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STASE REUMATOLOGI

Acute Lupus Pneumonitis

Pembimbing: DR. dr. Ayu Paramaiswari Sp.PD KR


Presentan : dr. Irmawati Suling
IDENTITAS
• Nama : Nn. YS
• Usia : 21 tahun
• Pekerjaan : Mahasiswa
• MRS : Selasa, 21 Juli 2020
ANAMNESIS
KU: Lemas memberat

10 hari SMRS os mengeluh muncul sariawan di rongga Hari masuk rumah sakit lemas makin memberat, sariawan
mulut (+), sulit menelan (+), demam (+), nyeri sendi (+), di rongga mulut makin penuh dan makin nyeri hingga sulit
batuk dan nyeri BAK disangkal, nyeri kepala (-), kejang menelan meskipun menela air, demam (+) batuk
(-). disangkal.

1 minggu SMRS os mengeluh lemes, sariawan makin penuh di rongga mulut,


nafsu makan menurun karena nyeri saat menelan, demam naik turun, Os kontrol
ke Poli Rheumato dan mendapat terapi ciprofloxacin dan nystatin drop.

RPD : Os adalah penderita SLE sejak 2017 manifestasi (NPSLE, mukokutan, artritis) telah menjalani kemoterapi 14
siklus, terakhir oktober 2019. Saat ini terapi rutin MP 16 mh (1-0-0) phenitoin 2x100 mg. HT dan DM disangkal.
RPK : SLE pada keluarga disangkal, HT, DM dan keganasan disangkal
PEMERIKSAAN FISIK

KU : lemah, CM Kepala leher :


Paru :
TD = 100/60 mmHg Conjunctiva pucat (-/-) Sklera
I : Simetris, retraksi (-/-)
N : 100x/menit ikterik (-), oral trust (+), faring
P :stem fremitus simetris normal
RR = 24 x/menit hiperemis (+) .
P : sonor (+/+) simetris
T = 36,7 C A : vesicular meningkat +/ +, crackles +/-,
SpO2 = 98 on NK 3 lpm RBB -/-, wheezing -/-
Jantung :
BB = 50 kg S1-S2 tunggal, bising (-), ictus
TB = 160 cm cordis teraba di ICS V linea
IMT = 25,78 axillaris anterior sinistra
Abdomen
I : Flat
A : Bising usus (+) 12x/menit
P : timpani (+), pekak alih (-)
P : NT (-), hepatomegali (-), splenomegali (-) Extremities:
Edema (-), eritema (-),
nyeri tekan (-),
Laboratorium
LAB 21/7/20 LAB 24/3/20 LAB 21/7/20 AGD 23/7/2020
Hb 11.8 Alb 3.0 Ph 6.5 FiO2 98
AL 4.36 SGOT 87 BJ 1.010 pH 7.47
AT 87 SGPT 54 Blood +2 pCo2 22.2
AE 4.29 GDS 92 Nit - pO2 62.7
HMT 33.4 BUN 19.7 LE - SO2 93.6
MCV 77.9 Cr 1.19 Erit 136 Beb -5.2
MCH 27.5 Na 122 Leu 17 HCO3 16.4
S 75.2 K 4.7 Bact 300 A-aDO2 594
L 17.7 Cl 93 Yeast 0 p/f 61.8
M 7.1 Osm Prot +2
E 0 Procal 0.44 silinder -
B 0
#lim 770
Rontgen Thorax

Kesan :
- Pneumonia dextra
- Besar Cor normal

21 Juli 2020
Mex Sledai
• Mukokutan : 2
• Nefritis : 6
• Trombositopnenia: 3
• Artritis : 2
• Limfopenia : 1
Total : 14
DIAGNOSIS
1. SLE flare manifestasi Nefritis, Hematologi, mukokutan, Artritis
riwayat NPSLE,
2. Kandidiasis oral dengan dehidrasi sedang
3. Community Acquired Pneumonia
4. Hiponatremia hipoosmolar hipovolemik
TERAPI PLAN

• O2 Nk 3 lpm • Monitor TTV berkala


• IVFD NaCl laoding 500-1000 cc  Nacl • Monitor UOP
0.9% : aminofluid 20 tpm • Cek Cat Gram, KS Sputum
• Diet per NGT 1500 kkal
• Inj. Ampi sulbactam 1.5 gr/6 jam
• Inj. Fluconazole loading dose 200 mg  100
mg/24 jam
• Inf. Paracetamol 1 gr/8 jam
• Nystatin drop 3x10 gtt
• Phenytoin 2x 100 mg
FOLLOW UP BANGSAL
HP 2 (Kamis , 23/7/20)
HP 1 (Rabu 22/7/20) (sore)
• kondisi memburuk , sesak HP-6 (Senin , 27/7)
• Kondisi menetap. nafas memberat • kondisi mbaik , sesak
kes : CM
• Plan: membaik RR 26
TD : 78/41, RR 30 , S 37,6,
 Pemantauan OT  OT 87 - 187 PT 58 - 74
Spo2 98% NRM 10 lpm
PT terkait IgM IgG sarscov2 Reaktif  Fluconazole stop , diganti
pemberian dengan mycamin 100
fluconazole Plan :
 Pemberian Pindah Gatot Kaca
mg/24 jam
antibitotik HAP Ab diganti Meropenem  hydroxychloroquin 1x200
sesuai TS paru Pemberian vasopresor setelah mg
loading cairan

HP-2 (Kamis, 23/7) HP-5 (Minggu, 26/7)


(pagi) • Keluhan menetap
• Kondisi menetap , • swab PCR I Sars Cov 2 :
• Plan: negatif
 MP rencana masuk • LED 40 , CRP 48 ,
setelah 3 hari • C3 complemen 28 ,
pemberian antibiotik • C4 complemen 5
terkait pneumonia • Procal 0,4
• Plan :
• pulse dose MP 500 mg/24
jam H1
HP 15 (Rabu , 5/8)
pasien BLPL :
HP-8 (Rabu , 29/7/20) - Cefixim 2x200 mg
- Paacetamol 3x500 mg
• Kondisi sesak menetap kp
RR 26 - Berotec 2x1
Hydorxychloroquin
1x200 mg
• Pindah HCU (2 kali
Minocep gargle /12 jam
PCR negatif)

HP-14 (selasa, 4/8)


• keluhan sesak membaik
• oral ulcer (-) , eritem (-)
oral thrush (-)

• Plan : pindah bangsal


biasa
Follow Up Terapi
22 juli 2020 23 juli 2020 24 Juli 2020
Terapi : Terapi Terapi
- Cukupi kebutuhan cairan 30cc/kgbb/24 - O2 NK 3lpm - O2 NRM 10 lpm
jam - Diet TKTP on NGT - - Diet TKTP on NGT
- Diet per NGT – T - IVFD NaCl 0.9% 30 tpm - IVFD NaCl 0.9% 30 tpm
- Tunda MP - Inj Ampicillin-Sulbactam 1.5 gr/6 jam - Inj Ampicillin-Sulbactam 1.5 gr/6 jam (22/7) stop
- Usul raber paru- Usul inj Ampicillin- (22/7) - Inj. Meropenem 1gr/8jam (23/7)
Sulbactam 1.5 gr/6 jam (atau sesuai TS - Inj. Fluconazole 100mg/24jam (22/7) - Inj. Ciprofloxacin 400mg/12jam (23/7)
paru) - Nystatin drop 3x10 gtt - Inj. Fluconazole 100mg/24jam (22/7)
- Nystatin drop 3x10 gtt - Phenytoin 2x100 mg - Nystatin drop 3x10 gtt
- Inj. Parasetamol 1 gr/8 jam untuk nyeri - Parasetamol 1 gr K.P i.v - Phenytoin 2x100 mg
dan demam T > 38C - Parasetamol 1 gr K.P i.v
- Phenytoin 2x100 mg- NaCl 3% 8 tpm - Minocep Gurgle/12jam
Plan:
Plan : - Swab PCR COVID-19 H1 dan H2
- Konfirmasi terkait rencana pemberian - Cukupi kebutuhan cairan 30cc/kgbb/24 jam
fluconazole dan adanya elevated liver - Nutrisi adekuat melalui NGT
enzime - Monitoring UOP/24 jam
- arget MAP > 65; Pertahankan dengan Noreinefrin
jika belum tercapai
Follow Up Terapi
27 Juli 2020 29 Juli 2020 30 Juli 2020
Terapi Terapi Terapi
- O2 NRM 10 lpm - O2 NRM 10 lpm - O2 NRM 10 lpm
- Diet TKTP on NGT - Diet TKTP on NGT - Diet TKTP –
- IVFD NaCl 0.9% 30 tpm - IVFD NaCl 0.9% 30 tpm - IVFD NaCl 0.9% 30 tpm-
- Inj MP 500mg/24 jam H2 - inj MP 500mg/24 jam H3 stop - Inj. Meropenem 1gr/8jam (23/7) H8-
- Inj. Meropenem 1gr/8jam (23/7) - Inj. Meropenem 1gr/8jam (23/7) H7 - Inj. Ciprofloxacin 400mg/12jam (23/7)
- Inj. Ciprofloxacin 400mg/12jam (23/7) - Inj. Ciprofloxacin 400mg/12jam (23/7) H7 H8
- Inj. Fluconazole 100mg/24jam (22/7) usul - Inj. Mycamin 100mg/24 jam (27/7) H3 - Inj. Mycamin 100mg/24 jam (27/7) H4-
stop diganti mycamin 100mg/24 jam - Nystatin drop 3x10 gtt - Nystatin drop 3x10 gtt- Phenytoin 2x100
- Nystatin drop 3x10 gtt - Phenytoin 2x100 mg mg
- Phenytoin 2x100 mg - Parasetamol 1 gr K.P i.v - Parasetamol 1 gr K.P i.v
- Parasetamol 1 gr K.P i.v - Minocep Gurgle/12jam - Minocep Gurgle/12jam- Ij PPI 1 amp/12
- Minocep Gurgle/12jam- Ij PPI 1 amp/12 jam - Ij PPI 1 amp/12 jam jam
Plan: Plan: Plan:
- Swab PCR COVID-19 H2 - Pindah bangsal perawatan Non-isolasi - Usul Evaluasi DR, Kimia darah, urin ruti
- Cukupi kebutuhan cairan 30cc/kgbb/24 jam - Usul Besok Evaluasi DR, SGOT, SGPT - Cukupi kebutuhan cairan 30cc/kgbb/24
- - Nutrisi adekuat melalui NGT - Cukupi kebutuhan cairan 30cc/kgbb/24 jam- jam
- Monitoring UOP/24 jam - Nutrisi adekuat melalui NGT - Nutrisi adekuat- Monitoring UOP/24 jam
- Monitoring UOP/24 jam
Follow Up Terapi
3 agustus 2020 4 agustus2020 5 agustus 2020
Terapi- Terapi BLPL terapi pulang :
O2 NRM 10 lpm- - O2 NRM 10 lpm- - Cefixim 2x200 mg
Diet TKTP - IVFD NaCl 0.9% 30 tpm - Diet TKTP - IVFD NaCl 0.9% 30 tpm - Paacetamol 3x500 mg kp
- Inj. Meropenem 1gr/8jam stop- - Inj. Mycamin 100mg/24 jam (27/7) H8- - Berotec 2x1
- Inj. Ciprofloxacin 400mg/12jam stop- - Inj. Ceftazidime 1gr/8jam (30/7) H5- Hydorxychloroquin 1x200 mg
- Inj. Mycamin 100mg/24 jam (27/7) H8- - Phenytoin 2x100 mg- Minocep gargle /12 jam
- Inj. Ceftazidime 1gr/8jam (30/7) H5 - Parasetamol 1 gr K.P i.v- Minocep Gurgle/12jam- Ij
- Phenytoin 2x100 mg- PPI 1 amp/12 jam-
- Parasetamol 1 gr K.P i.v- - Hydroxychloroquin 1x200 mg
- Minocep Gurgle/12jam- I
- j PPI 1 amp/12 jam- Plan:-
- Hydroxychloroquin 1x200 mg Cukupi kebutuhan cairan 30cc/kgbb/24 jam-
Plan Nutrisi adekuat-
:- Cukupi kebutuhan cairan 30cc/kgbb/24 jam Monitoring UOP/24 jam-
- Nutrisi adekuat- Transfusi Albumin-
- Monitoring UOP/24 jam- Usul pindah bangsal
- Transfusi Albumin- Usul pindah bangsal
Perkembangan AGD

23/7 27/7 29/7 30/7

PH 7,47 7,253 7,41 7,48

PO2 62,7 74 41,8 237,8

PCO2 22,2 27,5 29,1 26,4

HCO3 16,4 12,6 18,6 19,8

BE -7,3 -14 -6,6 -2,6

AaDO2 594,6 340,1 558,8 292,8

P/F 61,8 248 49,1 299,1


Evaluasi Ro. Thorax
Tanggal 25/7/2020 Tanggal 03/08/2020

Kesan : Pneumonia bilateral, terutama dextra Kesan : - Oedem pulmo, - Pneumonia bilateral
- Besar cor normal, dibandingkan foto sebelumnya pada - Besar cor normal
22/07/2020, tampak gambaran pneumonia bertambah.
Fokus Pembahasan

Manifestasi Paru yang muncul pada pasien ini, apakah


suatu pneumonia ec viral infection, bacteria, jamur atau
suatu acute pneumonitis lupus?
KS Darah 23 Juli 2020
Perkembangan Lab
22/7 23/7 26/7 24/7
LED 40
procalsitonin 0,44 0,62 0,4 CRP 48
C3 complemen 28
23/7 C4 complemen 5
Tbil 0,47 Anti DsDNA > 200
Dbil 0,46
LDH 1011
23/7 24/7 25/7
Ddimer 320
Fibrinogen 982 IgM igG sarscov2 Reaktif

PCR swab sarscov2 negatif negatif


Acute Lupus Pneumonitis
• Acute lupus pneumonitis (ALP) is an uncommon manifestation, occurring in 1%
to 12% of patients  is one of the most dreaded life-threatening syndromes
complicating SLE, with short-term mortality of 50-90%
• Two factors contribute to the diagnosis: on the one hand, the onset of
pneumonia is practically always contemporaneous with exacerbation of the
disease with multisystem involvement, including nephritis, arthritis, and serositis
and on the other hand, the pneumonia occurs in a majority of cases in patients
with anti-SSA antibodies (82%)
• Occasionally, there is a rapid clinical deterioration with progression to acute
respiratory failure that requires mechanical ventilation
Beatrice Memet, M.D.1 and Ellen M. Ginzler, M.D., M.P.H., Semin Respir Crit Care Med 2007;
Risk Factor
How To diagnosis
• The diagnosis is difficult as the clinical picture is nonspecific
• The clinical presentation is nonspecific with fever, dyspnea, pleuritic
chest pain, and cough with minimal sputum production and occasional
hemoptysis.
• Lung involvement may be unilateral or bilateral. Physical exam may
reveal crepitations, bronchial breath sounds or decreased breath sounds
in the basal regions owing to effusion.
• Arterial blood gas analysis reveals hypoxemia with hypocapnia.
• Most patients with lupus pneumonitis will be antidsDNA antibody
positive and low complement level

Diane L. Kamen, MD, MSCRa,*, Charlie Strange, MD, 2010


Cont...
• Bronchoalveolar lavage should be performed  exclusion of an infectious
process  BAL fluid analysis can be helpful, with eosinophilic or
neutrophilic lavage fluid predicting a worse prognosis
• Rise of CRP (or hs-CRP) is only modest, at best in active SLE without
infection while a high hs-CRP level (>5-6 mg/dL) is a strong predictor of
infection.
• ESR/CRP ratios >15 suggest lupus flare, while ratios <2 suggest infection.
• A lung biopsy is helpful to differentiate between ALP and DAH
Radiologic finding
• Chest radiography and computed tomography (CT) show
uni or bilateral alveolar infiltrates with a groundglass
appearance, usually in the lower lobes, Small pleural
effusions are common.
• Rarely, the initial chest radiograph may be normal.
• CT scan of the chest should reveal the underlying lesions
Role of Bronchoalveolar Lavage in Diagnosis
and Management of ALP
• Bronchoalveolar lavage (BAL) is given valuable information and may
help diagnosis in cases like infection.
• The main role of BAL is to help in ruling out differential diagnosis like
chest infection and pulmonary haemorrhage.
• The presence of blood or haemosiderin laden macrophages would
suggest pulmonary haemorrhage.
• The presence of haemosiderin in macrophages suggests that
pulmonary haemorrhage has occurred for more than 48 hours.
Role of High Resolution CT Scans in Diagnosis and
Management of ALP
• It has been shown by multiple studies that high resolution CT scan
(HRCT) is a sensitive means of detecting interstitial lung disease.
• It allows the identification of asymptomatic patient but the significance
of which has to be explored and correlated with BAL and lung biopsy.
• Serial HRCT serves as a good means for monitoring the disease
progress but it's role in pinpointing the diagnosis is limited.
• Patient with a ground glass and alveolar consolidation detected in
HRCT will be more likely to be steroid-sensitive.
• However, those with severe honeycomb lesion on the HRCT scans are
less likely to be treatment responsive
Cont..

• Computed tomography (CT) typically reveals a ground glass appearance; patchy areas of
consolidation, traction bronchiectasis, or pleural effusion may be present
Histologic Finding
• Histological features of ALP are nonspecific
and include alveolar wall damage and
necrosis, alveolar edema or hemorrhage,
hyaline membranes, and inflammatory cell
infiltration
• Capillary inflammation and fibrin thrombi may
also be present. Large-vessel vasculitis has
been rarely detected.
Management
• The basis of therapy for ALP is high-dose systemic corticosteroids along with broad-
spectrum antibiotics and supportive care.
• Empiric antibiotics are often started early, then discontinued as cultures return negative
for infections.
• Methylprednisolone is given intravenously at an equivalent prednisone dose of 1 to 2
mg/kg/d in divided doses, or for critically ill patients as a pulse-dose of 1 gm/d for 3-5 day
• High dose steroid for at least 6-8 weeks is suggested
• For refractory cases, cyclophosphamide, azathioprine and methotrexate may be
considered
• Plasmapheresis may be given as an adjunctive therapy.
• Immunosuppressive agents intravenous immunoglobulin, and plasmapheresis are used
alone or in combination in patients with a poor response to steroids
Prognosis and complication of ALP
• The overall prognosis for ALP is poor with 50-90% mortality despite
treatment Prompt identification and treatment are essential for survival during
the acute phase.
• For those who survived the acute episode, 50-100% would eventually
progress to chronic interstitial pneumonitis.
• Chronic interstitial pneumonitis can develop at any time. The prevalence of
symptomatic chronic interstitial pneumonitis has been reported to be 3%
• Unfortunately, favourable response occurred only in 15-30% of patien with
chronic interstitial lung disease.
• Complete remission occurred in less than 5%.1
Kesimpulan
• ALP merupakan komplikasi SLE yang jarang terjadi, namun memiliki prognosis buruk
dengan angka kematian 50-90% pada fase akut sehingga memerlukan identifikasi dan
terapi segera
• Ekslusi pneumonia karena infeksi pada tahapan awal diagnosis sangat diperlukan
• Kultur sputum/BAL membantu menyingkirkan infeksi oportunistik dan perdarahan, dan
membantu dalam diagnosis dan pengobatan dini.
• HRCT sangat sensitif dalam mendiagnosis penyakit dan perubahan abnormal dapat
dideteksi pada kasus asimtomatik.
• Prinsip terapi ALP adalah kortikosteroid sistemik dosis tinggi bersama dengan antibiotik
spektrum luas dan perawatan suportif.
• Steroid dosis tinggi disarankan untuk diberikan selama 6-8 minggu
MOHON ASUPAN

TERIMA KASIH

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