Presentation
most commonly, it presents as cranial neuropathy, but presentation ranges from encephalopathy, meningitis, hydrocephalus, and seizure to spinal cord dysfunction, peripheral neuropathy, and myopathy. 2, 3, 4, 10 Only rare cases of neurosarcoidosis presenting [ajnr.org]
Here, we present a case of isolated neurosarcoidosis that initially presented with hydrocephalus requiring ventriculoperitoneal shunt placement. [nnjournal.net]
Splenomegaly is present in about 17%. In approximately12%, osseous granulomas are present, most commonly in the phalanges of the fingers and toes. Involved phalanges appear swollen and deformed, often sausage-shaped. [dermaamin.com]
Case Report Mrs BM, a 52 year old lady presented in March 2008 to the ENT department at Royal Glamorgan Hospital, South Wales. [ispub.com]
About a third will have a nonspecific presentation with fever, fatigue, cachexia and lassitude and this presentation is more common in people of black or Indian origin. [patient.info]
Entire Body System
- Lymphadenopathy
Imaging: Chest radiograph (bihilar lymphadenopathy, infiltrates), high resolution computed tomography of the chest (intrathoracic lymphadenopathy, fibrosis), MRI plus gadolinium of heart or brain (for cardiac and neurological sarcoidosis), gallium-67 [ptpaces.wordpress.com]
[…] pulmonary infiltrates without lymphadenopathy. [rheumaknowledgy.com]
The lymphadenopathy specimens were biopsied via mediastinoscopy, and histological study revealed noncaseating epithelioid granulomatous inflammation consistent with sarcoidosis. [ncbi.nlm.nih.gov]
Skin lesions – more likely to have lymphadenopathy & hepatosplenomegaly than if no skin lesions. May portend prognosis. [slideshare.net]
A subject with type 1 diabetes complicated by peripheral and autonomic neuropathy presented cough and cervical lymphadenopathy. The chest computed tomography (CT) scan revealed bilateral hilar lymphadenopathy. [care.diabetesjournals.org]
- Weakness
Upon arrival, her neurological exam demonstrated direction-changing nystagmus, asymmetric proximal lower extremity weakness (iliopsoas right 3/5, left 2/5), and ataxia on finger-nose-finger and heel-shin testing. [nnjournal.net]
I first noticed this in my hands which became very weak and clumsy. I dropped and broke many things. Eventually my fingers became too weak to even operate an ATM key pad. [bacteriality.com]
On neurologic examination, the patient had dysarthria, mild aphasia, and right upper and lower extremity weakness without cranial neuropathy. [ajnr.org]
- Anorexia
Fatigue, malaise, weakness, anorexia, weight loss, and low-grade fever are also common. Sarcoidosis can manifest as fever of unknown origin. [merckmanuals.com]
Skin
- Skin Lesion
The skin involvement in juvenile idiopathic arthritis is a transient erythematous macular rash whereas sarcoidosis can present with varied skin eruptions including erythema, papules, plaques and ichthyosiform lesions. [3] As skin lesions may appear before [ijdvl.com]
Skin lesions – more likely to have lymphadenopathy & hepatosplenomegaly than if no skin lesions. May portend prognosis. [slideshare.net]
The most useful biopsy procedures are a skin punch biopsy of specific skin lesions, in the event that they are present [ 40, 48 ]. [em-consulte.com]
Uncommon but specific skin lesions of sarcoidosis include subcutaneous nodules, ulcerations, dystrophic nails, scarring alopecia, verrucous lesions, pustular lesions, and rarely erythroderma. [bobbybukamd.com]
If skin lesions are present, they are an obvious choice for biopsy. In the absence of skin lesions, a Kveim test was frequently used in the past. [dermaamin.com]
Eyes
- Lacrimation
Heerfordt-Waldenstrom Syndrome Fever, parotid enlargement, anterior uveitis, facial nerve palsy (can get lethargy, papilledema, meningism) Mikulicz’s Syndrome Bilateral sarcoidosis of the parotid, submandibular, sublingual and lacrimal glands. 44. [slideshare.net]
Ocular: Anterior uveitis, posterior uveitis, optic neuropathy, papilloedema, lacrimal gland enlargement. Musculoskeletal: Hot, swollen and/or tender joints, myopathy. [ptpaces.wordpress.com]
Sarcoid involvement of the exocrine glands of the head (e.g., parotid, lacrimal) may resemble the findings of Sjögren’s syndrome. [rheumaknowledgy.com]
Symmetric increased uptake in mediastinal and hilar nodes (lambda sign) and in lacrimal, parotid, and salivary glands (panda sign) are patterns highly suggestive of sarcoidosis. A negative result in patients taking prednisone is unreliable. [merckmanuals.com]
Figure 3: 67Ga citrate PET scan revealing increase bilateral radionuclide uptake in the lacrimal and parotid glands and normal nasopharyngeal uptake showing the Panda sign (red arrows). [ijem.in]
- Excessive Tearing
For self-monitoring they are taught symptoms to report such as shortness of breath, excessive tearing and inflamed eyes, chest pain, swollen joints, and an increased sense of being tired and listless. [medical-dictionary.thefreedictionary.com]
Neurologic
- Dysarthria
This previously healthy 27-year-old man was admitted due to several daily episodes of usually left hemiparesis and dysarthria lasting between 15 seconds and 3 minutes. He did not respond to aggressive antithrombotic treatment. [ncbi.nlm.nih.gov]
On neurologic examination, the patient had dysarthria, mild aphasia, and right upper and lower extremity weakness without cranial neuropathy. [ajnr.org]
- Tingling
It is relatively safe but nearly all patients experience the side effect of paresthesias, or numbness and tingling in the extremities. Patients also report an alteration in taste such that carbonated soft drinks taste metallic. [eyerounds.org]
- Paresis
In about 5%, one encounters paresis of a cranial nerve, most commonly of the facial nerve. Oral mucosal involvement occurs very rarely. [dermaamin.com]
Workup
This case highlights the importance of tissue diagnosis and the diagnostic workup for sarcoidosis in cases of cryptogenic hydrocephalus. [nnjournal.net]
She was diagnosed with idiopathic intracranial hypertension and referred to a neurologist for further workup and management. The neurologist performed a lumbar puncture which showed an elevated opening pressure of 50 cm H 2 O. [eyerounds.org]
Definitive diagnosis of neurosarcoidosis requires the exclusion of other causes of neuropathy and the identification of noncaseating sarcoid granulomas by histologic analysis of nerve and muscle biopsy specimens (see Workup). [emedicine.medscape.com]
"Sarcoidosis Workup". Medscape Reference. WebMD. Archived from the original on 1 March 2014. Retrieved 19 February 2014. ^ Allmendinger A, Perone R (2009). "Case of the Month". Diagnostic Imaging. 31 (9): 10. ^ Joanne Mambretti (2004). [en.wikipedia.org]
Foreign bodies in granulomatous cutaneous lesions and sarcoidosis are not mutually exclusive and, therefore, a workup to exclude systemic sarcoidosis should be undertaken [ 20, 25, 26, 27, 28, 29, 30, 31, 32 ]. [em-consulte.com]
Other Pathologies
- Granulomatous Tissue
Patient was subjected to FNAC thyroid which revealed ill-formed elements of granulomatous tissue. Patient refused to undergo the biopsy of the thyroid gland. [ijem.in]
Granulomatous production of parathyroid-hormone related protein (PTH-rP) may also play a role in abnormal calcium metabolism, 4 where tissue necrosis factor-alpha (TNF-α) and interleukin-6, produced by macrophages, increase PTH-rP gene expression. 4 PTH-rP [academic.oup.com]
Treatment
[…] in the 4-mg treatment group showed a significant increase compared with placebo ( P =.012). [clinicalpainadvisor.com]
At this time, there is no consistently effective treatment for necrobiosis lipoidica. Treatment options include corticosteroids, fibrinolytics, antiplatelet agents, and surgical treatments (i.e. excision and grafting). [bobbybukamd.com]
A variety of treatments for IIH have been proposed and there is much anecdotal evidence for these treatments. [eyerounds.org]
[…] as a treatment for cases with liver involvement. [113] Thalidomide has also been tried successfully as a treatment for treatment-resistant lupus pernio in a clinical trial, which may stem from its anti-TNF activity, although it failed to exhibit any [en.wikipedia.org]
The response to treatment often takes at least four weeks, with maximum treatment responses being observed at 12 weeks [ 142 ]. Relapse after discontinuation of treatment is common [ 141 ]. [em-consulte.com]
Prognosis
Seite 117 - Pulmonary sarcoidosis: role of CT in the evaluation of disease activity and functional impairment and in prognosis assessment. Radiology 191 (3): 675-680 141. [books.google.de]
May portend prognosis. Classified as: Specific (lesions contain granulomas) Non-specific (lesions are reactive processes and do not contain granulomas). 18. [slideshare.net]
They may be interpreted as a defense reaction against a tumor’s antigens, and may have a more favorable prognosis than classical malignant nodes. [omicsonline.org]
Early sarcoid arthritis is often self-limited, lasting for days to months, and the prognosis is excellent. [rheumaknowledgy.com]
Prognosis The prognosis of patients with sarcoidosis mainly depends on the extent and severity of the systemic involvement [ 15, 17, 18, 20 ]. [em-consulte.com]
Etiology
A discussion of the etiology, diagnosis, systemic and ocular manifestations, and treatment of sarcoidosis is presented. Format: video Series: Moran Eye Center Grand Rounds Identifier: Moran_CORE_21552 Copyright statement: Copyright 2016. [morancore.utah.edu]
Etiology: Although exposure to an infectious organism or some other environmental agent has long been hypothesized to be etiologically relevant to the disease, none has been conclusively implicated. [rheumaknowledgy.com]
Etiology and Clinical Diagnosis The etiologies of microscopic hematuria are numerous and range from clinically insignificant causes to potentially life-threatening neoplastic lesions 5 ( Table 1 2, 3, 7 – 9 ). [aafp.org]
Etiology Despite multiple investigations, the cause of sarcoidosis remains unknown. Currently, the most likely etiology is an infectious or noninfectious environmental agent that triggers an inflammatory response in a genetically susceptible host. [what-when-how.com]
Keywords Sarcoidosis; Schwannoma; Tumor; Benign Introduction Sarcoidosis is a multisystemic granulomatous disorder characterized by non caseating granulomas, of undetermined etiology, with bilateral mediastinal lymphadenopathy, pulmonary infiltration, [omicsonline.org]
Epidemiology
Gastroenterology, 127, S5-S16. [ 2 ] El-Serag, H.B. (2002) Hepatocellular carcinoma: An epidemiologic view. [scirp.org]
Epidemiology ► Exact prevalence (estimated at 10 to 20 per 100,000 population). ► Annual incidence of sarcoidosis are not known with certainty ► Incidence peaks in winter and early spring. Women greater incidence. [slideshare.net]
Epidemiology [ edit ] The prevalence of sarcoid is estimated to be 10 to 20 per 100,000 people. [en.wikibooks.org]
James DG, Hosoda Y: Epidemiology. In: James DG, ed. Sarcoidosis and other granulomatous disorders. New York, Marcel Dekker; 1994 :729–43 2. Stern BJ: Neurological complications of sarcoidosis. Curr Opin Neurol; 2004:17:311-6 3. [ispub.com]
"The epidemiology of sarcoidosis in Rochester, Minnesota: a population-based study of incidence and survival" (PDF). American Journal of Epidemiology. 123 (5): 840–5. doi : 10.1093/oxfordjournals.aje.a114313. [en.wikipedia.org]
Pathophysiology
Posterior reversible encephalopathy syndrome, part 2: Controversies surrounding pathophysiology of vasogenic edema. Am J Neuroradiol 2008;29:1043-9. [ PUBMED ] [ FULLTEXT ] 7. Saigal G, Bhatia R, Bhatia S, Wakhloo AK. [annalsofian.org]
ARA 290 relieves pathophysiological pain by targeting TRPV1 channel: Integration between immune system and nociception. Peptides. 2016;76:73-79. doi: 10.1016/j.peptides.2016.01.003 Heij L, Niesters M, Swartjes M, et al. [clinicalpainadvisor.com]
Pathophysiology [ edit ] The diagnosis of sarcoid is based on compatible clinical and radiographic findings, supportive laboratory data (elevated ACE levels, anergy, elevated serum gamma globulins, positive Kveim test), and evidence of noncaseating granulomas [en.wikibooks.org]
Pathophysiology and Immunopathogenesis The granuloma is the pathologic hallmark of sarcoidosis. A distinct feature of sarcoidosis is the local accumulation of inflammatory cells. [what-when-how.com]
Pathophysiology In IIH, there is no structural obstruction to the circulation of CSF. [eyerounds.org]
Prevention
Reducing Risk & Slowing Down the Disease Sarcoidosis can’t currently be cured, but it can be managed to prevent other problems, such as heart failure, from developing over time. [heart.templehealth.org]
Major goal : prevent fibrosis. Steroids first line : Help prevent fibrosis. Scarring cutaneous lesions Hypercalcemia Severe lung disease Liver disease Cardiac inflammation Posterior uveitis Neurosarcoidosis Severe sarcoidosis of other organs 46. [slideshare.net]
Prevention Although sarcoidosis cannot be prevented, some preventative measures may reduce complications. For example: Influenza vaccination. Osteoporosis prophylaxis (steroid usage). [patient.info]
Although primary prevention studies suggest that cholecalciferol is not entirely effective in preventing glucocorticoid-induced bone loss, 16 it is well tolerated by patients, and can be used once hypercalcaemia has been excluded. [academic.oup.com]
Prevention Prevention Because the cause of transient global amnesia is unknown and the rate of recurrence is low, no standard approaches for preventing the condition exist. [go2jo.com]