distributed most often on the face. They can become itchy, and scratching the scratching the lesions appear. Other signs are skin lesions in the form of plaques, localized on the trunk, extremities, and buttocks. Nodules, hyperpigmented or hypopigmented lesions, ulcers, erythema nodosum and subcutaneous tumors and they appear frequently. Erythema nodosum is one of the defining signs of cutaneous sarcoidosis, especially in Europe, and consists in the appearance of painful nodules, reddish, poorly defined, in front of the leg, accompanied by pain and swelling in joints adjacent;
- Musculo-skeletal system: over 50% of patients for signs of arthritis. In a smaller percentage appears and joint pain (arthralgia), breaking of intra-articular, and more rarely, bone lesions. Localized cystic lesions may occur in the phalanges, metacarpals or metatarsals;
- Endocrine system and kidneys: Renal granulomatosis may cause proteinuria, leucocyturia, hematuria, hypertension and renal failure;
- Cardio-vascular system: the patient may present arrhythmias, conduction abnormalities (due to cardiac granulomas) or may occur pericardial, valvular disease, papillary muscle dysfunction;
- Nervous System: Sarcoidosis may affect the visual axis to 5% of patients, may cause seizures, neurologic signs (paralysis of cranial nerves).

Neurosarcoidoza may be manifested by facial nerve damage (the most common abnormality), optic nerve and the acoustic-vestibular. In children, sarcoidosis can occur in two distinct forms. A similar form of multisystemic aduti with frequent lymphadenopathy and pulmonary disease as well as general signs (fever and altered general state). Early-onset form that occurs in patients younger than 4 years, characterized by the triad formed by the characteristic rash, uveitis and arthritis.

In order to establish the correct diagnosis will make a series of special investigations, including blood tests, imaging tests, biopsies and histopathological analysis. Following laboratory evaluations can be observed acute phase reactants, C reactive protein, an increased ESR (erythrocyte sedimentation rate). Patients may have anemia, leukopenia and eosinophilia. At 75% of patients may occur hipergamaglobulinemie.

The most useful non-invasive investigation is chest radiography, observing her adenopathy is typically bilateral hilarious, but with normal lung parenchyma. High resolution computed tomography of the chest can be very useful in the delineation of possible parenchymal lesions hilarious. The CT may appear well-defined nodules, which are in contact with the pleura, interlobular septa and centrilobulare structures having a distribution peribronhovasculara (airlines around structures and vascular parenchyma).

Classically, after imaging investigations (radiological), impairment of pulmonary sarcoidosis was staged as follows:
- Stage 0: normal appearance of parenchyma;
- Stage 1: adenopathy accompanied by bilateral lymphadenopathy paratraheala hilarious;
- Stage 2: adenopathy hilarious bilateral pulmonary infiltrates;
- Stage 3: pulmonary infiltrates without adenopathy hilarious;
- Stage 4: Advanced fibrosis with retraction of the hilum, blisters, cysts and emphysema.

Patient II can be made:
- Functionality tests to assess pulmonary
- Bronchoscopy: bronchi and inspects the state of the node can extract a fragment of tissue for biopsy (which will be examined microscopically), which will then determine if it is an injury or infectious granuloma. Bronchoscopy involves placing a thin, flexible tube through the main airways until the lungs;
- Biopsy of one of the investigations is essential for diagnosis. Current guidelines recommend the collection of tissue from the most accessible organ that presents as granulomatous nodules suspected. Biopsy in children prefer to be harvested from peripheral lymph nodes from superficial skin lesions, and sometimes the conjunctiva. Erythema nodosum biopsy is not recommended, because in that area are not granulomas.

Transbronchial biopsy is recommended for adults and insist many are harvested specimens (4-5) in different lung regions accessible. If transbronchial biopsy can not be achieved or failed to provide tissue that can be properly analyzed, may be used to biopsy by thoracoscopy or open thoracic cavity. The diagnosis of sarcoidosis is established if the biopsy is established and no caseous granulomatous nature of the nodule. Active disease may also have areas of fibrinoid necrosis. The diagnosis is supported by the exclusion of infectious diseases that could be responsible for the symptoms.

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