Systemic lupus erythematosus physical examination

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahshid Mir, M.D. [2]

Overview

In the earlier stages of the disease, the patients are more well-appearing while in the late stages of the disease, patients are ill with multi-organ involvement.

Physical Examination

Appearance of the Patient

  • In the earlier stages of the disease, the patients are more well-appearing.
  • In the late stages of the disease, patients are ill with multi-organ involvement.

Vital Signs

  • Fever More than 38o C aft er the exclusion of infection
  • Tachycardia/ Bradycardia depends on the accompanying complication
  • Tachypnea / bradypnea depends on the accompanying complication

Skin

[1][2][3]

  • Cyanosis
    • Secondary to repiratory complications
  • Jaundice
    • Secondary to hepatitis complications
  • Pallor
    • Secondary to anemia
  • Generalised erythema
  • Bruises
    • Secondary to thrombocytopenia
  • Urticaria
  • Bullous lesions
  • Sclerodactyly
  • Skin ulceration
  • Malar rash. The classic lupus ‘butterfly’ rash
    • Erythematous, elevated lesion, pruritic or painful, in a malar distribution, commonly precipitated by exposure to sunlight
  • Annular or psoriasiform skin lesions
    • 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
  • Scarring chronic alopecia
    • Mostly present on face, neck, and scalp
  • Telangiectasias
  • Dyspigmentation (hyper- or hypopigmentation) of skin in scar places
  • Follicular plugging
    • Discrete, erythematous, slightly infiltrated plaques covered by a well-formed adherent scale that extends into dilated hair follicles
  • Nodules
    • Can be firm (Lupus profundus) and often are painful
  • photodistributed lesions with chronic pink indurated plaques or broad lesions that are slow to heal (more seen in lupus tumidus)
  • Thin hair that easily fractures (lupus hair)
  • 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
  • Periungual erythema
    • Dilated tortuous loops of capillaries and a prominent subcapillary venous plexus along
    • Involves the base of the nail and edges of the upper eyelid

HEENT

  • Ophthalmology[4][5]
    • Nystagmus
    • Visual Retinal changes from systemic lupus erythematosus cytoid bodies, retinal haemorrhages, serous exudate or haemorrhage in the choroid, optic neuritis (not due to hypertension, drugs or infection)
    • Cataract
    • Optic nerve atrophy
  • Icteric sclera
  • Cotton wool spots in the retina in ophthalmoscopic exam
  • Nasal ulcers[6]
    • Mostly painless
    • Mostly in lower nasal septum and bilateral and parallel
    • Nasal perforation secondary to vasculitis may happen rarely
  • Oral ulcers[7][8]
    • Mostly painless
    • White plaques with areas of erythema, or punched-out erosions or ulcers with surrounding erythema on the soft or hard palate or buccal mucosa

Neck

  • Jugular venous distension[9]
    • Secondary to hypertension and cardiac complications
  • Lymphadenopathy [10]
    • Lymph nodes are soft, non-tender, discrete
    • Usually detected in the cervical, axillary, and inguinal

Lungs

  • Fine and coarse crackles upon auscultation of the lung[11][12]
  • In case of pleural effusion:[13][14]
    • Asymmetric tactile fremitus
    • Asymmetric chest expansion

Heart

  • Chest tenderness upon palpation
  • Diastolic murmur, or systolic murmur >3/6 due to valvular disease
  • Chest pain or discomfort secondary to costochondritis
  • Loud second heart sound (due to pulmonary hypertension)

Abdomen

Extremities

  • Clubbing
  • Cyanosis
  • Muscle atrophy or weakness
  • Livedo reticularis
    • Reddish-cyanotic, reticular pattern on the skin of the arms, legs, and torso, particularly with cold exposure
  • Arthritis
    • Symmetrical
    • Polyarticular
    • Mostly involve knees, carpal joints, and joints of the fingers, especially the proximal interphalangeal (PIP) joint
  • Decrease in affected joints range of motion
  • Joints erythema
    • Due to synovitis
  • Joint effusion
  • Muscle atrophy
  • Fasciculations in the upper/lower extremity
  • Claudication
  • Loss of digit or limb

Neuromuscular

  • Patient is usually oriented to persons, place, and time based on the disease course
  • Cognitive impairment
  • Hallucinations
  • Memory deficit
    • Loose associations
    • Impoverished thought content
    • Illogical thinking
    • Bizarre disorganised or catatonic behaviour
Constitutional symptoms Fatigue the most common complaint 11085805

multidimensional phenomenon due to different factors 7779127

does not correlate with disease activity 9598886

frequently associated with depression, sleep disturbances, and concomitant fibromyalgia
Fever a manifestation of active disease-lupus flare, especially within short-term evolution of the disease 14530779

Can be hard to differentiate the cause of fever from other inflammatory and infection diseases

Leukopenia or normal range of leukocytes in the setting of fever is more suggestive of lupus activity rather than infection

Myalgia

muscle tenderness

muscle weakness

may be first complaint and initial reason for the patient to seeks medical attention

May happen as a result of treatment with glucocorticoids or glucocorticoid withdrawal

perivascular and perifascicular mononuclear cell infiltrates in 25 percent of patients

muscle atrophy, microtubular inclusions, mononuclear infiltrate, fiber necrosis, and, occasionally, vacuolated muscle fibers 2319520

Weight change
  • Weight loss due to:
    • Decreased appetite
    • Side effects of medications (particularly diuretics)
    • Gastrointestinal symptoms related to lupus (e.g. gastroesophageal reflux, abdominal pain, peptic ulcer disease, or pancreatitis)
  • Weight gain due to:
    • Hypoalbuminemia:
      • Result in salt and water retention (e.g. due to nephrotic syndrome or protein losing enteropathy)
    • Increased appetite associated with the use of glucocorticoids
Extremities Arthritis
  • migratory arthritis means that the symptoms may be gone by 24 hours
  • Usually symmetrical and polyarticular with a predilection for the knees, carpal joints, and joints of the fingers, especially the proximal interphalangeal (PIP) joint
  • Morning stiffness with a really short duration (less than 1 hour)
  • Painful arthritis and decrease range of motion
  • The SLE arthritis is mostly nondeforming
  • It has been noted that 15-50% of patients may develop flexion deformities, ulnar deviation, soft tissue laxity, and swan neck deformities 3041493 9849313 19591780 7841997

12528115

  • Synovial effusions are infrequent in patients with SLE, usually small with slightly cloudy or clear fluid
  • septic arthritis may occurs as a result of low immune response and medication. it can be secondary to infections with Salmonella, gonococci, meningococci, and other organisms
Skin and mucous membrane involvement  acute cutaneous lupus erythema (ACLE) (also known as “the butterfly rash”)
  • Localized ACLE (ie, malar rash, butterfly rash)
  • Generalized ACLE
  • Toxic epidermal necrolysis-like ACLE

presents as erythema in a malar distribution over the cheeks and nose (but sparing the nasolabial folds), which appears after sun exposure

discoid lesions more inflammatory and which have a tendency to scar
Photosensitivity common theme for skin lesions associated with SLE
oral and/or nasal ulcers usually painless
Nonscarring alopecia may occur at some point during the course of their disease
Subacute cutaneous lupus erythematosus (SCLE) Annular SCLE

Papulosquamous SCLE

Drug-induced SCLE

Less common variants: erythrodermic, poikilodermatous, erythema multiforme-like (Rowell syndrome), and vesiculobullous annular SCLE

Chronic cutaneous lupus erythematosus (CCLE) Discoid lupus erythematosus (DLE)

Localized DLE

Generalized DLE

Hypertrophic DLE

Lupus erythematosus tumidus (LE tumidus)

Lupus profundus (also known as lupus panniculitis)

Chilblain lupus erythematosus (chilblain LE)

Lichenoid cutaneous lupus erythematosus-lichen planus overlap syndrome (LE-LP overlap syndrome)

nephritis Minimal mesangial lupus nephritis (class I) the earliest and mildest form of glomerular involvement

normal urinalysis, no or minimal proteinuria, and a normal serum creatinine

mesangial immune deposits that are identified by immunofluorescence alone or by both immunofluorescence and electron microscopy, but such patients do not have light microscopic abnormalities

Mesangial proliferative lupus nephritis (class II)  microscopic hematuria and/or proteinuria

Light microscope:

mesangial hypercellularity (of any degree) or mesangial matrix expansion

A few isolated subepithelial or subendothelial deposits may be seen on immunofluorescence or electron microscopy

Focal lupus nephritis (class III)  hematuria and proteinuria

may also have hypertension, a decreased glomerular filtration rate, and/or nephrotic syndrome

Light microscopy: Active or inactive endocapillary or extracapillary segmental glomerulonephritis (Less than 50 percent of glomeruli are affected)

Electron microscopy: immune deposits in the subendothelial space of the glomerular capillary and mesangium

Glomeruli affected by fibrinoid necrosis and crescents

Presence of tubulointerstitial or vascular abnormalities

Class III (A)=focal proliferative lupus nephritis

class III disease with active lesions

Class III (C)=focal sclerosing lupus nephritis

chronic inactive lesions with scarring

Diffuse lupus nephritis (class IV) most common and most severe form 

Hematuria and proteinuria

nephrotic syndrome, hypertension, and reduced glomerular filtration rate

hypocomplementemia (especially C3) and elevated anti-DNA levels, especially during active disease  7231154

Light microscopy: more than 50 percent of glomeruli are affected that display endocapillary with or without extracapillary glomerulonephritis and Mesangial abnormalities

Electron microscopy: subendothelial deposits, at least during the active phase and diffuse wire loop deposits, but with little or no glomerular proliferation

This class is classified based on the tissue involvement to segmentally (S) or globally (G), and based on the inflammatory activity (or chronicity) of the lesions into 6 subclasses include:

Class IV-S (A)

Class IV-G (A)

Class IV-S (A/C)

Class IV-G (A/C)

Class IV-S (C)

Class IV-G (C)

Vascular abnormalities
Livedo reticularis a reddish-cyanotic, reticular pattern on the skin of the arms, legs, and torso, particularly with cold exposure

induced by vasospasm of the dermal ascending arterioles

Vasospasm in these cutaneous vessels 

Pathologic examination of involved blood vessels reveals thickening of the walls of the dermal vessels with subsequent narrowing of the lumens and, in some cases, intravascular thrombi

Raynaud phenomenon vasospastic process

blanching of the nail beds, fingers, and toes (and occasionally ears, nose, tongue, and nipples) with accompanying pain

Vasculitis urticarial vasculitis: lesions may remain for more than 24 hours and frequently evolve into painful petechiae or purpura that may heal with hyperpigmentation

small arteries involvement: microinfarcts of the tips of the fingers, the toes, the cuticles of the nail folds (splinter hemorrhages), and the extensor surface of the forearm and shin

Periarteritis nodosa-like lesions may occur

Nail abnormalities pitting, ridging, and onycholysis
Mucosal manifestations painless oral ulcers  Treatment: topical corticosteroids, tacrolimus 0.1% ointment, intralesional corticosteroids, and systemic antimalarial drugs
Bullous cutaneous lupus erythematosus rare and distinct complication of SLE 

Skin

Discoid Lupus Erythematosus
Head
Face
Lupus Erythematosus-Systemic
Face
Extremity
Trunk

Lupus Erythematosus Chronicus Disseminatus Superficialis

Lupus Erythematosus Chronicus Verrucous

Lupus Erythematosus Profundus

Lupus Erythematosus-Subacute Cutaneous Lupus Erythematosus

Lupus Vulgaris

References

  1. ↑ Parodi A, Cozzani E (2014). "Cutaneous manifestations of lupus erythematosus". G Ital Dermatol Venereol. 149 (5): 549–54. PMID 25077888.
  2. ↑ Szczęch J, Rutka M, Samotij D, Zalewska A, Reich A (2016). "Clinical characteristics of cutaneous lupus erythematosus". Postepy Dermatol Alergol. 33 (1): 13–7. doi:10.5114/pdia.2014.44031. PMC 4793050. PMID 26985173.
  3. ↑ Walling HW, Sontheimer RD (2009). "Cutaneous lupus erythematosus: issues in diagnosis and treatment". Am J Clin Dermatol. 10 (6): 365–81. doi:10.2165/11310780-000000000-00000. PMID 19824738.
  4. ↑ Preble JM, Silpa-archa S, Foster CS (2015). "Ocular involvement in systemic lupus erythematosus". Curr Opin Ophthalmol. 26 (6): 540–5. doi:10.1097/ICU.0000000000000209. PMID 26367085.
  5. ↑ Silpa-archa S, Lee JJ, Foster CS (2016). "Ocular manifestations in systemic lupus erythematosus". Br J Ophthalmol. 100 (1): 135–41. doi:10.1136/bjophthalmol-2015-306629. PMID 25904124.
  6. ↑ Robson AK, Burge SM, Millard PR (1992). "Nasal mucosal involvement in lupus erythematosus". Clin Otolaryngol Allied Sci. 17 (4): 341–3. PMID 1526055.
  7. ↑ Anyanwu CO, Ang CC, Werth VP (2013). "Oral mucosal involvement in bullous lupus". Arthritis Rheum. 65 (10): 2622. doi:10.1002/art.38051. PMC 4333153. PMID 23780804.
  8. ↑ Ranginwala AM, Chalishazar MM, Panja P, Buddhdev KP, Kale HM (2012). "Oral discoid lupus erythematosus: A study of twenty-one cases". J Oral Maxillofac Pathol. 16 (3): 368–73. doi:10.4103/0973-029X.102487. PMC 3519212. PMID 23248469.
  9. ↑ Sacre K, Escoubet B, Pasquet B, Chauveheid MP, Zennaro MC, Tubach F, Papo T (2014). "Increased arterial stiffness in systemic lupus erythematosus (SLE) patients at low risk for cardiovascular disease: a cross-sectional controlled study". PLoS ONE. 9 (4): e94511. doi:10.1371/journal.pone.0094511. PMC 3983200. PMID 24722263.
  10. ↑ Melikoglu MA, Melikoglu M (2008). "The clinical importance of lymphadenopathy in systemic lupus erythematosus". Acta Reumatol Port. 33 (4): 402–6. PMID 19107085.
  11. ↑ Salvati F (2015). "[The involvement of pulmonary interstitial tissue in multisystemic lupus erythematosus: interdisciplinarity and role of the pneumologists]". Clin Ter (in Italian). 166 (5): 205–7. PMID 26550810.
  12. ↑ Torre O, Harari S (2011). "Pleural and pulmonary involvement in systemic lupus erythematosus". Presse Med. 40 (1 Pt 2): e19–29. doi:10.1016/j.lpm.2010.11.004. PMID 21194884.
  13. ↑ Alamoudi OS, Attar SM (2015). "Pulmonary manifestations in systemic lupus erythematosus: association with disease activity". Respirology. 20 (3): 474–80. doi:10.1111/resp.12473. PMC 4418345. PMID 25639532.
  14. ↑ Finucci Curi P, Pierrestegui M, Ortiz A, Ceccato F, Paira S (2015). "[Pulmonary hemorrhage in patients with systemic lupus erythematosus. Clinical manifestations and prognosis]". Med Clin (Barc) (in Spanish; Castilian). 145 (9): 375–9. doi:10.1016/j.medcli.2014.07.034. PMID 25458514.
  15. ↑ 15.00 15.01 15.02 15.03 15.04 15.05 15.06 15.07 15.08 15.09 15.10 15.11 15.12 15.13 15.14 15.15 15.16 15.17 15.18 15.19 15.20 15.21 15.22 15.23 15.24 15.25 15.26 15.27 15.28 15.29 15.30 15.31 15.32 15.33 15.34 15.35 15.36 15.37 15.38 15.39 15.40 15.41 15.42 15.43 15.44 15.45 15.46 15.47 15.48 15.49 15.50 15.51 15.52 15.53 15.54 15.55 15.56 15.57 15.58 15.59 15.60 15.61 15.62 15.63 15.64 15.65 15.66 15.67 15.68 15.69 15.70 15.71 15.72 15.73 15.74 15.75 15.76 15.77 "Dermatology Atlas".
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