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| SLE resident survival guide:
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| <div style="font-size: 90%;">
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| {{Familytree/start}}
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| {{Familytree|boxstyle=width: 700px; text-align: left; font-size: 100%; padding: 0px;| A01 | | |A01=<div style="padding: 10px;">
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| ==SLE Presentation==
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| *[[Fatigue]]<ref name="pmid11085805">{{cite journal |vauthors=Tench CM, McCurdie I, White PD, D'Cruz DP |title=The prevalence and associations of fatigue in systemic lupus erythematosus |journal=Rheumatology (Oxford) |volume=39 |issue=11 |pages=1249–54 |year=2000 |pmid=11085805 |doi= |url=}}</ref><ref name="pmid7779127">{{cite journal |vauthors=McKinley PS, Ouellette SC, Winkel GH |title=The contributions of disease activity, sleep patterns, and depression to fatigue in systemic lupus erythematosus. A proposed model |journal=Arthritis Rheum. |volume=38 |issue=6 |pages=826–34 |year=1995 |pmid=7779127 |doi= |url=}}</ref><ref name="pmid9598886">{{cite journal |vauthors=Wang B, Gladman DD, Urowitz MB |title=Fatigue in lupus is not correlated with disease activity |journal=J. Rheumatol. |volume=25 |issue=5 |pages=892–5 |year=1998 |pmid=9598886 |doi= |url=}}</ref>
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| *[[Fever]]
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| *[[Myalgia]]
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| *Joint [[tenderness]]
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| *[[Muscle weakness]]
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| *[[Weight]] changes
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| ==Less common Presentation==
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| *Dysphagia
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| *Peptic ulcer disease
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| *Intestinal pseudo-obstruction
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| *Protein-losing enteropathy
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| *Acute pancreatitis
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| *Pneumonitis
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| *Pleuritis
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| *Pulmonary hemorrhage
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| *Interstitial lung disease
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| *Pulmonary emboli
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| *Pulmonary hypertension
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| *Pericarditis
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| *Myocarditis
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| *Seizures
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| *Stroke
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| *Psychosis
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| *Nephrotic syndrome
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| </div>}}
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| ==Focused History==
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| * Onset, duration and progression of symptoms
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| * History of [[Weight change|weight changes]] (gain or loss)
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| * [[Anorexia]]
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| * [[Arthritis]], [[Arthralgia|arthralgias]], or [[muscle pain]]
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| * Morning [[stiffness]]
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| * [[Skin rashes]] and their association to flare ups, especially after exposure to sunlight
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| * [[Medications]] and their association to flare ups
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| * [[Infections]] especially [[mononucleosis]]
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| * Sores in the mouth, nose, or other [[Mucous membrane|mucous membranes]]
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| * Symptoms of other organ failure
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| ** [[Renal failure]]: Recent peripheral [[edema]] and [[weight gain]]
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| ** [[Cardiac]] involvement: [[Tachycardia]], [[dyspnea]], [[Chest pain|chest pains]]
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| * History of having a pet
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| * [[Hair loss]]
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| * Job history
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| * [[Comorbid|Co-morbid]] conditions include:
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| ** Other [[Rheumatologic disease|rheumatologic]] and [[autoimmune diseases]]
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| ** [[Hypertension]]
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| ** [[Diabetes]], [[immunodeficiency]]
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| * [[Seizure|Seizures]], or other [[nervous system]] symptoms
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| * [[Family history]] of [[Rheumatologic disease|rheumatologic diseases]]
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| </div>}}
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| {{Familytree|boxstyle=width: 700px; text-align: left; font-size: 100%; padding: 0px;| |!| | | |}}
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| ==Physical Examination==
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|
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| ===Appearance of the Patient===
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| *Patient appears well in the earlier stages of the disease
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| *Patient appears ill in the late stages of the disease due to multi-organ involvement
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| ===Vital Signs===
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| *[[Fever]] higher than 38 after the exclusion of [[infection]]
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| *[[Tachycardia]]/[[bradycardia]] depends on the accompanying complication
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| *[[Tachypnea]]/[[bradypnea]] depends on the accompanying complication
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|
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| === Skin<ref name="pmid25077888">{{cite journal |vauthors=Parodi A, Cozzani E |title=Cutaneous manifestations of lupus erythematosus |journal=G Ital Dermatol Venereol |volume=149 |issue=5 |pages=549–54 |year=2014 |pmid=25077888 |doi= |url=}}</ref><ref name="pmid26985173">{{cite journal |vauthors=Szczęch J, Rutka M, Samotij D, Zalewska A, Reich A |title=Clinical characteristics of cutaneous lupus erythematosus |journal=Postepy Dermatol Alergol |volume=33 |issue=1 |pages=13–7 |year=2016 |pmid=26985173 |pmc=4793050 |doi=10.5114/pdia.2014.44031 |url=}}</ref><ref name="pmid19824738">{{cite journal |vauthors=Walling HW, Sontheimer RD |title=Cutaneous lupus erythematosus: issues in diagnosis and treatment |journal=Am J Clin Dermatol |volume=10 |issue=6 |pages=365–81 |year=2009 |pmid=19824738 |doi=10.2165/11310780-000000000-00000 |url=}}</ref> ===
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| *[[Cyanosis]]
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| **Secondary to [[respiratory]] complications
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| *[[Jaundice]]
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| **Secondary to [[hepatitis]]
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| *[[Pallor]]
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| **Secondary to [[anemia]]
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| *Generalised [[erythema]]
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| *[[Bruises]]
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| **Secondary to [[thrombocytopenia]]
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| *[[Urticaria]]
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| *[[Bullous|Bullous lesions]]
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| *[[Sclerodactyly]]
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| *Skin [[Ulcerations|ulceration]]
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| *[[Malar rash]]: the classic lupus ‘butterfly’ rash
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| ** [[Erythema|Erythematous]], elevated [[Lesions|lesion]], [[Pruritis|pruritic]] or painful, in a [[malar]] distribution, commonly precipitated by exposure to sunlight
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| * Annular or psoriasiform skin [[lesions]]
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| ** Small, [[erythematous]], slightly scaly [[papules]] that evolve into either a psoriasiform (papulosquamous) or annular form and tend to involve shoulders, forearms, neck, and upper torso
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| * [[Scarring]] [[chronic]] [[alopecia]]
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| ** Mostly present on face, neck, and scalp
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| * [[Telangiectasias]]
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| * Dyspigmentation ([[hyperpigmentation]] or [[hypopigmentation]]) of skin in [[scar]] places
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| * Follicular plugging
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| ** Discrete, [[erythematous]], slightly infiltrated [[plaques]] covered by a well-formed adherent scale that extends into dilated [[hair follicles]]
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| * [[Nodules]]
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| ** Can be firm (Lupus profundus) and painful
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| * Photodistributed [[lesions]] with chronic pink indurated plaques or broad [[lesions]] that are slow to heal (more seen in lupus tumidus)
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| * Thin hair that easily fractures (lupus hair)
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| * Irregularly shaped raised white plaques, areas of erythema, silvery white scarred [[lesions]], and [[ulcers]] with surrounding [[erythema]] on the soft or hard palate or buccal [[mucosa]]
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| * Periungual [[erythema]]
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| ** Dilated tortuous loops of [[capillaries]] and a prominent subcapillary venous plexus along
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| ** Involves the base of the nail and edges of the upper [[eyelid]]
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| [[File:Systemic lupus erythematosus 055.jpg|300px]]
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| '''For more pictures of the rash presentation in lupus, click [[Systemic lupus eryhthematosus physical examination:Gallery|here]].'''
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|
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| ===HEENT===
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| *[[Ophthalmology]]<ref name="pmid26367085">{{cite journal |vauthors=Preble JM, Silpa-archa S, Foster CS |title=Ocular involvement in systemic lupus erythematosus |journal=Curr Opin Ophthalmol |volume=26 |issue=6 |pages=540–5 |year=2015 |pmid=26367085 |doi=10.1097/ICU.0000000000000209 |url=}}</ref><ref name="pmid25904124">{{cite journal |vauthors=Silpa-archa S, Lee JJ, Foster CS |title=Ocular manifestations in systemic lupus erythematosus |journal=Br J Ophthalmol |volume=100 |issue=1 |pages=135–41 |year=2016 |pmid=25904124 |doi=10.1136/bjophthalmol-2015-306629 |url=}}</ref>
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| **[[Nystagmus]]
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| **Visual [[Retinal|retinal changes]] from systemic lupus erythematosus cytoid bodies, [[Retina|retinal]] [[haemorrhages]], serous exudate or [[haemorrhage]] in the [[choroid]], [[optic neuritis]] (not due to [[hypertension]], drugs or [[infection]])
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| **[[Cataract]]
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| **[[Optic nerve atrophy]]
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| **[[Icterus|Icteric sclera]]
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| **[[Cotton wool spots]] in the [[retina]] in [[Ophthalmoscopy|ophthalmoscopic exam]]
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| *Nasal [[ulcers]]<ref name="pmid1526055">{{cite journal |vauthors=Robson AK, Burge SM, Millard PR |title=Nasal mucosal involvement in lupus erythematosus |journal=Clin Otolaryngol Allied Sci |volume=17 |issue=4 |pages=341–3 |year=1992 |pmid=1526055 |doi= |url=}}</ref>
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| **Mostly painless
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| **Mostly in lower [[nasal septum]] and [[bilateral]] and parallel
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| **Nasal perforation secondary to [[vasculitis]] may happen rarely
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| *Oral [[ulcers]]<ref name="pmid23780804">{{cite journal |vauthors=Anyanwu CO, Ang CC, Werth VP |title=Oral mucosal involvement in bullous lupus |journal=Arthritis Rheum. |volume=65 |issue=10 |pages=2622 |year=2013 |pmid=23780804 |pmc=4333153 |doi=10.1002/art.38051 |url=}}</ref><ref name="pmid23248469">{{cite journal |vauthors=Ranginwala AM, Chalishazar MM, Panja P, Buddhdev KP, Kale HM |title=Oral discoid lupus erythematosus: A study of twenty-one cases |journal=J Oral Maxillofac Pathol |volume=16 |issue=3 |pages=368–73 |year=2012 |pmid=23248469 |pmc=3519212 |doi=10.4103/0973-029X.102487 |url=}}</ref>
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| **Mostly painless
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| **White plaques with areas of [[erythema]], or punched-out erosions or ulcers with surrounding [[erythema]] on the soft or hard palate or [[buccal mucosa]]
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|
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| ===Neck<ref name="pmid19107085">{{cite journal |vauthors=Melikoglu MA, Melikoglu M |title=The clinical importance of lymphadenopathy in systemic lupus erythematosus |journal=Acta Reumatol Port |volume=33 |issue=4 |pages=402–6 |year=2008 |pmid=19107085 |doi= |url=}}</ref><ref name="pmid24722263">{{cite journal |vauthors=Sacre K, Escoubet B, Pasquet B, Chauveheid MP, Zennaro MC, Tubach F, Papo T |title=Increased arterial stiffness in systemic lupus erythematosus (SLE) patients at low risk for cardiovascular disease: a cross-sectional controlled study |journal=PLoS ONE |volume=9 |issue=4 |pages=e94511 |year=2014 |pmid=24722263 |pmc=3983200 |doi=10.1371/journal.pone.0094511 |url=}}</ref>===
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| *[[Jugular venous distension]]
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| **Secondary to [[hypertension]] and cardiac complications
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| *[[Lymphadenopathy]]
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| ** [[Lymph node|Lymph nodes]] are soft, non-tender, discrete
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| ** Usually detected in the [[cervical]], [[axillary]], and [[inguinal]]
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|
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| ===Lungs<ref name="pmid21194884">{{cite journal |vauthors=Torre O, Harari S |title=Pleural and pulmonary involvement in systemic lupus erythematosus |journal=Presse Med |volume=40 |issue=1 Pt 2 |pages=e19–29 |year=2011 |pmid=21194884 |doi=10.1016/j.lpm.2010.11.004 |url=}}</ref><ref name="pmid26550810">{{cite journal |vauthors=Salvati F |title=[The involvement of pulmonary interstitial tissue in multisystemic lupus erythematosus: interdisciplinarity and role of the pneumologists] |language=Italian |journal=Clin Ter |volume=166 |issue=5 |pages=205–7 |year=2015 |pmid=26550810 |doi= |url=}}</ref><ref name="pmid25639532">{{cite journal |vauthors=Alamoudi OS, Attar SM |title=Pulmonary manifestations in systemic lupus erythematosus: association with disease activity |journal=Respirology |volume=20 |issue=3 |pages=474–80 |year=2015 |pmid=25639532 |pmc=4418345 |doi=10.1111/resp.12473 |url=}}</ref>===
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| *Fine and coarse [[crackles]] upon auscultation of the [[lung]]
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| **May be due to [[pneumonitis]]
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| *In case of [[pleural effusion]]:
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| **Asymmetric [[tactile fremitus]]
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| **Asymmetric [[chest expansion]]
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|
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| ===Heart<ref name="pmid24790989">{{cite journal |vauthors=Mak A, Kow NY |title=Imbalance between endothelial damage and repair: a gateway to cardiovascular disease in systemic lupus erythematosus |journal=Biomed Res Int |volume=2014 |issue= |pages=178721 |year=2014 |pmid=24790989 |pmc=3984775 |doi=10.1155/2014/178721 |url=}}</ref><ref name="pmid24722263">{{cite journal |vauthors=Sacre K, Escoubet B, Pasquet B, Chauveheid MP, Zennaro MC, Tubach F, Papo T |title=Increased arterial stiffness in systemic lupus erythematosus (SLE) patients at low risk for cardiovascular disease: a cross-sectional controlled study |journal=PLoS ONE |volume=9 |issue=4 |pages=e94511 |year=2014 |pmid=24722263 |pmc=3983200 |doi=10.1371/journal.pone.0094511 |url=}}</ref><ref name="pmid23052654">{{cite journal |vauthors=Canpolat N, Kasapcopur O, Caliskan S, Gokalp S, Bor M, Tasdemir M, Sever L, Arisoy N |title=Ambulatory blood pressure and subclinical cardiovascular disease in patients with juvenile-onset systemic lupus erythematosus |journal=Pediatr. Nephrol. |volume=28 |issue=2 |pages=305–13 |year=2013 |pmid=23052654 |doi=10.1007/s00467-012-2317-3 |url=}}</ref>===
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| *Chest [[tenderness]] or discomfort upon palpation
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| **May be due to [[costochondritis]]
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| *[[Diastolic murmurs|Diastolic murmur]], or [[Systolic murmurs|systolic murmur]] >3/6 due to [[valvular disease]]
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| *Loud second heart sound (due to [[pulmonary hypertension]])
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| *Loud S1-S2 due to [[cardiomegaly]]
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| *Decrease heart sounds if associated with [[pericardial effusion]]
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|
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| ===Abdomen<ref name="pmid20572299">{{cite journal |vauthors=Tian XP, Zhang X |title=Gastrointestinal involvement in systemic lupus erythematosus: insight into pathogenesis, diagnosis and treatment |journal=World J. Gastroenterol. |volume=16 |issue=24 |pages=2971–7 |year=2010 |pmid=20572299 |pmc=2890936 |doi= |url=}}</ref><ref name="pmid27329649">{{cite journal |vauthors=Alves SC, Fasano S, Isenberg DA |title=Autoimmune gastrointestinal complications in patients with systemic lupus erythematosus: case series and literature review |journal=Lupus |volume=25 |issue=14 |pages=1509–1519 |year=2016 |pmid=27329649 |doi=10.1177/0961203316655210 |url=}}</ref><ref name="pmid27055518">{{cite journal |vauthors=Fawzy M, Edrees A, Okasha H, El Ashmaui A, Ragab G |title=Gastrointestinal manifestations in systemic lupus erythematosus |journal=Lupus |volume=25 |issue=13 |pages=1456–1462 |year=2016 |pmid=27055518 |doi=10.1177/0961203316642308 |url=}}</ref><ref name="pmid28523968">{{cite journal |vauthors=Li Z, Xu D, Wang Z, Wang Y, Zhang S, Li M, Zeng X |title=Gastrointestinal system involvement in systemic lupus erythematosus |journal=Lupus |volume= |issue= |pages=961203317707825 |year=2017 |pmid=28523968 |doi=10.1177/0961203317707825 |url=}}</ref>===
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| *[[Abdominal distention]]
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| *[[Ascites]]
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| **Due to [[nephrotic syndrome]]
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| *[[Abdominal pain]]
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| **Due to [[Vasculitis|mesentric vasculitis]]
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| *[[Hepatomegaly]]
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| *[[Splenomegaly]]
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| *Costo-vertebral angle tenderness
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|
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| ===Extremities<ref name="pmid15580980">{{cite journal |vauthors=Zoma A |title=Musculoskeletal involvement in systemic lupus erythematosus |journal=Lupus |volume=13 |issue=11 |pages=851–3 |year=2004 |pmid=15580980 |doi=10.1191/0961203303lu2021oa |url=}}</ref><ref name="pmid22956550">{{cite journal |vauthors=Gabba A, Piga M, Vacca A, Porru G, Garau P, Cauli A, Mathieu A |title=Joint and tendon involvement in systemic lupus erythematosus: an ultrasound study of hands and wrists in 108 patients |journal=Rheumatology (Oxford) |volume=51 |issue=12 |pages=2278–85 |year=2012 |pmid=22956550 |doi=10.1093/rheumatology/kes226 |url=}}</ref><ref name="pmid19591780">{{cite journal |vauthors=Grossman JM |title=Lupus arthritis |journal=Best Pract Res Clin Rheumatol |volume=23 |issue=4 |pages=495–506 |year=2009 |pmid=19591780 |doi=10.1016/j.berh.2009.04.003 |url=}}</ref><ref name="pmid24862229">{{cite journal |vauthors=Zhu KK, Xu WD, Pan HF, Zhang M, Ni J, Ge FY, Ye DQ |title=The risk factors of avascular necrosis in patients with systemic lupus erythematosus: a meta-analysis |journal=Inflammation |volume=37 |issue=5 |pages=1852–64 |year=2014 |pmid=24862229 |doi=10.1007/s10753-014-9917-y |url=}}</ref><ref name="pmid23731640">{{cite journal |vauthors=Voulgari PV, Kosta P, Argyropoulou MI, Drosos AA |title=Avascular necrosis in a patient with systemic lupus erythematosus |journal=Joint Bone Spine |volume=80 |issue=6 |pages=665 |year=2013 |pmid=23731640 |doi=10.1016/j.jbspin.2013.03.018 |url=}}</ref>===
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| *[[Clubbing]]
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| *[[Cyanosis]]
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| *[[Muscle weakness|Muscle atrophy or weakness]]
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| *[[Livedo reticularis]]
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| **Reddish-cyanotic, reticular pattern on the skin of the arms, legs, and torso, particularly with cold exposure
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| *[[Arthritis]]
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| **Symmetrical
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| **Polyarticular
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| **Mostly involve knees, carpal joints, and joints of the fingers, especially the proximal [[interphalangeal]] (PIP) joint
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| **Decrease range of motion in affected joints
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| *Joints [[erythema]]
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| **Due to [[synovitis]]
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| *Joint effusion
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| *Muscle [[atrophy]]
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| *[[Fasciculations]] in the upper/lower extremity
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| *[[Claudication]]
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| *Loss of digit or limb
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|
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| ===Neuromuscular<ref name="pmid19366083">{{cite journal |vauthors=Cojocaru IM, Cojocaru M, Tănăsescu R, Burcin C, Atanasiu AN, Silosi I |title=Detection of autoantibodies to ribosome P in lupus patients with neurological involvement |journal=Rom J Intern Med |volume=46 |issue=3 |pages=239–42 |year=2008 |pmid=19366083 |doi= |url=}}</ref><ref name="pmid22594009">{{cite journal |vauthors=Madrane S, Ribi C |title=[Central neuropsychiatric involvement in systemic lupus erythematosus] |language=French |journal=Rev Med Suisse |volume=8 |issue=337 |pages=848–53 |year=2012 |pmid=22594009 |doi= |url=}}</ref><ref name="pmid7555923">{{cite journal |vauthors=Sivri A, Hasçelik Z, Celiker R, Başgöze O |title=Early detection of neurological involvement in systemic lupus erythematosus patients |journal=Electromyogr Clin Neurophysiol |volume=35 |issue=4 |pages=195–9 |year=1995 |pmid=7555923 |doi= |url=}}</ref><ref name="pmid19217587">{{cite journal |vauthors=Juncal Gallego L, Almuíña Simón C, Muíños Esparza LF, Díaz Soto R, Ramil Fraga C, Quiroga Ordóñez E |title=[Systemic lupus erythematosus with fulminant neurological involvement] |language=Spanish; Castilian |journal=An Pediatr (Barc) |volume=70 |issue=2 |pages=202–4 |year=2009 |pmid=19217587 |doi=10.1016/j.anpedi.2008.09.009 |url=}}</ref>===
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| *Patient is usually oriented to persons, place, and time based on the disease course
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| *[[Cognitive impairment]]
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| *[[Hallucination|Hallucinations]]
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| **[[Visual hallucinations|Visual]]
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| **[[Auditory hallucinations|Auditory]]
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| *Memory deficit
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| **Loose associations
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| **Impoverished thought content
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| **Illogical thinking
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| **Bizarre disorganised or catatonic behaviour
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|
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| </div>}}
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| ==Laboratory Workup==
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| * [[Complete blood count|CBC with DC]]
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| * [[Basic metabolic panel|SMA-7]]
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| * [[Creatine kinase]]
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| * [[Urinalysis]] with [[Urinalysis#Microscopic examination|microscopic examination]]
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| * [[Erythrocyte sedimentation rate]]
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| * [[C-reactive protein]]
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| * [[Coombs test]]
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| * [[Antinuclear antibodies]]
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| * [[Rheumatoid factor]]
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| * [[Cryoglobulins]]
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| * Antiphospholipid antibodies
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| * Complement levels
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| * Urine protein-to-creatinine ratio
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| * Anti-dsDNA antibody
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| * Anti-SM antibodies
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| * Anti-Ro/SSA antibodies
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| * Anti-La/SSB antibodies
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| * Anti-U1 RNP antibodies
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| * Antiribosomal P protein antibodies
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| </div>}}
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| ==Imaging Study==
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|
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| ===Chest Radiograph===
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| * Chest radiograph should be considered as a part of the initial diagnostic workup.
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|
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| ===Echocardiography===
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| * Echocardiography should be considered when suspecting [[endocarditis]].
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|
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| ===Abdominal Ultrasonography===
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| * Abdominal ultrasonography should be considered when suspecting hepatobiliary pathology.
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|
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| ===Chest CT Scan===
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| * Chest CT scan may detect nodular lesions (s/o [[malignancy]] or [[fungal]]/[[mycobacterial]]/[[nocardial]] [[infection]]) or [[mediastinal]] [[adenopathy]] (s/o [[lymphoma]], [[histoplasmosis]], or [[sarcoidosis]]).
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|
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| ===Abdominal CT Scan===
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| * Abdominal CT scan should be considered when suspecting [[intra-abdominal abscess]] or [[malignancy]].
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|
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| ===Positron Emission Tomography===
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| * PET may be useful in localizing the nidus of fever of unknown origin.
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| </div>}}
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| ==Other Investigation==
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|
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| ===Lymph Node Biopsy===
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| * Lymph node biopsy may be useful when suspecting [[lymphoma]], [[lymphogranuloma venereum]], [[toxoplasmosis]], and [[Kikuchi disease]].
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| * Granuloma may indicate disorders associated with granulomatous [[inflammation]] (eg, [[tuberculosis]], [[sarcoidosis]]) or [[lymphoma]].
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|
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| ===Bone Marrow Biopsy===
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| * Bone marrow biopsy may be considered when suspecting intracellular infectious pathogens or hematologic malignancies.
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|
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| ===Discontinuation of Nonessential Medications===
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| * Nonessential medications should be discontinued.
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| * Defervescence in less than 72 hours after discontinuing the culprit medication suggests [[drug fever]].
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| * Rechallenge with the offending agent usually results in recurrence of [[drug fever]].
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|
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| ===Trial of Empiric Antibiotics===
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| * Therapeutic trials of antimicrobial agents may be considered if other techniques fail to disclose the etiology.
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| * An infectious etiology is likely if abatement of fever occurs after the administration of empiric antibiotics.
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|
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| ===Naproxen Test===
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| * Naproxen test (375 mg twice daily) can be used to distinguish [[neoplastic]] [[fever]] from other etiologies.
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| * Naproxen test is considered positive when there is a rapid or sustained abatement of fever during the 3 days of the trial period.
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| * Defervescence within 12 hours occurs in almost all patients with [[neoplastic]] [[fever]].
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| * Fever recurs after discontinuation of naproxen in patients with [[neoplasms]].
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| * Naproxen demonstrated no antipyretic activity against fever in patients with occult infection.
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| </div>}}
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| {{Familytree/end}}
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| </div>
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