Phy­si­ci­an-Pati­ent Relationship

Lupus is a rare dise­a­se with diver­se, often non-spe­ci­fic sym­ptoms. Years can pass until a cor­rect dia­gno­sis is made. 

Joint pain or inflamma­ti­on is the initi­al sym­ptom in 60% of affec­ted indi­vi­du­als and prompts a doc­tor’s visit.

It can be dif­fi­cult to dia­gno­se Syste­mic Lupus Ery­the­ma­to­sus (SLE), as not all clas­si­fi­ca­ti­on cri­te­ria are always met at the outset.

Skin chan­ges are the first sym­ptom in appro­xi­m­ate­ly 20% of affec­ted individuals.

Diver­se sym­ptoms com­pli­ca­te diagnosis

Gene­ral sym­ptoms such as seve­re fati­gue and fever are often pre­sent con­curr­ent­ly and are the pre­do­mi­nant initi­al sym­ptom in 5–10% of affec­ted individuals. 

In 10–15% of affec­ted indi­vi­du­als, SLE beg­ins with the invol­vement of inter­nal organs such as the kid­neys or the ner­vous system.

It is important that Syste­mic Lupus Ery­the­ma­to­sus is dia­gno­sed as quick­ly as pos­si­ble to ensu­re opti­mal and time­ly tre­at­ment for tho­se affected.

Sym­ptoms and Labo­ra­to­ry Values

During a doc­tor’s con­sul­ta­ti­on, it is important for affec­ted indi­vi­du­als to pro­vi­de pre­cise infor­ma­ti­on about all their symptoms.

The dia­gno­sis of SLE typi­cal­ly invol­ves: A cli­ni­cal exami­na­ti­on by the phy­si­ci­an, which fol­lows the SLE dia­gno­stic cri­te­ria. Labo­ra­to­ry tests, which spe­ci­fi­cal­ly deter­mi­ne auto­an­ti­bo­dies, among other things.

The search for spe­ci­fic anti­bo­dies in the blood – anti­nu­clear anti­bo­dies (ANA), anti­bo­dies against dsDNA, anti­bo­dies against the Smith anti­gen, and against car­dio­li­pin – can aid in diagnosis. 

Other labo­ra­to­ry values also con­tri­bu­te to the dia­gno­sis of SLE: inflamma­ti­on para­me­ters, decrea­sed com­ple­ment fac­tors (C3, C4), decrea­se in leu­ko­cytes, lym­pho­cytes, or plate­lets, etc.

Exami­na­ti­on of kid­ney func­tion para­me­ters, inclu­ding uri­na­ly­sis, can also pro­vi­de indi­ca­ti­ons of an SLE condition.

SLE Dia­gno­stic Criteria

The dia­gno­sis of SLE is based on the ACR/​EULAR clas­si­fi­ca­ti­on criteria. 

The new ACR and EULAR clas­si­fi­ca­ti­on cri­te­ria from 2019 for lupus include posi­ti­ve ANA anti­bo­dies as an ent­ry cri­ter­ion for lupus diagnosis.

Cli­ni­cal Criteria

Con­sti­tu­tio­nal Symptoms
Points
Fever
2
Skin
Points
Non-scar­ri­ng alopecia
2
Oral ulcers
2
Subacu­te cuta­neous or dis­co­id LE
4
Acu­te cuta­neous LE
6
Arthri­tis
Points
Syn­ovi­tis in ≥ 2 joints or ten­der­ness in ≥ joints with mor­ning stiff­ness ≥ 30 min
6
Neu­ro­lo­gy
Points
Deli­ri­um
2
Psy­cho­sis
3
Sei­zu­res
5
Sero­si­tis
Points
Pleu­ral or peri­car­di­al effusion
5
Acu­te pericarditis
6
Hema­to­lo­gy
Points
Leu­ko­pe­nia
3
Throm­bo­cy­to­pe­nia
4
Auto­im­mu­ne hemolysis
4
Kid­neys
Points
Pro­te­in­uria ≥ 0.5g/24h
4
Lupus nephri­tis (histol.) Type II, V
8
Lupus nephri­tis (histol.) Type III, IV
10

Immu­no­lo­gi­cal Criteria

Anti­phos­pho­li­pid-AK
Points
Anti­car­dio­li­pin IgG ≥ 40 GPL
2
Anti-β2G­P1-lgG ≥ 40 GPL
2
Lupus anti­co­agu­lant
2
Com­ple­ment
Points
C3 or C4 decreased
3
C3 and C4 decreased
4
Auto-AK
Points
Anti-dsDNA-AK
6
Anti-Smith-AK
6

Clas­si­fi­ca­ti­on as SLE ANA ≥ 1:80 and ≥ 10 points

ACR/​EULAR cri­te­ria 2019 for the clas­si­fi­ca­ti­on of syste­mic lupus ery­the­ma­to­sus by: Arin­ger M.: EULAR/​ACR clas­si­fi­ca­ti­on cri­te­ria for SLE. Semin Arthri­tis Rhe­um 2019; 49, S14-S17

The­ra­py and Tre­at­ment Goals

In the­ra­py, the phy­si­ci­an and lupus pati­ents each have their own responsibilities. 

The­re are are­as for which the phy­si­ci­an is respon­si­ble: labo­ra­to­ry, medi­cal state­ments, kee­ping abreast of the latest rese­arch. Then the­re are are­as for which lupus pati­ents them­sel­ves are respon­si­ble: life­style, adhe­rence to the­ra­py (com­pli­ance), taking medi­ca­ti­on. Howe­ver, the­re are also are­as that phy­si­ci­ans and lupus pati­ents can only address together. 

Pati­ents should, in con­sul­ta­ti­on with their trea­ting phy­si­ci­an, deter­mi­ne which the­ra­py is most sui­ta­ble for them with the grea­test pos­si­ble effect. Many fac­tors play a role, inclu­ding per­so­nal ones (fami­ly situa­ti­on, auto­no­my, mobi­li­ty, moti­va­ti­on, values, etc.). 

We recom­mend a com­pre­hen­si­ve eva­lua­ti­on by a spe­cia­list phy­si­ci­an for lupus.

Lupus pati­ents and their phy­si­ci­an should defi­ne a tre­at­ment goal. The tre­at­ment goal is re-eva­lua­ted at each con­sul­ta­ti­on; if neces­sa­ry, the the­ra­py and tre­at­ment goal are adju­sted to the cour­se of the lupus. It is important that lupus pati­ents are invol­ved in the con­side­ra­ti­ons and decisions. 

Fun­da­men­tal­ly, the goal of the­ra­py is to ensu­re a sta­ble sta­te of lupus.