Extra-pulmonary manifestations of sarcoidosis

Lupus pernio (LP) is a violaceous or erythematous indurated papules, plaques, or nodules that’re mainly observed on the nose, cheeks, chin, & ears.

 

 

Extra-pulmonary manifestations of sarcoidosis (Sc)

Lupus pernio: Red-to-purple indurated plaques & nodules affecting the nose & cheeks.

Lupus pernio (LP) is a violaceous or erythematous indurated papules, plaques, or nodules that’re mainly observed on the nose, cheeks, chin, & ears. LP is associated with a higher risk for nasal involvement with Sc

1)    Sc can affect all systemic organs. The most commonly affected site of extra-pulmonary disease include the skin, eyes, reticuloendothelial system, musculoskeletal system, exocrine glands, heart, kidney, and CNS. Extra-pulmonary affection vary according to sex, age at presentation, & ethnicity.

2)    Cutaneous affection is seen in about 25 % of Sc ptns and usually an early finding.  Sc can cause anterior, intermediate, & posterior uveitis, in addition to retinal peri-phlebitis.

3)    Extraocular affection may involve the lacrimal gland, conjunctiva, & ocular muscles. Ptns with suspected Sc should commence ophthalmologic testing.  

4)    Sc can involve the larynx, pharynx, nares, and/or sinuses and should be suspected in all ptns with systemic Sc and upper respiratory tract Sms.  

5)    Cardiac Sc may be benign, incidentally observed or a life-threatening state leading to sudden death. The spectrum of Sc cardiac affection may include heart block & arrhythmias (due to involved conducting system), HF, valvular malfunction, & pericardial disease. Initial asessment should include an ECG.  

6)    Acute polyarthritis due to Sc typically induce symmetric affection of the ankle joint, but can affect other joints. A characteristic criterion is extension of the swelling to involve the soft tissue around the joint, leading to peri-arthritis rather than a true arthritis.

7)    Neurologic affection seen in about 5 % of ptns with Sc and could be the presenting feature. Clinical syndromes may include cranial mononeuropathy, neuroendocrine malfunction, a focal/multifocal encephalopathy, myelopathy, hydrocephalus, aseptic meningitis, peripheral neuropathy.

8)    Löfgren syndrome (LS) is a combination of erythema nodosum (EN) + hilar adenopathy + migratory poly-arthralgia + pyrexia. The presence of all features of LS has almost 95 % specificity to diagnose Sc. However, EN with no added features has a wide DD. Painless swelling affecting salivary & parotid glands seen in 5 % of ptns with Sc.

9)    Endocrine involvement of Sc include hypothalamic affection (basilar granulomatous meningitis) with infiltrated thyroid gland. Sc may also occasionally affect the uterus, ovaries, & testes.  

 

Alterations related to Ca⁺ metabolism are the most commonly seen renal & electrolyte changes observed among Sc ptns. Abnormal Ca⁺ metabolism can lead to nephrocalcinosis & nephrolithiasis. Other relatively common renal complications may include interstitial nephritis & MN. Clinically determined GIT lesions seen in < 1 % of ptns with Sc. Affected areas may include the esophagus, appendix, colon, rectum, liver, pancreas, & rarely small intestine. Routine monitoring of Sc may include evaluation for the types of extra-pulmonary Sc that can induce organ or life-threatening disease, despite the validity of this monitoring has not been totally formalized.  

 

RENAL & ELECTROLYTE ALTERATIONS OF SARCOIDOSIS (Sc)

 

Ptns with Sc having neither renal Sms nor established renal Sc, consensus guideline of ATS (American Thoracic Society) suggests basal SCr measuring to evaluate renal Sc. Ptns with Sc with no Sms or Sns of hypercalcemia, guidelines from the ATS recommend baseline s. Ca⁺ value for screening abnormal Ca⁺ metabolism. Disorders related to Ca⁺ metabolism are the most common renal & electrolyte alterations seen among Sc ptns. Defects in Ca⁺ metabolism is related to extrarenal production of calcitriol by activated macrophages. Manifestations of metabolic Ca⁺ alterations include higher intestinal Ca⁺ absorption, hypercalciuria (seen in 50 % of ptns), hypercalcemia (seen in 10-20 %), nephrocalcinosis + nephrolithiasis. Untreated, deposited kidney Ca⁺ >> CRF & ESRD.

 

Sc interstitial nephritis is more commonly observed on the initial presentation of Sc, rather than ptns with chronic disease. Dgx is suggested by the combination of high Cr level + bland urine sediment in a ptn with known or likely Sc. Kidney biopsy reveals normal glomeruli, mononuclear cell infiltration, & non-caseating granulomas. Despite the renal granulomatous infiltration is not commonly seen, it’s rarely a single cause of kidney dysfunction. Other sequelae of Sc include MN, a proliferative or crescentic GN, FGS, polyuria (owing to nephrogenic and/or central DI), HT, & some tubular deficits.