A 22-year-old female presented with fever, cough for 6 months, chest X-ray shows mediastinal widening and blood tests revealed an Increased level of ACE levels. What is the most probable diagnosis:
Correct Answer: Sarcoidosis
Description: Ans: A (Sarcoidosis) Ref Harrison's Principles of Internal Medicine 18th edition & Nelson Textbook of Pediatrics. 19th ed. Explanation: Raised ACE levels, points towards Sarcoidosis. The given history is also compatible with pulmonary tuberculosis, but serum ACE levels will be normal. Sarcoidosis It is a multisystem granulomatous disease and lesions may occur anywhere in the body. The clinical manifestations depend on the extent and degree of granulomatous inflammation and are extremely variable. Children may present with nonspecific symptoms, such as fever, weight loss, and general malaise. In adults and older children, pulmonary involvement is most frequent, with infiltration of the thoracic lymph nodes and lung parenchyma. Isolated bilateral hilar adenopathy on chest radiograph is the most common finding, but parenchymal infiltrates and miliary nodules may also be seen Patients with lung involvement are commonly found to have restrictive changes on pulmonary function testing. Extrathoracic lymphadenopathy and infiltration of the liver, spleen, and bone marrow leading on to isolated hepatomegaly and splenomegaly Cutaneous disease, such as plaques, nodules, erythema nodosum in acute disease, or lupus pernio in chronic sarcoidosis Red-brown to purple maculopapular lesions <1 cm on the face, neck, upper back, and extremities are the most common skin finding Ocular involvement is frequent, anterior or posterior uveitis, conjunctival granulomas, eyelid inflammation, and orbital or lacrimal gland infiltration. The arthritis in sarcoidosis can be confused with JR A Central nervous system (CNS) involvement is rare in childhood hut may manifest as seizures, cranial nerve involvement, intracranial mass lesions, and hypothalamic dysfunction. Kidney disease also occurs infrequently in 'children but typically manifests as renal insufficiency, proteinuria, transient pyuria, or microscopic hematuria as a result of either early monocellular infiltration or granuloma formation in kidney tissue. Only a small fraction of children have hypercalcemia or hypercalciuria, which is, therefore, an infrequent cause of kidney disease. Sarcoid granulomas can also infiltrate the heart and lead to cardiac arrhythmias and sudden death. In contrast to the variable clinical presentation of sarcoidosis in older children, early-onset sarcoidosis classically manifests as the triad uveitis, arthritis, and rash. Pulmonary disease and lymphadenopathy are less common. The arthritis is polyarticular and symmetric, with large boggy effusions. The rash is diffuse, erythematous, papular Noncaseating granulomas are demonstrated with biopsy of the skin or joint synovium Laboratory Findings There is no single laboratory test diagnostic of sarcoidosis. Anemia, leukopenia, and eosinophilia may be seen. Hypergammaglobulinemia and elevations in acute-phase reactants (ESR & CRP) Hypercalcemia and/or hypercalciuria Angiotensin-converting enzyme LACE) is produced by the epithelioid cells of the granuloma, and its serum value may be elevated. but this finding lacks diagnostic sensitivity and specificity. Fluorodeoxyglucose FI8 positron emission tomography (18FDG PET) can help identify non-pulmonary sites for a diagnostic biopsy. A positive gallium scan can support the diagnosis if an increased activity is noted in the parotids and lacrimal glands (panda sign) or in the right paratracheal and left hilar area (lambda sign).
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